Materiały źródłowe

• #1 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Hairy Cell Leukemia (HCL) is a rare malignancy, but one of the most successfully treated of all leukemias. Since the advent of effective agents in the 1980s, most patients will enjoy very prolonged disease remission. Although current treatment is not considered curative, different therapies can be given sequentially as needed to control the disease. […] Careful and experienced clinical judgment is necessary to make the most appropriate decision for each individual patient regarding the optimal time to start treatment as well as which agent to select to treat the patients HCL. […] The decision of when to start treatment depends upon the character of the symptoms experienced by the patient and the degree of abnormality in the blood count. […] Not all patients will require treatment immediately after the diagnosis is made and can be monitored until it is needed. This is termed active monitoring and helps avoid the potential side effects of treatment when not necessary. This watch and wait approach can be difficult and anxiety-provoking for patients and their families. However, unlike other types of cancer or some other more acute subtypes of leukemias, it is safe to wait to start treatment for hairy cell leukemia until there are more definite signs that treatment is really needed.

• #2 Hairy Cell Leukemia Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq

  Hairy cell leukemia is highly treatable but rarely cured. Because it is easily controlled, many patients have prolonged survival with the use of sequential therapies. The decision to treat is based on signs of disease progression, including any of the following factors: […] If the patient is asymptomatic and if blood counts are maintained in an acceptable range, therapy may not be needed. […] Cladribine, with or without rituximab, remains the standard of care. However, due to the risk of serious and prolonged immunosuppression, nonchemotherapy treatment options may be preferable for older, frail patients with higher risks of infection (or for those who have active infections). BRAF inhibitors such as vemurafenib, dabrafenib, or encorafenib are nonchemotherapeutic options that can be combined with rituximab or obinutuzumab.

• #3 Hairy Cell Leukemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23177-hairy-cell-leukemia

  Theres no cure for hairy cell leukemia. Research shows treatment, including chemotherapy, can put this condition into remission for 10 years or more. Remission means you dont have symptoms anymore and tests dont find any signs of the condition. One analysis of peoples experience with hairy cell leukemia suggests that, after treatment, theyll live as long as people who dont have the condition. […] That said, hairy cell leukemia can come back after several years of initial treatment. But there are treatments to put the disease back into remission.

• #4 Treatment of hairy cell leukemia – UpToDate

  https://www.uptodate.com/contents/treatment-of-hairy-cell-leukemia

  Treatment of hairy cell leukemia […] The treatment of HCL will be reviewed here; clinical features and diagnosis of this disorder are discussed separately. […] Our pretreatment evaluation also includes the following studies, some of which are performed as part of the diagnostic evaluation: Laboratory studies include a complete blood count with differential and evaluation of the peripheral smear, chemistries with liver and renal function and electrolytes, uric acid, and lactate dehydrogenase. Hepatitis B and C serology should be obtained, especially for patients whose planned treatment includes rituximab. […] […] […] TREATMENT OF RESISTANT OR RELAPSED DISEASE: Resistant disease, Relapsed disease, Targeted therapies: Rituximab, BRAF inhibition (vemurafenib or dabrafenib), Venetoclax, Ibrutinib. […] […] […] Initial management of hairy cell leukemia. […] […] […] Assessment of response in hairy cell leukemia.

• #5 https://www.lls.org/leukemia/hairy-cell-leukemia/treatment/watch-and-wait

  https://www.lls.org/leukemia/hairy-cell-leukemia/treatment/watch-and-wait

  A small percentage of people can manage their hairy cell leukemia with their doctors using a watch-and-wait approach. By using the watch-and-wait method, your doctor can monitor your condition with regular physical exams and lab tests. You won’t take any drugs or undergo any treatment during this period. […] You must visit your doctor regularly so he or she can check you for any health changes, specifically watching whether your disease remains stable or starts to progress. Your doctor monitors your test results to decide when it’s time to start treatment and what the best treatment option is for you.

• #6 Treating Hairy Cell Leukemia | American Cancer Society

  https://www.cancer.org/cancer/types/chronic-lymphocytic-leukemia/treating/hairy-cell-leukemia.html

  Hairy cell leukemia (HCL) tends to grow slowly. People with HCL who aren’t having symptoms often don’t need to be treated right away, but they do need to be watched carefully. […] Treatment may be advised if you have low blood cell counts, recurrent infections, an enlarged spleen or lymph nodes, or other bothersome symptoms. Treatment doesn’t cure HCL, but it can help with symptoms, stop the HCL from progressing, and help you live longer. […] The first treatment for HCL is most often chemotherapy (chemo) with either cladribine (2-CdA) or pentostatin. Sometimes the monoclonal antibody rituximab is given after chemo. HCL usually responds well to these drugs, and the responses often last for many years. […] If the leukemia comes back at some point, it can often be treated with the same drug again, especially if the HCL stayed in remission for a long time (typically at least 2 years). If one chemo drug doesn’t work, another can be tried.

• #7 Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group

  https://www.mdpi.com/2072-6694/16/12/2185

  Here are the extracted sentences and paragraphs related to „Hairy cell leukemia Treatment, therapy”: […] We encourage inclusion in clinical trials whenever possible both in front-line and relapsed/refractory (R/R) settings. Inclusion in real-life registries and retrospective studies is also recommended whenever possible, because HCL is rare, and only collaborative studies can increase our knowledge and improve clinical outcomes for patients. As in all indolent B-cell lymphoproliferative disorders, HCL and HCL-like disorders should be treated only if one or more of the following criteria are present: (1) Hb < 11 g/dL and/or platelets < 100 G/L and/or neutrophils < 1 G/L, (2) symptomatic organomegaly, (3) constitutional symptoms (fever, weight loss, night sweats), (4) recurrent infections. If none of these criteria are present, we recommend a ‘watch and wait’ strategy. If a treatment is required, our expert panel recommends the following therapeutic algorithms for HCL (Figure 2), HCL-V (Figure 3) and SDRPL (Figure 4). Details on the therapeutic regimens are given in Table 4. [...]

• #8 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Hairy Cell Leukemia (HCL) is a rare malignancy, but one of the most successfully treated of all leukemias. Since the advent of effective agents in the 1980s, most patients will enjoy very prolonged disease remission. Although current treatment is not considered curative, different therapies can be given sequentially as needed to control the disease. […] Careful and experienced clinical judgment is necessary to make the most appropriate decision for each individual patient regarding the optimal time to start treatment as well as which agent to select to treat the patients HCL. […] The decision of when to start treatment depends upon the character of the symptoms experienced by the patient and the degree of abnormality in the blood count. […] Not all patients will require treatment immediately after the diagnosis is made and can be monitored until it is needed. This is termed active monitoring and helps avoid the potential side effects of treatment when not necessary. This watch and wait approach can be difficult and anxiety-provoking for patients and their families. However, unlike other types of cancer or some other more acute subtypes of leukemias, it is safe to wait to start treatment for hairy cell leukemia until there are more definite signs that treatment is really needed.

• #9 Hairy Cell Leukemia — Leukemia Research Foundation

  https://leukemiarf.org/leukemia/chronic-lymphocytic-leukemia/hairy-cell-leukemia/

  Most people with hairy cell leukemia have normal life expectancy with treatment. […] Treatment depends on your current symptoms, how fast the HCL progresses, and your response to treatment. […] Treatment options for HCL include: […] Your doctor may recommend monitoring your condition without immediate treatment. HCL progresses very slowly, so the doctor starts treatment when symptoms appear. This method delays harmful side effects of treatment. […] Chemotherapy drugs kill cancer cells. You receive this treatment either in pill form or as injections into a vein, muscle, or underneath the skin. […] Immunotherapy is a type of treatment that enhances your immune systems ability to fight the cancer. Immunotherapy drugs called monoclonal antibodies treat HCL recurrence (return of the cancer).

• #10 Hairy cell leukaemia | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma/hairy-cell-leukaemia

  The treatment you have for hairy cell leukaemia depends on how it is affecting you. […] You might not need treatment straightaway. This might be the case if you dont have troublesome symptoms, your liver or spleen are not too swollen, and your blood counts arent too low. Instead, your medical team monitors you every 3 to 6 months until you need treatment. This is called active monitoring or watch and wait. This approach allows you to avoid the side effects of treatment for as long as possible. […] You are likely to start treatment if: […] The most likely treatment for people with hairy cell leukaemia is chemotherapy. The most common chemotherapy drugs for hairy cell leukaemia are: […] Very rarely, your medical team might recommend having an operation to remove your spleen (a splenectomy). This is usually only considered if your spleen is very enlarged and causing serious problems.

• #11 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  For the past 30 years, the mainstay of treatment for HCL has been pentostatin and cladribine, two drugs in the class called purine analogues. Pentostatin was introduced in the 1980s followed by cladribine in the early 1990s. Today, there is a great deal of experience and long-term follow up using these two agents. Both are highly effective in inducing clinical response and a remission (in almost all patients), with no significant difference in efficacy between the two. Most of these remissions are complete (i.e., no evidence of any remaining disease in the bone marrow using standard methods). Such remissions often last for prolonged periods of time, sometimes even more than 10 years. […] In September 2018, moxetumomab pasudotox, an anti-CD22 immunotoxin, was approved by the U.S. Food and Drug Administration (FDA) for the treatment of adult patients with HCL who have already received at least two prior systemic therapies, including treatment with a purine nucleoside analog.

• #12 Hairy Cell Leukemia Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq

  Treatment options for hairy cell leukemia include: Watchful waiting, if feasible. Cladribine with or without rituximab. BRAF inhibitors (vemurafenib or dabrafenib) with or without rituximab or trametinib. Rituximab. Pentostatin. Ibrutinib. Re-treatment with cladribine or pentostatin. Bendamustine with rituximab. Splenectomy. Interferon. […] Patients with hairy cell leukemia who have a relapse after the first course of cladribine or pentostatin often respond well to re-treatment with the same or another purine analogue, especially if relapse occurs after several years. […] Splenectomy plays a decreasing role in treating hairy cell leukemia because effective alternatives are available. Splenectomy will partially or completely normalize the peripheral blood in most patients with hairy cell leukemia. After a splenectomy, there is usually little or no change in the bone marrow, and virtually all patients have progressive disease within 12 to 18 months.

• #13 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Pentostatin is administered as a short intravenous (IV) infusion every 2-3 weeks until a remission is achieved. […] Cladribine can be given in a number of ways, including as a 7-day continuous IV infusion (which may require hospital admission), daily IV infusion over 2 hours for 5 doses or weekly for 6 doses, or as a subcutaneous injection on 5 consecutive days. All modes of administration have been shown to be effective. The choice will largely depend on the physician and patient and most are given as outpatient treatment. […] Both drugs are generally well tolerated but are associated with a temporary reduction in normal blood counts, which need to be monitored closely (weekly initially) until recovery. […] Splenectomy is rarely performed nowadays since the agents used today are very effective in reducing the size of the spleen. The spleen is often enlarged at the time of diagnosis.

• #14 Hairy cell leukaemia cladribine

  https://www.eviq.org.au/haematology-and-bmt/leukaemias/hairy-cell-leukaemia/364-hairy-cell-leukaemia-cladribine

  Patients with leukaemia should be considered for inclusion into clinical trials. […] Response is usually manifested after a single course of cladribine. If a second course is necessary, it should be given after resolution of all haematological toxicities. […] Various regimens for cladribine in hairy cell leukaemia have been reported: Continuous IV infusion of 0.1 mg/kg/day for seven days, IV infusion of 0.14 mg/kg/day over 2 hours for five days, IV infusion of 0.15 mg/kg over 2 hours weekly for six weeks, Subcutaneous injection of 0.085 mg/kg/day for seven days. […] The eviQ Reference Committee believes that each of these regimens is a reasonable option. The 5 day IV protocol has been selected for its feasibility, as it is able to be administered in the outpatient setting within a short discrete treatment timeframe. However, it is acknowledged that clinicians may prefer to use one of the other regimens.

• #15 https://www.lls.org/leukemia/hairy-cell-leukemia

  https://www.lls.org/leukemia/hairy-cell-leukemia

  The drug cladribine is the initial treatment for most people who have hairy cell leukemia. […] The 5-year event-free survival rate after treatment is approximately 90 percent of patients initially treated with cladribine. The advent of cladribine therapy has resulted in approximately an 85 percent rate of complete remission and approximately 10 percent rate of partial response.

• #16 What Is Hairy Cell Leukemia? Treatment, Symptoms, and Causes

  https://www.everydayhealth.com/hairy-cell-leukemia/

  Hairy cell leukemia is considered a chronic condition, meaning it is incurable. […] But the good news is that it is highly treatable. People can go into long, sustained remissions, with levels of hairy cells in the blood and marrow that are so low, they are undetectable even using the most advanced technologies. […] Treatment for hairy cell leukemia can take a number of forms. These include: […] About 10 percent of patients with this slow-growing form of leukemia dont take any medication or receive any other form of therapy at all. Instead, doctors monitor the condition with regular physical exams and blood tests. […] This is one of the front-line treatment for hairy cell leukemia. Chemo drugs are toxic: They damage or kill hairy cells. […] Patients typically receive a type of chemo drug called a purine analog usually cladribine (Leustatin), administered intravenously every day for up to a week.

• #17 Hairy Cell Leukemia Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.hairy-cell-leukemia-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062926

  Pentostatin given IV every other week for 3 to 6 months produced a 50% to 76% complete response rate and an 80% to 87% overall response rate. […] Ibrutinib, a tyrosine kinase inhibitor, has been studied in the treatment of hairy cell leukemia. […] Patients with hairy cell leukemia who have a relapse after the first course of cladribine or pentostatin often respond well to re-treatment with the same or another purine analogue, especially if relapse occurs after several years. […] Splenectomy plays a decreasing role in treating hairy cell leukemia because effective alternatives are available.

• #18 Chemotherapy for hairy cell leukaemia | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/hairy-cell-leukaemia/treatment/about-chemotherapy

  Chemotherapy is the main treatment for hairy cell leukaemia. The most common chemotherapy drugs are cladribine or pentostatin. […] You might need further chemotherapy if your leukaemia comes back (relapses). You might have chemotherapy on its own or combined with a targeted drug called rituximab. […] The two main chemotherapy drugs for hairy cell leukaemia are clardibine and pentostatin. […] You have pentostatin into a vein every 2 weeks, until all signs of the leukaemia have gone or it is under control. The length of treatment varies from one person to another and depends on how the hairy cell leukaemia responds. […] Generally the treatment lasts between 3 to 6 months. […] You might have a different chemotherapy treatment if the first treatment doesn’t work. Or if your leukaemia comes back (relapses) after treatment. You often have this second line chemotherapy combined with the targeted cancer drug rituximab.

• #19 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Pentostatin is administered as a short intravenous (IV) infusion every 2-3 weeks until a remission is achieved. […] Cladribine can be given in a number of ways, including as a 7-day continuous IV infusion (which may require hospital admission), daily IV infusion over 2 hours for 5 doses or weekly for 6 doses, or as a subcutaneous injection on 5 consecutive days. All modes of administration have been shown to be effective. The choice will largely depend on the physician and patient and most are given as outpatient treatment. […] Both drugs are generally well tolerated but are associated with a temporary reduction in normal blood counts, which need to be monitored closely (weekly initially) until recovery. […] Splenectomy is rarely performed nowadays since the agents used today are very effective in reducing the size of the spleen. The spleen is often enlarged at the time of diagnosis.

• #20 Hairy cell leukaemia cladribine

  https://www.eviq.org.au/haematology-and-bmt/leukaemias/hairy-cell-leukaemia/364-hairy-cell-leukaemia-cladribine

  Cladribine produces profound and prolonged immunosuppression due to reduction of CD4 and CD8 T-lymphocytes. An increased incidence of opportunistic infections is expected during and six months following therapy with cladribine. […] The main toxicities are haematological and infectious. In the Zenhausern et al. study (5 days of IV cladribine), 90% developed grade 3-4 neutropenia. 44% developed any grade of infection. 38% required hospitalisation at some point in their therapy. 22% required packed cell transfusion while 8% required platelets. […] The Hairy Cell Leukaemia Foundation and the British Society for Haematology have both developed guidelines for the diagnosis and management of patients with hairy cell leukaemia (HCL). Various methods of administration of cladribine in HCL have been investigated. These include a single course of a seven day continuous intravenous (IV) infusion of 0.1mg/kg/day, IV infusion of 0.14 mg/kg/day over 2 hours for five days, weekly 2 hour IV infusion of 0.15 mg/kg for six weeks and subcutaneous (SC) bolus administration of 0.085 mg/kg/day for seven days. […] Each is considered to be a reasonable option in the above guidelines.

• #21 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Rituximab used as a single agent in first line treatment of HCL is not as effective in inducing remissions as the purine analogues. Its use would be reserved for patients unable to tolerate purine analogues. […] Targeted therapies such as BRAF inhibitors (vemurafenib) and B-cell receptor inhibitors (ibrutinib) have activity in HCL. Currently, these therapies have been examined in relapsed or refractory HCL and only in a very small number of patients for whom purine analogues cannot be given as first line therapy. […] Moxetumumab pasudotox (Lumoxiti) is administered as an intravenous infusion over 30 minutes on days 1, 3, and 5 of each 28-day cycle. […] HCL is a rare disease and there are very few clinical studies being undertaken worldwide. To continue to make progress in new therapies, it is important for patients at every stage of treatment to consider participation in clinical trials.

• #22 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hairy-Cell-Leukemia-Prognosis.aspx

  The treatment of hairy cell leukemia depends on how far advanced the disease is at the time of diagnosis. […] Most patients respond adequately when treated with cladribine or pentostatin. These drugs usually lead to remission for at least ten years, even if they are only taken once. Furthermore, the drugs are effective at treating any instances of disease relapse, although the disease-free remission is usually shorter lived after subsequent treatments. […] With adequate treatment, a patients lifespan may not be significantly reduced. The risk of death is highest in the first two years after diagnosis but if a patient stays in remission for more than five years, the outlook is positive and they may even qualify for life insurance.

• #23 Treatment of Classic Hairy Cell Leukemia: Targeting Minimal Residual Disease beyond Cladribine

  https://www.mdpi.com/2072-6694/14/4/956

  The first intriguing approach to pursue the novel therapeutic goal of MRD negativity was to combine purine analogues with the anti-CD20 monoclonal antibody rituximab, which has been associated with marked improvements in clinical outcomes in combination with chemotherapy in various non-Hodgkin’s lymphomas. […] The clinical benefit of combination treatment was confirmed in a smaller cohort of 14 patients in first relapse with a CR rate of 100% and a longer remission duration than with front-line purine analogue treatment in 6 evaluable patients. […] To better define the best strategy for combining rituximab with purine analogues, Chihara et al. performed a phase II study randomizing 68 previously untreated HCL patients 1:1 to either cladribine concurrently with eight weekly rituximab infusions or cladribine plus delayed rituximab infusions at least 6 months apart in case of MRD positivity. […] These efficacy and safety results strongly support a treatment strategy with up-front combination chemoimmunotherapy.

• #24 Hairy cell leukemia: 6 questions, answered | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/hairy-cell-leukemia–6-questions–answered.h00-159539745.html

  Theres been an evolution in therapy for hairy cell leukemia over the past 50 years. […] In the 1980s, interferon became the standard of care for a while. But it was not very curative. […] In the 1990s, the chemotherapy drugs cladribine and pentostatin were developed. Both proved effective against hairy cell leukemia, with many publications reporting an 80% to 100% response rate among patients who received them. Eventually, cladribine became the standard. […] Rituximab proved to be very effective against hairy cell leukemia, even as a single agent. So, we developed a combination of it and cladribine here at MD Anderson. Thats what weve been using ever since. Its a big success story in leukemia treatment. […] Patients whove received the combination of cladribine and rituximab here at MD Anderson have had a 100% response rate, with rare relapses over the past 18 years.

• #25 Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group

  https://www.mdpi.com/2072-6694/16/12/2185

  In first-line and fit patients, we recommend CDAR regimen with CDA plus rituximab (R). The data with P plus rituximab are limited. CDA plus R was demonstrated to be more effective than PNA in monotherapy, allowing to reach undetectable MRD (uMRD) in most patients and to achieve more durable responses. […] […] The simultaneous combination of R + CDA (i.e., R started at day 1 of CDA) was shown to be superior to the sequential schedule of CDA followed by R (i.e., R started 6 months later). R is given at day 1 of CDA for 8 weekly infusions. The overall response rate (ORR) and the CR rate were both 100%, with 97% of patients reaching uMRD. […] […] For unfit patients not able to receive CDA + R, PNA in monotherapy is a good option. Two or three days (instead of five) of subcutaneous CDA may be used in very elderly people. If patients are unfit to receive PNA alone, BRAFi +/− R or IFNα are alternative choices. R alone is not recommended because responses are partial and short-lasting. […]

• #26 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Usually within a few weeks of receiving treatment, the blood counts begin to recover and will often return to completely normal levels. However, to assess response to anti-HCL treatment, a bone marrow examination is necessary. […] About 40% of patients will relapse, even 10 years or more after initial therapy. Regular monitoring by a hematologist takes place, usually once or twice a year for patients in stable remission.

• #27 Treatment options for hairy cell leukaemia | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/hairy-cell-leukaemia/treatment/decisions

  The next lot of treatment you have is called second line treatment. You can have a further remission with more treatment. The remissions tend to get shorter, the more treatment you have. […] Treatment options include: the same chemotherapy drug (cladribine or pentostatin) as you had before – you have it on its own or combined with rituximab; the other chemotherapy drug (cladribine or pentostatin), usually combined with rituximab; clinical trials looking at different targeted drugs. […] Most people respond well to treatment and have another remission. But there are other treatment options if you relapse again. These include: a different chemotherapy (such a bendamustine) combined with rituximab; clinical trials looking at different targeted drugs. […] Other treatments you might have for hairy cell leukaemia include: surgery to remove your spleen; supportive treatments such as antibiotics, blood products or steroids. […] Your doctor might ask if youd like to take part in a clinical trial. Doctors and researchers do trials to make existing treatments better and develop new treatments.

• #28 Hairy Cell Leukemia Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.hairy-cell-leukemia-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062926

  Pentostatin given IV every other week for 3 to 6 months produced a 50% to 76% complete response rate and an 80% to 87% overall response rate. […] Ibrutinib, a tyrosine kinase inhibitor, has been studied in the treatment of hairy cell leukemia. […] Patients with hairy cell leukemia who have a relapse after the first course of cladribine or pentostatin often respond well to re-treatment with the same or another purine analogue, especially if relapse occurs after several years. […] Splenectomy plays a decreasing role in treating hairy cell leukemia because effective alternatives are available.

• #29 Hairy Cell Leukemia Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq

  Treatment options for hairy cell leukemia include: Watchful waiting, if feasible. Cladribine with or without rituximab. BRAF inhibitors (vemurafenib or dabrafenib) with or without rituximab or trametinib. Rituximab. Pentostatin. Ibrutinib. Re-treatment with cladribine or pentostatin. Bendamustine with rituximab. Splenectomy. Interferon. […] Patients with hairy cell leukemia who have a relapse after the first course of cladribine or pentostatin often respond well to re-treatment with the same or another purine analogue, especially if relapse occurs after several years. […] Splenectomy plays a decreasing role in treating hairy cell leukemia because effective alternatives are available. Splenectomy will partially or completely normalize the peripheral blood in most patients with hairy cell leukemia. After a splenectomy, there is usually little or no change in the bone marrow, and virtually all patients have progressive disease within 12 to 18 months.

• #30 Hairy cell leukaemia | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma/hairy-cell-leukaemia

  The treatment you have depends on how long your remission lasted. […] If you experience a relapse more than 2 years after your first treatment, you are likely to have the same treatment as you had before. This may be combined with an antibody therapy called rituximab. […] If you experience a relapse less than 2 years after your first treatment, your medical team might recommend: […] Occasionally, your medical team might recommend having an operation to remove your spleen (a splenectomy). This is usually considered only if your spleen is very enlarged and causing serious problems. […] If you do not have troubling symptoms, a period of active monitoring may be recommended. The most likely treatment for people with HCL-V is rituximab combined with chemotherapy (typically cladribine or pentostatin). Some people have surgery to remove their spleen (a splenectomy), if it is very enlarged. […] HCL-V can be more difficult to treat than hairy cell leukaemia. If your HCL-V is refractory (doesnt respond well to treatment) or relapses (returns after successful treatment) there are other treatment options, including targeted drugs as part of a clinical trial.

• #31 Hairy Cell Leukemia Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/200580-treatment

  The data regarding the use of either pentostatin or cladribine alone for hairy cell leukemia suggest that these agents are equally effective in terms of response rate and remission duration. […] Substantial improvement in overall survival has been reported with initial use of either agent. […] Although the best way to initiate therapy remains unsettled, there is a consensus that attaining a complete remission is important to reduce the possibility of a late relapse. […] The first-line therapy for HCL is cladribine, 0.1 mg/kg/d by continuous intravenous infusion for 7 days. […] With one course of therapy of cladribine, 80% of patients obtain a complete remission (CR), and the remainder obtain a partial remission (PR). […] For patients with relapsed HCL who have previously been treated with splenectomy, interferon, or pentostatin, retreatment with cladribine in the same manner is indicated, especially if their disease had previously responded to cladribine.

• #32 Hairy cell leukemia – Wikipedia

  https://en.wikipedia.org/wiki/Hairy_cell_leukemia

  Rituximab, the anti-CD20 monoclonal antibody, may be added to purine analogs during initial treatment and is associated with increasing the remission rate to nearly 100%. Purine analogs may be restarted in those with relapsed disease, with or without rituximab. […] If a patient is resistant to either cladribine or pentostatin, or in those with relapsed disease, then second-line therapy is pursued. […] BRAF inhibitors such as vemurafenib or dabrafenib inhibit the MAPK/ERK pathway which is constitutively active in those with a BRAF V600E kinase activating mutation (which is 95% of those with HCL). Constitutively active MAPK kinase activity is what leads to disease activity in HCL by increasing cell survival, increasing proliferation and reducing apoptosis of the leukemic cells. BRAF inhibitors have an overall response rate of greater than 90% with a complete response of 30-35% in people with relapsed or refractory HCL. They are also known to have less toxic effects than the purine analogs.

• #33 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia.com/en/onkopedia/guidelines/hairy-cell-leukemia

  The detection of the BRAF V600E mutation in almost all patients with classic HCL offers a new target for molecular targeted therapy. […] In the two largest phase II trials of vemurafenib in patients in relapse after purine analogs or in refractoriness, 96-100% achieved a hematologic remission. […] The HCL variant is biologically and clinically distinct from classic HCL. […] Response rates to purine analogs are approximately 50%. […] Other effective therapeutic options are BTK inhibitors. […] Splenectomy was the first effective therapy for HCL and resulted in hematologic remissions in up to 70% of patients.

• #34 Hairy cell leukemia and COVID-19 adaptation of treatment guidelines | Leukemia

  https://www.nature.com/articles/s41375-021-01257-7

  In these patients with the current extent of the pandemic, alternatives to nucleoside analogs are being considered: BRAF inhibitors. […] The use of a BRAF inhibitor as a bridge therapy for patients with active infection during this pandemic is therefore also worthy of consideration. […] IFN alpha also induces remissions in cHCL patients without the neutrophil nadir associated with nucleoside analogs. […] While treatment of patients in relapse was often approached with a plan to use a combination of a purine analog and an anti-CD20 monoclonal antibody, concerns regarding excessive immunosuppression during this pandemic have resulted in alternative strategies. […] BRAF inhibitors vemurafenib alone or with rituximab induce rapid responses without myelosuppression and with no or less immune suppression compared to chemo-immunotherapy.

• #35 Emerging Role of BRAF Inhibitors in Relapsed/Refractory Hairy Cell Leukemia Treatment

  https://www.pharmacytimes.com/view/emerging-role-of-braf-inhibitors-in-relapsed-refractory-hairy-cell-leukemia-treatment

  The discovery of BRAF V600E as a driver mutation in roughly 90% of HCL cases represents a landmark advancement in understanding the diseases pathogenesis. […] As a result, targeted therapy with BRAF inhibitors, such as vemurafenib and dabrafenib, has been incorporated into the National Comprehensive Cancer Network guidelines despite their lack of FDA approval for this disease state. […] Vemurafenib monotherapy in 36 patients with R/R HCL showed a 4-year OS rate of 82% and a median RFS of 19 months. […] The overall incidence of adverse effects (AEs) was similar between patients who received initial treatment vs those who received retreatment with vemurafenib. […] CR was achieved in 87% of patients treated with vemurafenib plus rituximab. […] The ORR was 80% for dabrafenib monotherapy, with a CR achieved in 30% of patients.

• #36 Emerging Role of BRAF Inhibitors in Relapsed/Refractory Hairy Cell Leukemia Treatment

  https://www.pharmacytimes.com/view/emerging-role-of-braf-inhibitors-in-relapsed-refractory-hairy-cell-leukemia-treatment

  The discovery of BRAF V600E as a driver mutation in roughly 90% of HCL cases represents a landmark advancement in understanding the diseases pathogenesis. […] As a result, targeted therapy with BRAF inhibitors, such as vemurafenib and dabrafenib, has been incorporated into the National Comprehensive Cancer Network guidelines despite their lack of FDA approval for this disease state. […] Vemurafenib monotherapy in 36 patients with R/R HCL showed a 4-year OS rate of 82% and a median RFS of 19 months. […] The overall incidence of adverse effects (AEs) was similar between patients who received initial treatment vs those who received retreatment with vemurafenib. […] CR was achieved in 87% of patients treated with vemurafenib plus rituximab. […] The ORR was 80% for dabrafenib monotherapy, with a CR achieved in 30% of patients.

• #37 Improving Treatment for Hairy Cell Leukemia – News Center

  https://news.feinberg.northwestern.edu/2023/01/11/improving-treatment-for-hairy-cell-leukemia/

  Patients with relapsed or refractory hairy cell leukemia who were treated with vemurafenib experienced excellent response and relapse-free survival, according to a recent clinical trial published in Blood. […] The results suggest that vemurafenib is an effective second-line treatment option for these patients. […] In the case of relapsed or refractory HCL, patients are prescribed vemurafenib, a small molecular inhibitor drug. […] The findings suggest vemurafenib monotherapy can achieve high response rates in patients with relapsed or refractory HCL with acceptable side effects, according to Tallman. […] A randomized trial comparing the current standard-of-care for untreated patients of Cladribine plus Rituximab versus Vemurafenib plus Rituximab would be a logical next step. A new standard of care may emerge, Tallman said.

• #38 Emerging Role of BRAF Inhibitors in Relapsed/Refractory Hairy Cell Leukemia Treatment

  https://www.pharmacytimes.com/view/emerging-role-of-braf-inhibitors-in-relapsed-refractory-hairy-cell-leukemia-treatment

  The primary end point of ORR was 89.1% in the HCL cohort treated with dabrafenib plus trametinib, with a CR achieved in 65.5% of patients. […] Future research should focus on optimizing treatment duration, managing resistance mechanisms, and identifying patients with HCL most likely to benefit from regimens incorporating BRAF inhibitors.

• #39 FDA approves new kind of treatment for hairy cell leukemia | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-new-kind-treatment-hairy-cell-leukemia

  The U.S. Food and Drug Administration today approved Lumoxiti (moxetumomab pasudotox-tdfk) injection for intravenous use for the treatment of adult patients with relapsed or refractory hairy cell leukemia (HCL) who have received at least two prior systemic therapies, including treatment with a purine nucleoside analog. […] Lumoxiti fills an unmet need for patients with hairy cell leukemia whose disease has progressed after trying other FDA-approved therapies, said Richard Pazdur, M.D., director of the FDAs Oncology Center of Excellence and acting director of the Office of Hematology and Oncology Products in the FDAs Center for Drug Evaluation and Research. […] The efficacy of Lumoxiti was studied in a single-arm, open-label clinical trial of 80 patients who had received prior treatment for HCL with at least two systemic therapies, including a purine nucleoside analog.

• #40 FDA approves new kind of treatment for hairy cell leukemia | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-new-kind-treatment-hairy-cell-leukemia

  Thirty percent of patients in the trial achieved durable CR, and the overall response rate (number of patients with partial or complete response to therapy) was 75 percent. […] Common side effects of Lumoxiti include infusion-related reactions, swelling caused by excess fluid in body tissue (edema), nausea, fatigue, headache, fever (pyrexia), constipation, anemia and diarrhea. […] The prescribing information for Lumoxiti includes a Boxed Warning to advise health care professionals and patients about the risk of developing capillary leak syndrome, a condition in which fluid and proteins leak out of tiny blood vessels into surrounding tissues.

• #41 Hairy Cell Leukemia Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/200580-treatment

  For patients with HCL that is refractory to cladribine, or if relapse occurs after two cycles of cladribine, the authors recommend treatment with pentostatin, 4 mg/m2 intravenously every 2 weeks for 3-6 months. […] Another option for relapsed or refractory HCL is moxetumomab pasudotox (Lumoxiti), an anti-CD22 recombinant immunotoxin approved by the US Food and Drug Administration (FDA) in 2018. […] National Comprehensive Cancer Network (NCCN) guidelines now include the off-label use of ibrutinib as a recommended treatment for progressive disease after relapse/refractory therapy.

• #42 Current and emerging treatment options for hairy cell leukemia | OTT

  https://www.dovepress.com/current-and-emerging-treatment-options-for-hairy-cell-leukemia-peer-reviewed-fulltext-article-OTT

  Recently, a subgroup of patients has been described that does not respond to first-line PA or subsequent treatments. […] HCL-v responds differently to standard HCL treatment, being generally resistant to interferon alpha and rarely achieving CR with either pentostatin or cladribine. […] Emerging treatment options in HCL […] Agents targeting these molecular pathways are under investigation, and some of them have demonstrated significant activity in relapsed patients. […] The BRAF (V600E) mutation is a molecular hallmark of HCL-c. […] Vemurafenib is an oral agent that inhibits the thymidine kinase enzyme and specifically targets cells containing BRAF (V600E) mutations. […] A Phase II clinical trial is currently evaluating the use of ibrutinib in patients with relapsed HCL.

• #43

  https://link.springer.com/article/10.1007/s11864-021-00927-z

  Hairy cell leukemia variant (HCL-V) is a rare B cell lymphoproliferative disorder with a clinical-pathological distinction from the classic form of hairy cell leukemia (HCL-C). HCL-V is more aggressive in nature, has a higher tendency to be refractory to conventional purine analog pharmacotherapies, and leads to a poorer prognosis. […] To date, combination chemoimmunotherapies, such as cladribine and rituximab, have shown the best results in HCL-V. Future directions include targeted therapies such as moxetumomab pasudotox, ibrutinib, trametinib, and binimetinib and potentially anti-CD22 chimeric antigen receptor T cell therapy. […] The presence of activating MAP2K1 mutations in patients with HCL-V (up to 50%) is a potential therapeutic target with MEK inhibitors. […] Bendamustine (70 mg/m2 on days 1 and 2) and rituximab (375 mg/m every 28 days for 4 cycles) combination led to CR in three elderly patients with TP53 unmutated HCL-V with manageable toxicity profiles.

• #44 Hairy Cell Leukemia Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq

  Treatment options for hairy cell leukemia include: Watchful waiting, if feasible. Cladribine with or without rituximab. BRAF inhibitors (vemurafenib or dabrafenib) with or without rituximab or trametinib. Rituximab. Pentostatin. Ibrutinib. Re-treatment with cladribine or pentostatin. Bendamustine with rituximab. Splenectomy. Interferon. […] Patients with hairy cell leukemia who have a relapse after the first course of cladribine or pentostatin often respond well to re-treatment with the same or another purine analogue, especially if relapse occurs after several years. […] Splenectomy plays a decreasing role in treating hairy cell leukemia because effective alternatives are available. Splenectomy will partially or completely normalize the peripheral blood in most patients with hairy cell leukemia. After a splenectomy, there is usually little or no change in the bone marrow, and virtually all patients have progressive disease within 12 to 18 months.

• #45 Hairy-Cell Leukemia — Onkopedia

  https://www.onkopedia-guidelines.info/en/onkopedia/guidelines/hairy-cell-leukemia

  Response rates to purine analogs are approximately 50%. […] The detection of the BRAF V600E mutation in almost all patients with classic HCL offers a new target for molecular targeted therapy. […] In the treatment of patients with early relapse, the combination of purine analogs with rituximab is a highly effective option. […] Splenectomy was the first effective therapy for HCL and resulted in hematologic remissions in up to 70% of patients. However, the recurrence rate is 90%. […] The goals of HCL therapy are symptomatic relief, hematologic remission, and normal life expectancy. Achieving complete hematologic remission is associated with longer progression-free survival, but overall survival is not affected.

• #46 Treating Hairy Cell Leukemia | American Cancer Society

  https://www.cancer.org/cancer/types/chronic-lymphocytic-leukemia/treating/hairy-cell-leukemia.html

  In rare cases where HCL doesn’t respond to chemo, or if the response to treatment doesn’t last long, other types of medicines might be tried. Targeted drugs known as BRAF inhibitors might be an option. This could include vemurafenib (sometimes with rituximab) or dabrafenib (plus trametinib). […] BTK inhibitors are another type of targeted drug that might also be an option, as might rituximab alone or peginterferon-alfa, a type of immunotherapy. […] If you are uncomfortable because of an enlarged spleen, surgery to remove your spleen (splenectomy) can often help relieve pain.

• #47 Hairy Cell Leukemia Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq

  Treatment options for hairy cell leukemia include: Watchful waiting, if feasible. Cladribine with or without rituximab. BRAF inhibitors (vemurafenib or dabrafenib) with or without rituximab or trametinib. Rituximab. Pentostatin. Ibrutinib. Re-treatment with cladribine or pentostatin. Bendamustine with rituximab. Splenectomy. Interferon. […] Patients with hairy cell leukemia who have a relapse after the first course of cladribine or pentostatin often respond well to re-treatment with the same or another purine analogue, especially if relapse occurs after several years. […] Splenectomy plays a decreasing role in treating hairy cell leukemia because effective alternatives are available. Splenectomy will partially or completely normalize the peripheral blood in most patients with hairy cell leukemia. After a splenectomy, there is usually little or no change in the bone marrow, and virtually all patients have progressive disease within 12 to 18 months.

• #48 Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group

  https://www.mdpi.com/2072-6694/16/12/2185

  In case of active infection, we recommend to delay the start of HCL treatment until infection is controlled. If delaying the start of treatment is not possible, the use of a bridging therapy with low-dose BRAFi is our recommendation due to the absence of myelotoxic and myelosuppressive effects. […] […] It has been demonstrated that BRAFi can rapidly improve the neutrophil count and thereby facilitate infectious disease control. Once infection is controlled, a switch to CDA + R is recommended. In the same way, in case of pandemic periods such as COVID-19, it is preferable to delay HCL treatment and, if it is not possible, to recommend BRAFi as a bridging therapy. […] […] In the rare case of HCL occurring during pregnancy, we recommend delaying treatment until after childbirth. If delaying HCL therapy is not possible, use of IFNα is preferable. Of note, there are no data on BRAFi during pregnancy, even if some case reports have been published in patients with melanoma. […]

• #49 Hairy Cell Leukemia Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/leukemia/hp/hairy-cell-treatment-pdq

  Interferon alfa is no longer available because production has been halted. According to the Hairy Cell Leukemia Foundation, ropeginterferon alfa-2b-njft is the best available preparation, but it is not FDA approved for hairy cell leukemia. Interferon is useful when treating hairy cell leukemia during pregnancy because it does not involve cytotoxic agents.

• #50 Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group

  https://www.mdpi.com/2072-6694/16/12/2185

  In case of active infection, we recommend to delay the start of HCL treatment until infection is controlled. If delaying the start of treatment is not possible, the use of a bridging therapy with low-dose BRAFi is our recommendation due to the absence of myelotoxic and myelosuppressive effects. […] […] It has been demonstrated that BRAFi can rapidly improve the neutrophil count and thereby facilitate infectious disease control. Once infection is controlled, a switch to CDA + R is recommended. In the same way, in case of pandemic periods such as COVID-19, it is preferable to delay HCL treatment and, if it is not possible, to recommend BRAFi as a bridging therapy. […] […] In the rare case of HCL occurring during pregnancy, we recommend delaying treatment until after childbirth. If delaying HCL therapy is not possible, use of IFNα is preferable. Of note, there are no data on BRAFi during pregnancy, even if some case reports have been published in patients with melanoma. […]

• #51 Hairy cell leukemia and COVID-19 adaptation of treatment guidelines | Leukemia

  https://www.nature.com/articles/s41375-021-01257-7

  At present, our best efforts have been directed to preventing exposure to COVID-19 while the development of effective strategies for immunization is being vigorously pursued. […] The 2017 consensus statement described criteria for treatment of cHCL. […] Since active cancer including advanced and progressing hematological malignancies are associated with increased mortality in patients with COVID-19, start of antineoplastic treatment should not be unnecessarily delayed in HCL patients. […] Considering the consequences of untreated progressive pancytopenia, initiation of effective therapy should not be delayed in the absence of ongoing infection. […] The combination of a BRAF inhibitor either with a monoclonal anti-CD20 antibody or a MEK inhibitor can further increase the rate of durable remissions without inducing severe myelosuppression.

• #52 Hairy Cell Leukemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499845/

  Relapsed disease can be treated with either another course of purine analog (if relapse is more than 1 year from initial treatment); however, response rates are often lower, and remissions shorter after relapse. Many other options for relapsed or refractory disease exist, including a combination of cladribine or pentostatin with rituximab, fludarabine alone or in combination with rituximab, bendamustine, and interferon-alpha. The BRAF mutation in hairy cell leukemia can be targeted by the oral BRAF inhibitor vemurafenib, which has been shown to be efficacious in both the relapsed and refractory settings. […] Supportive care measures such as antimicrobial prophylaxis for viral and pneumocystis pneumonia should be considered for those with significant cytopenias. Transfused blood products, if necessary, should be irradiated to prevent transfusion-associated graft-versus-host disease.

• #53 Hairy cell leukemia | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/chronic-lymphocytic-leukemia-cll/treatment/hairy-cell-leukemia

  Ibrutinib is a type of targeted therapy called a Bruton’s tyrosine kinase (BTK) inhibitor. […] Moxetumomab pasudotox targets the CD22 gene on cancer cells. […] Dabrafenib, which is a BRAF inhibitor, may be combined with another drug called trametinib, which is a MEK inhibitor. […] Venetoclax is a type of targeted therapy called a BCL2 inhibitor. […] HCL and its treatments can cause certain problems, including infection, bleeding and low blood cell counts. If you develop these problems, you may be given: antibiotics to prevent and treat infections, transfusions of red blood cells and platelets to raise low blood cell counts, growth factors such as filgrastim and pegfilgrastim. […] You may be asked if you want to join a clinical trial for HCL. Clinical trials look at new ways to prevent, find and treat cancer. […] Follow-up care lets your healthcare team keep track of your health for a period of time after treatment ends.

• #54 Hairy Cell Leukemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK499845/

  Interferon-alpha is the treatment of choice during pregnancy when treatment is warranted. It may be preferred for the initial treatment in patients with severe pancytopenia and/or active infection to improve blood counts and allow for subsequent therapy with purine analogs. However, patients with HCL who express the CD5 antigen appear to respond poorly to IFN-a. […] Splenectomy may be considered in patients with symptomatic massive splenomegaly, severe pancytopenia due to splenic sequestrations and as a temporizing measure in symptomatic pregnant women. […] Repeat bone marrow biopsy should be performed after treatment to confirm complete remission. Complete remission is defined as the absence of hairy cells in blood and bone marrow, resolution of splenomegaly, and recovery of peripheral blood counts (Hgb greater than 12, platelet greater than 100, ANC greater than 1500). Partial response is defined as normalization of peripheral blood counts along with a 50% decrease in splenomegaly, and less than 5% of circulating hairy cells remain. Currently, the clinical implications for minimal residual disease (MRD) are poorly defined. Many patients MRD will still have prolonged, complete hematologic remissions.

• #55 Understanding Hairy Cell Leukemia — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/hairy-cell-leukemia

  Usually within a few weeks of receiving treatment, the blood counts begin to recover and will often return to completely normal levels. However, to assess response to anti-HCL treatment, a bone marrow examination is necessary. […] About 40% of patients will relapse, even 10 years or more after initial therapy. Regular monitoring by a hematologist takes place, usually once or twice a year for patients in stable remission.

• #56 ClinicalTrials — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/clinicaltrials

  Clinical trials are carefully controlled research studies, and are necessary to develop and study ways to improve treatment and quality of life for patients with hairy cell leukemia. […] The researchers are doing this study to compare the safety of vemurafenib in combination with obinutuzumab to the standard of approach of cladribine in combination with rituximab. […] The current standard-of-care for HCL involves chemotherapy, with agents such as cladribine or pentostatin. Chemotherapy is associated with infection, low blood counts and predisposition to future cancers. This study tests a new yet previously validated drug combination for the treatment of hairy cell leukemia. […] To determine the overall response rate (ORR) to binimetinib, in patients with BRAF WT HCL and HCLv. […] To assess the safety and feasibility of administering escalating doses of autologous anti-CD22-CAR (M971BBz) engineered T-cells in subjects with HCL/HCLv following a cyclophosphamide/fludarabine lymphodepletion regimen.

• #57 ClinicalTrials — Hairy Cell Leukemia Foundation

  https://www.hairycellleukemia.org/clinicaltrials

  To test if treatment with a combination of encorafenib and binimetinib in BRAF mutant HCL is more effective than treatment with vemurafenib. […] Purpose: To test the safety of Moxetumomab pasudotox (Lumoxiti) taken with Rituximab for people with HCL or HCL variant, both agents already approved for HCL or commonly used for HCL. […] To this end, we are conducting a phase II clinical trial combining the BRAF inhibitor, vemurafenib, and anti-CD20 antibody, obinutuzumab, in patients with previously untreated HCL to achieve a deeper molecular response with more favorable side effect profile and durable remissions. […] Determine the response rate of HCL after 32 weeks of single-agent ibrutinib treatment.

• #58 Chemotherapy-Free Treatment of Classical Hairy Cell Leukemialogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na56612/2023/10/12/chemotherapy-free-treatment-classical-hairy-cell-leukemia

  In a phase 2 study, 90% of patients achieved complete remission with frontline obinutuzumab plus the BRAF inhibitor vemurafenib. […] Investigators have now conducted an industry- and grant-funded, multicenter, phase 2 trial of vemurafenib for previously untreated classical HCL patients with cytopenias. Patients received vemurafenib (960 mg orally twice daily for four 28-day cycles) plus obinutuzumab (1000 mg intravenously on days 1, 8, and 15 of cycle 2 and on day 1 of cycles 3 and 4). […] Among 30 patients, the 27 (90%) who completed four treatment cycles achieved complete remission; the remaining three discontinued treatment early due to rash, pneumonia, or verrucous hyperplasia. […] The current study of a chemotherapy-free regimen shows durable remissions in all fully treated patients without the T-cell depletion that occurs with nucleoside analogues. Further study of this regimen is warranted using lower-dose vemurafenib, which may be sufficient to maintain the high response rate while mitigating rash and arthralgias.

• #59 Chemotherapy-Free Treatment of Classical Hairy Cell Leukemialogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na56612/2023/10/12/chemotherapy-free-treatment-classical-hairy-cell-leukemia

  In a phase 2 study, 90% of patients achieved complete remission with frontline obinutuzumab plus the BRAF inhibitor vemurafenib. […] Investigators have now conducted an industry- and grant-funded, multicenter, phase 2 trial of vemurafenib for previously untreated classical HCL patients with cytopenias. Patients received vemurafenib (960 mg orally twice daily for four 28-day cycles) plus obinutuzumab (1000 mg intravenously on days 1, 8, and 15 of cycle 2 and on day 1 of cycles 3 and 4). […] Among 30 patients, the 27 (90%) who completed four treatment cycles achieved complete remission; the remaining three discontinued treatment early due to rash, pneumonia, or verrucous hyperplasia. […] The current study of a chemotherapy-free regimen shows durable remissions in all fully treated patients without the T-cell depletion that occurs with nucleoside analogues. Further study of this regimen is warranted using lower-dose vemurafenib, which may be sufficient to maintain the high response rate while mitigating rash and arthralgias.

• #60 Treatment for Hairy Cell Leukemia | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/leukemias/treatment/treatment-hairy-cell-leukemia

  Cancer-targeted antibody therapy is another approved form of anticancer therapy sometimes used to treat HCL. One example is rituximab (Rituxan). Rituximab may be used by itself or with other chemotherapy drugs, such as cladribine. […] In very rare cases of HCL, removing the spleen (splenectomy) may be considered. […] MSK is leading a clinical trial investigating a chemotherapy-free drug combination for people with HCL who are receiving treatment for the first time. This trial is studying the combination of two therapies: obinutuzumab (Gazyva) and a BRAF-targeted therapy called vemurafenib (Zelboraf). This approach does not include standard chemotherapy. People who receive it may experience fewer side effects than those who receive initial treatment with chemotherapy drugs. […] Among the therapies tested at MSK is a targeted therapy called vemurafenib (Zelboraf). Vemurafenib is an oral drug that can bring about remission (when no trace of cancer is detectable) or slow the growth of HCL in people with a BRAF mutation. MSK led the largest clinical trial of vemurafenib in the United States. For the first time, vemurafenib was shown to be highly effective in people with HCL that has been resistant to or has come back after standard treatments.

• #61 Hairy cell leukemia: Outlook, treatment, and what to expect

  https://www.medicalnewstoday.com/articles/hairy-cell-leukemia-prognosis

  Hairy cell leukemia is a rare type of blood cancer that can affect adults. In people who receive treatment, the long-term outlook for hairy cell leukemia is good. […] HCL is a chronic disease, and although there is no cure for it, the condition is treatable. Treatment is usually highly effective and can help people continue to live normal lives. […] The Leukemia and Lymphoma Society reports that the 5-year event-free survival rate for HCL is 90% in people who received initial treatment with the chemotherapy drug cladribine. This means 90% of people will still be alive 5 years after diagnosis. […] Treatment with cladribine has led to roughly 85% complete remission and around 10% partial response in people with HCL. […] HCL responds very well to treatment, which aims to manage the cancer rather than cure it.

• #62 Hairy cell leukemia: Outlook, treatment, and what to expect

  https://www.medicalnewstoday.com/articles/hairy-cell-leukemia-prognosis

  Hairy cell leukemia is a rare type of blood cancer that can affect adults. In people who receive treatment, the long-term outlook for hairy cell leukemia is good. […] HCL is a chronic disease, and although there is no cure for it, the condition is treatable. Treatment is usually highly effective and can help people continue to live normal lives. […] The Leukemia and Lymphoma Society reports that the 5-year event-free survival rate for HCL is 90% in people who received initial treatment with the chemotherapy drug cladribine. This means 90% of people will still be alive 5 years after diagnosis. […] Treatment with cladribine has led to roughly 85% complete remission and around 10% partial response in people with HCL. […] HCL responds very well to treatment, which aims to manage the cancer rather than cure it.

• #63 https://www.lls.org/leukemia/hairy-cell-leukemia

  https://www.lls.org/leukemia/hairy-cell-leukemia

  The drug cladribine is the initial treatment for most people who have hairy cell leukemia. […] The 5-year event-free survival rate after treatment is approximately 90 percent of patients initially treated with cladribine. The advent of cladribine therapy has resulted in approximately an 85 percent rate of complete remission and approximately 10 percent rate of partial response.

• #64 Long-term follow-up of cladribine treatment in hairy cell leukemia: 30-year experience in a multicentric Italian study | Blood Cancer Journal

  https://www.nature.com/articles/s41408-022-00702-9

  Hairy cell leukemia (HCL) is a rare lymphoproliferative disease with an excellent prognosis after treatment with cladribine (2CDA), although relapse may occur during follow-up. […] A total of 330 (64.3%) patients received 2CDA intravenously and 183 (35.7%) subcutaneously. […] ORR was 91.8%: CR was obtained in 335 patients (65.3%), PR in 96 (18.7%), and hematological response in 40 (7.8%) patients; in 42 (8.2%) no response was observed. […] At a median follow-up of 6.83 years (range 0.0428.52), the median time to relapse was 12.2 years. […] A significant difference in duration of response was identified between patients that obtained a CR and PR (19.4 years versus 4.8 years, p0.0001). […] Following treatment of HCL with 2CDA, 80% of patients are estimated to be alive 15 years after diagnosis.

• #65 Long-term follow-up of cladribine treatment in hairy cell leukemia: 30-year experience in a multicentric Italian study | Blood Cancer Journal

  https://www.nature.com/articles/s41408-022-00702-9

  Hairy cell leukemia (HCL) is a rare lymphoproliferative disease with an excellent prognosis after treatment with cladribine (2CDA), although relapse may occur during follow-up. […] A total of 330 (64.3%) patients received 2CDA intravenously and 183 (35.7%) subcutaneously. […] ORR was 91.8%: CR was obtained in 335 patients (65.3%), PR in 96 (18.7%), and hematological response in 40 (7.8%) patients; in 42 (8.2%) no response was observed. […] At a median follow-up of 6.83 years (range 0.0428.52), the median time to relapse was 12.2 years. […] A significant difference in duration of response was identified between patients that obtained a CR and PR (19.4 years versus 4.8 years, p0.0001). […] Following treatment of HCL with 2CDA, 80% of patients are estimated to be alive 15 years after diagnosis.

• #66 Hairy Cell Leukemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23177-hairy-cell-leukemia

  Theres no cure for hairy cell leukemia. Research shows treatment, including chemotherapy, can put this condition into remission for 10 years or more. Remission means you dont have symptoms anymore and tests dont find any signs of the condition. One analysis of peoples experience with hairy cell leukemia suggests that, after treatment, theyll live as long as people who dont have the condition. […] That said, hairy cell leukemia can come back after several years of initial treatment. But there are treatments to put the disease back into remission.

• #67 Hairy Cell Leukemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23177-hairy-cell-leukemia

  Theres no cure for hairy cell leukemia. Research shows treatment, including chemotherapy, can put this condition into remission for 10 years or more. Remission means you dont have symptoms anymore and tests dont find any signs of the condition. One analysis of peoples experience with hairy cell leukemia suggests that, after treatment, theyll live as long as people who dont have the condition. […] That said, hairy cell leukemia can come back after several years of initial treatment. But there are treatments to put the disease back into remission.

• #68 Side effects of hairy cell leukaemia treatment – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/leukaemia/hairy-cell-leukaemia/treatment-side-effects/

  Side effects of hairy cell leukaemia treatment. All treatments can cause side effects. However, the type and severity will vary between individuals, depending on the type of treatment used and how an individual responds to it. In general, more intensive treatment is associated with more severe side effects. It is important to report any symptoms you are having to your doctor or nurse. In most cases they can be treated and are reversible. […] What are the side effects of hairy cell leukaemia treatment? Chemotherapy kills cells that multiply quickly, such as leukaemic cells. It also causes damage to fast-growing normal cells, including hair cells and cells that make up the tissues in your mouth, gut and bone marrow. The side effects of chemotherapy occur as a result of this damage. […] Hair loss is unfortunately a very common side effect of chemotherapy and some forms of radiotherapy. However, it is usually only temporary. The hair starts to fall out within a couple of weeks of treatment and tends to grow back three to six months later.

• #69 Side effects of hairy cell leukaemia treatment – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/leukaemia/hairy-cell-leukaemia/treatment-side-effects/

  Side effects of hairy cell leukaemia treatment. All treatments can cause side effects. However, the type and severity will vary between individuals, depending on the type of treatment used and how an individual responds to it. In general, more intensive treatment is associated with more severe side effects. It is important to report any symptoms you are having to your doctor or nurse. In most cases they can be treated and are reversible. […] What are the side effects of hairy cell leukaemia treatment? Chemotherapy kills cells that multiply quickly, such as leukaemic cells. It also causes damage to fast-growing normal cells, including hair cells and cells that make up the tissues in your mouth, gut and bone marrow. The side effects of chemotherapy occur as a result of this damage. […] Hair loss is unfortunately a very common side effect of chemotherapy and some forms of radiotherapy. However, it is usually only temporary. The hair starts to fall out within a couple of weeks of treatment and tends to grow back three to six months later.

• #70 Hairy cell leukaemia cladribine

  https://www.eviq.org.au/haematology-and-bmt/leukaemias/hairy-cell-leukaemia/364-hairy-cell-leukaemia-cladribine

  Cladribine produces profound and prolonged immunosuppression due to reduction of CD4 and CD8 T-lymphocytes. An increased incidence of opportunistic infections is expected during and six months following therapy with cladribine. […] The main toxicities are haematological and infectious. In the Zenhausern et al. study (5 days of IV cladribine), 90% developed grade 3-4 neutropenia. 44% developed any grade of infection. 38% required hospitalisation at some point in their therapy. 22% required packed cell transfusion while 8% required platelets. […] The Hairy Cell Leukaemia Foundation and the British Society for Haematology have both developed guidelines for the diagnosis and management of patients with hairy cell leukaemia (HCL). Various methods of administration of cladribine in HCL have been investigated. These include a single course of a seven day continuous intravenous (IV) infusion of 0.1mg/kg/day, IV infusion of 0.14 mg/kg/day over 2 hours for five days, weekly 2 hour IV infusion of 0.15 mg/kg for six weeks and subcutaneous (SC) bolus administration of 0.085 mg/kg/day for seven days. […] Each is considered to be a reasonable option in the above guidelines.

• #71 Emerging Role of BRAF Inhibitors in Relapsed/Refractory Hairy Cell Leukemia Treatment

  https://www.pharmacytimes.com/view/emerging-role-of-braf-inhibitors-in-relapsed-refractory-hairy-cell-leukemia-treatment

  The discovery of BRAF V600E as a driver mutation in roughly 90% of HCL cases represents a landmark advancement in understanding the diseases pathogenesis. […] As a result, targeted therapy with BRAF inhibitors, such as vemurafenib and dabrafenib, has been incorporated into the National Comprehensive Cancer Network guidelines despite their lack of FDA approval for this disease state. […] Vemurafenib monotherapy in 36 patients with R/R HCL showed a 4-year OS rate of 82% and a median RFS of 19 months. […] The overall incidence of adverse effects (AEs) was similar between patients who received initial treatment vs those who received retreatment with vemurafenib. […] CR was achieved in 87% of patients treated with vemurafenib plus rituximab. […] The ORR was 80% for dabrafenib monotherapy, with a CR achieved in 30% of patients.

• #72 Hairy cell leukemia – Wikipedia

  https://en.wikipedia.org/wiki/Hairy_cell_leukemia

  Rituximab may cause a serum sickness reaction which presents with fevers, joint pain and rash approximately 4-10 days after any infusion. The reaction is successfully treated with steroids. […] Interferon-alpha is an immune system hormone that is very helpful to a relatively small number of patients, and somewhat helpful to most patients. In about 65% of patients, the drug helps stabilize the disease or produce a slow, minor improvement for a partial response. […] Splenectomy (once commonly used in the treatment of HCL) has been replaced by purine analogs and other first line therapies, which are associated with greater response rates and a longer response. But splenectomy may still be done when other therapies fail and there is a large disease burden (clonal B cells) in the spleen. […] Hematopoietic stem cell transplantation for HCL does not improve relapse free survival at 5 years and is associated with a high mortality rate above 15% and is not recommended.

• #73 FDA approves new kind of treatment for hairy cell leukemia | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-new-kind-treatment-hairy-cell-leukemia

  Thirty percent of patients in the trial achieved durable CR, and the overall response rate (number of patients with partial or complete response to therapy) was 75 percent. […] Common side effects of Lumoxiti include infusion-related reactions, swelling caused by excess fluid in body tissue (edema), nausea, fatigue, headache, fever (pyrexia), constipation, anemia and diarrhea. […] The prescribing information for Lumoxiti includes a Boxed Warning to advise health care professionals and patients about the risk of developing capillary leak syndrome, a condition in which fluid and proteins leak out of tiny blood vessels into surrounding tissues.

• #74 Side effects of hairy cell leukaemia treatment – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/leukaemia/hairy-cell-leukaemia/treatment-side-effects/

  Some types of chemotherapy and radiotherapy may cause a temporary or permanent reduction in your fertility. It is very important that you discuss any questions or concerns you might have regarding your future fertility with your doctor, if possible before you commence treatment. […] Some cancer treatments can affect the normal functioning of the ovaries. This can sometimes lead to infertility and an earlier than expected onset of menopause, even at a young age. The onset of menopause in these circumstances can be sudden and, understandably, very distressing.

• #75 Side effects of hairy cell leukaemia treatment – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/leukaemia/hairy-cell-leukaemia/treatment-side-effects/

  Side effects of hairy cell leukaemia treatment. All treatments can cause side effects. However, the type and severity will vary between individuals, depending on the type of treatment used and how an individual responds to it. In general, more intensive treatment is associated with more severe side effects. It is important to report any symptoms you are having to your doctor or nurse. In most cases they can be treated and are reversible. […] What are the side effects of hairy cell leukaemia treatment? Chemotherapy kills cells that multiply quickly, such as leukaemic cells. It also causes damage to fast-growing normal cells, including hair cells and cells that make up the tissues in your mouth, gut and bone marrow. The side effects of chemotherapy occur as a result of this damage. […] Hair loss is unfortunately a very common side effect of chemotherapy and some forms of radiotherapy. However, it is usually only temporary. The hair starts to fall out within a couple of weeks of treatment and tends to grow back three to six months later.

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