Materiały źródłowe

• #1 Treatment Options for Cutaneous B-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/treatment-options-cutaneous-b-cell-lymphoma

  The treatment for cutaneous B-cell lymphoma (CBCL) is based on the histology (what the cells look like under the microscope), where the lesions are located on the body, and the size and number of lesions. Depending on these factors, the types of therapy chosen can vary from a watch and wait approach, skin directed therapy, or even in some cases systemic therapy. […] When there are more widespread lesions or for primary cutaneous diffuse large B-cell lymphomas, systemic (internal) therapies such as rituximab or combination chemotherapy may be used. […] For patients with primary cutaneous follicle center lymphoma or primary cutaneous marginal zone lymphoma who are not experiencing symptoms, treatment can be deferred. This approach is called watchful waiting or watch and wait. […] If only one or a few skin lesions are present, surgery can be performed to remove the lesions. Surgical removal of lesions is usually very effective.

• #1 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Primary cutaneous B-cell lymphomas (pCBCL) include an infrequent group of non-Hodgkin lymphomas that are limited to skin sites at the time of diagnosis. They comprise roughly 2025% of all cutaneous lymphomas and are subdivided into primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large cell B cell lymphoma, leg type (PCDLCBCL, LT). The first two show a rather indolent course while PCDLCBCL, LT carries a worse prognosis. […] For solitary, single-site PCMZL and PCFCL, several topical treatment options exist. They include, but are not limited to, excision, radiotherapy, and intralesional therapies, discussed in this review. However, in selected cases, even watchful waiting is reasonable. […] Indolent types of pCBCL rarely require systemic treatment. However, in extended cases and more importantly DLCBCL, LT, systemic treatment is the first choice. Monoclonal anti-CD20-antibody rituximab is often used as monotherapy in PCMZL and PCFCL or combined with chemotherapy in PCDLBCL, LT. Newer options are monoclonal anti-CD40 antibody dacetuzumab, anti-PD-1 and anti-PD-L1 checkpoint inhibitors, and Bruton tyrosine kinase inhibitors.

• #1 Cutaneous B-cell Lymphoma: Treatment Options – LRF

  https://www.lymphoma.org/understanding-lymphoma/aboutlymphoma/nhl/cbcl/cbcltreatment/

  First-line treatment options for cutaneous B-cell lymphoma depend on the type of CBCL, the patients symptoms and overall health, and the stage of the disease. […] Treatment options for primary cutaneous follicle center lymphoma and cutaneous marginal zone B-cell lymphoma, depending on the number of lesions present, include: […] Observation without active treatment which is known as active surveillance (also known as watch and wait or watchful waiting). During this period, regular follow-up with your healthcare team is required. […] Radiation Therapy […] Surgery […] There is no known standard treatment strategy for the more aggressive types of CBCL, such as cutaneous diffuse large B-cell lymphoma, leg type. For single tumors, radiation with or without chemotherapy is recommended. […] Similar to other B-cell NHLs, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with or without radiation therapy is commonly used to treat CBCL that has spread and become a more generalized lymphoma. […] Clinical trials are also often recommended. Although all of these treatments usually result in disease remission, relapse is common.

• #1 Treatment Options for Cutaneous B-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/treatment-options-cutaneous-b-cell-lymphoma

  Examples of topical therapies used to treat CBCL include topical corticosteroids, imiquimod, topical nitrogen mustard and bexarotene. […] Radiation therapy is also highly effective, with most patients having a complete response (100% clearance of skin lesions) in the area treated. […] When indolent CBCL is present, a small amount of a drug usually corticosteroid, interferon, or the antibody drug rituximab (Rituxan) can be injected directly into the lesion. […] Systemic (internal) therapies for CBCL may be used when there are widespread lesions, when there is spread outside the skin, when skin directed therapy isn’t feasible for cosmetic reasons, or in the case of primary cutaneous diffuse large-B cell lymphoma. […] The most common systemic therapies used for CBCLs are rituximab, combination chemotherapies such as CHOP, and combinations of rituximab and chemotherapy (R-CHOP). […] Most patients with CBCL will respond to chemotherapy, but the rates of relapse are high.

• #1 Skin (cutaneous) B-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-b-cell-lymphoma

  If you have lymphoma in several areas of your skin and you are experiencing symptoms, you might have a chemotherapy drug called chlorambucil or an antibody treatment called rituximab. This can be given through a drip into a vein (intravenously) or as an injection directly into the affected skin. […] If your lymphoma has spread to your lymph nodes or other parts of your body, you might have rituximab plus a chemotherapy regimen (combination of treatment) such as bendamustine or CVP (cyclophosphamide, vincristine and prednisolone). […] Primary cutaneous diffuse large B-cell lymphoma, leg type, is usually treated with chemotherapy, often followed by radiotherapy to the affected area. The chemotherapy regimen (combination of treatment) most commonly used is called CHOP and is usually given alongside an antibody treatment called rituximab (R-CHOP).

• #1 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Lenalidomide is an immunomodulatory drug used in multiple myeloma. […] Venetoclax shows remarkable clinical activity in chronic lymphocytic leukemia; however, despite high-level expression of BCL-2 in systemic DLBCL, responses in this malignancy are seldom. […] Localized pCBCL (PCMZL, PCFCL) shows high 5-year disease-specific survival rates (99 vs. 95%, respectively); hence, an active wait and see approach is often applicable. […] Systemic treatments almost exclusively play a role in PCDLBCL, LT, and systemic (extracutaneous) lymphoma. […] For topical regimens, there is a new approach with the intralesional application of T-VEC, currently investigated in a phase I trial. […] The field of systemic treatment is getting more interesting with a growing number of monoclonal antibodies, checkpoint inhibitors (PD1), Bruton kinase inhibitors and other small molecules.

• #1 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Photodynamic therapy (PDT) is the combination of a photosensitizer, e.g., 5-aminolevulinic acid (5-ALA, or its methyl ester), light, and oxygen. […] Rituximab is the first chimeric monoclonal antibody (mAb) that showed positive results in systemic B cell malignancies and also cutaneous B-cell lymphomas. […] In advanced pCBCL and nearly all DLCBCL, LT cases, a combination of immunochemotherapy with R-CHOP is the first-line treatment. […] A new generation of higher affinity, lower immunogenicity, and increased efficacy anti-CD20 antibodies are being investigated. […] Dacetuzumab (SNG-40) is a humanized IgG1 anti-CD40-Mab. […] Immunotherapies are playing an increasing role in modern cancer therapy. […] Ibrutinib is a first-in-class irreversible BTK inhibitor, which binds to the kinase by a covalent bond and inhibits continuous signaling, thus inhibiting cell proliferation.

• #1 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  To increase the efficacy of immunotherapy, antibodies have been combined with radioisotopes. […] Recently rituximab has also been combined with lenalidomide. […] Other immunotherapeutic approaches include intralesional adenovirus-interferon gamma (TG1042), a non-replicating human adenovirus vector allowing the intracellular transfer of human interferon (IFN)-gamma cDNA. […] Topical imiquimod monotherapy has been recently studied in CBCL in a retrospective monocentric cohort in the United States. […] PCDLBCL, LT is an aggressive lymphoma affecting elderly people and its management is thus challenging. […] Currently, first line treatment is rituximab and combination chemotherapy, most commonly cyclophosphamide, doxorubicine, vincristine and prednisone (CHOP)-like regimens if the general condition of the patient allows it.

• #1 Cutaneous B-cell lymphoma

  https://dermnetnz.org/topics/cutaneous-b-cell-lymphoma

  The rarity of PCBCL and lack of comparative prospective, randomised studies limits the choice of therapy as most treatments are based on data from small retrospective studies. The European Organisation for Research and Treatment of Cancer Cutaneous Lymphoma Group (EORTC-CLG) and the International Society for Cutaneous Lymphoma (ISCL) have uniform recommendations on treating the three main types of PCBCL. Treatment is also influenced by whether the lesion is single or limited to a single site. […] Without any treatment, PCFCL lesions may be stable, gradually enlarge, or rarely, regress. The histological growth pattern does not influence survival or treatment choice. […] If PCFCL presents with a solitary lesion or is limited to a single site, treatment involves radiation therapy or surgery. Skin recurrences are common and often are outside the treated site but these do not affect prognosis.

• #1 Primary Cutaneous B-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/primary-cutaneous-b-cell-lymphoma

  Primary cutaneous follicle center lymphoma and cutaneous marginal zone lymphoma are among the most slow growing or indolent lymphomas: […] Depending on the number of lesions present, solitary or few lesions may be treated locally with either radiation or surgery. […] Topical steroid cream, topical chemotherapy, or injections may also be used. […] Sometimes no treatment is needed. Most often these slow-growing lymphomas recur over time, usually only on the skin. As many as 50 percent of people with single lesions that get radiation or excision will never get another lesion. […] Numerous and highly bothersome lesions may be eligible for treatment with rituximab. […] Primary cutaneous diffuse large-B cell lymphoma-leg type, often require treatment chemotherapy, radiation, or a combination of both.

• #1

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  Intralesional triamcinolone, interferon-, and rituximab are non US Food and Drug Administration (FDA)-approved treatments that are considered second-line therapy in patients with solitary lesions, or first-line therapy in patients with multiple localized lesions. […] The efficacy of these therapies is confined to case series and reports, with reported 44% complete response (CR) rates to intralesional triamcinolone, 100% to intralesional interferon-alpha, and 71-89% to intralesional rituximab. […] Treatment of multiple lesions include systemic treatments such as intravenous rituximab or oral chlorambucil, or multi-site local RT. […] There are no randomized trials to compare these treatments. […] As an aggressive lymphoma primarily affecting the elderly population, management of PCDLBCL, LT proves challenging.

• #1 Cutaneous B-cell Lymphoma: Treatment Options – LRF

  https://lymphoma.org/understanding-lymphoma/aboutlymphoma/nhl/cbcl/cbcltreatment/

  First-line treatment options for cutaneous B-cell lymphoma depend on the type of CBCL, the patients symptoms and overall health, and the stage of the disease. […] Treatment options for primary cutaneous follicle center lymphoma and cutaneous marginal zone B-cell lymphoma, depending on the number of lesions present, include: Observation without active treatment which is known as active surveillance (also known as watch and wait or watchful waiting). During this period, regular follow-up with your healthcare team is required. Radiation Therapy. Surgery. […] There is no known standard treatment strategy for the more aggressive types of CBCL, such as cutaneous diffuse large B-cell lymphoma, leg type. For single tumors, radiation with or without chemotherapy is recommended. Similar to other B-cell NHLs, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with or without radiation therapy is commonly used to treat CBCL that has spread and become a more generalized lymphoma. Clinical trials are also often recommended. Although all of these treatments usually result in disease remission, relapse is common.

• #1 Cutaneous B-cell lymphoma

  https://dermnetnz.org/topics/cutaneous-b-cell-lymphoma

  Patient with multiple lesions can be observed or given radiation, topical agents, cryotherapy, intralesional steroids or systemic therapy. Where disseminated skin lesions or large tumours occur, chemotherapy may be required such as ie, CHOP or R-CHOP. […] Immune-based therapies like rituximab, interferon and imiquimod are being explored. […] The recommended therapies for PCMZL are similar to PCFCL. […] Radiotherapy is highly effective and potentially curative for solitary or few contiguous lesions, but the relapse rates of PCMZL can be higher than PCFCL. […] Primary surgical excision can be effective for solitary or localised PCMZL. […] Local treatments include topical corticosteroids, intralesional steroids, topical nitrogen mustard, intralesional rituximab and cryotherapy. […] Multifocal skin lesions are treated with chemotherapy agents such as chlorambucil, interferon-alpha and anti-CD20 antibody (rituximab).

• #1 Skin (cutaneous) B-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-b-cell-lymphoma

  B-cell skin lymphoma (also called cutaneous B-cell lymphoma or CBCL) is rare. Only around 100 people are diagnosed with B-cell skin lymphoma in the UK each year. […] If you have primary cutaneous marginal zone lymphoma and your blood tests show you have an infection with Borrelia burgdorferi, you have treatment with antibiotics first. This might be the only treatment you need. […] Otherwise, all types of low-grade B-cell skin lymphoma are treated in the same way. […] You might have low dose superficial radiotherapy on and around the margin of the lymphoma after diagnosis. In many cases this might be all the treatment you need. […] If only one, small area of skin is affected by lymphoma, you might have surgery to remove it. This may be followed by radiotherapy to prevent it coming back (relapsing).

• #1 Cutaneous B-cell lymphoma

  https://dermnetnz.org/topics/cutaneous-b-cell-lymphoma

  Rarely, disseminated skin lesions are treated with chemotherapy (eg, CHOP). […] Localised or solitary leg-type B-cell lymphoma is usually are treated with local radiotherapy alone or in conjunction with R-CHOP. […] Generalised PCDLBCL-LT can be treated with R-CHOP and/or local radiotherapy. Response rates are high but relapse rates are 58% Around 30% develop extracutaneous disease. […] Trials are assessing the efficacy of biological agents like ofatumumab, lumilixumab, dacetuzumab and intralesional TG1042 in the treatment of PCDLBCL-LT.

• #1 Unveiling Primary Cutaneous B-Cell Lymphomas: New Insights into Diagnosis and Treatment Strategies

  https://www.mdpi.com/2072-6694/17/7/1202

  PCDLBCL,LT has an aggressive clinical course; despite a high rate of initial CR rates following standard treatment, relapses occur in roughly 70% of cases. The 5-year survival rate ranges from 40% to 60%, influenced by factors such as patient age, extent of tumor dissemination, protein expression patterns (particularly loss of P16 and presence of MYC expression exceeding 40%, both associated with a poor prognosis), and underlying molecular characteristics, including mutations in B-cell receptor signaling genes such as CARD11 and CD79A/B. […] The standard first-line treatment for PCDLBCL,LT involves polychemotherapy with the R-CHOP regimen. The addition of ISRT has been shown to enhance local control and prolong PFS, with a median PFS of 58 months when combined with R-CHOP, compared to 14 months with R-CHOP alone.

• #1 Skin (cutaneous) B-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-b-cell-lymphoma

  If you are not well enough to have chemotherapy, you might have radiotherapy on its own to help control your symptoms. Some people have rituximab on its own. […] If the lymphoma comes back (relapses), your doctor will probably suggest more chemotherapy. […] There is more information on our page on treatment of skin lymphoma.

• #1 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  The prognosis may be further improved using age-adapted rituximab-polychemotherapy. […] PCDLBCL, LT have the phenotype and gene expression profile of ABC-type DLBCL and the ESMO guidelines recommend that they should be treated as other ABC-type DLBCLs. […] The discovery of a subset of PCDLBCL, LT with recurrent genetic alterations in PD-L1/PD-L2 suggests that the use of immune checkpoint inhibitors could be explored.

• #1

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  First-line treatment for a solitary lesion or a single site of involvement is surgical excision with curative intent or radiation therapy (RT). […] Both have response rates around 90%. […] For a few localized lesions, RT is a better treatment option. […] However, the side effects of RT therapy are common including acute toxicities (local erythema, pain, edema, pruritus, anesthesia, and immediate pigmentation) and long-term side effects (alopecia, pruritus, pigmentation changes, atrophy, and telangiectasia). […] While the recommended total dose for PCMZL ranges between 4 and 36 Gy, multiple recent studies recommend the use of low-dose RT (4 Gy) because of improved efficacy and decreased toxicities. […] Antibiotic treatment is indicated in cases positive for Borrelia burgdorferi infection.

• #1 Rituximab in the Treatment of Primary Cutaneous B-Cell Lymphoma: A Review | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-rituximab-in-treatment-primary-cutaneous-articulo-S1578219014001073

  In recent years, the use of intralesional rituximab has become widespread for FL and MZL. The aim is to make treatment more comfortable and accessible while minimizing side effects. […] The complete response rates with this treatment in indolent B-cell lymphomas are somewhat greater than those achieved with intravenous administration. […] The conclusion from these studies is that intralesional use of rituximab is becoming more widespread in selected patients with low-grade CBCL in view of the convenience, lower dose, and fewer side effects. […] Rituximab is an anti-CD20 antibody that is increasingly used in the treatment of CBCL. Good response rates are obtained in indolent B-cell lymphomas, FL, and MZL, for both the intravenous and intralesional route of administration, although recurrence is frequent. Rituximab is not the preferred treatment, but it should be considered in patients with multiple and/or recurrent lesions at visible sites where radiotherapy or surgery may leave sequelae or scars. Intralesional use has become widespread in recent years with similar outcomes to intravenous use, but with fewer side effects and a lower cost. More studies are needed to establish the usefulness and the optimal protocol in other B-cell cutaneous lymphomas.

• #1 Whole-Body Medications for Cutaneous T-Cell Lymphoma | NYU Langone Health

  https://nyulangone.org/conditions/cutaneous-t-cell-lymphoma/treatments/whole-body-medications-for-cutaneous-t-cell-lymphoma

  NYU Langone doctors may use the immunomodulator interferon, which is given by injection under the skin, to manage cutaneous T-cell lymphoma. […] Our doctors may prescribe the oral retinoid bexarotene to treat cutaneous T-cell lymphoma. […] The enzyme inhibitor romidepsin targets and blocks enzymes found at particularly high levels in cancerous T-cell lymphocytes, helping to slow or stop cancer growth. […] Methotrexate is a form of chemotherapy given to people with cutaneous T-cell lymphoma. […] Alemtuzumab and brentuximab vedotin are two monoclonal antibodies used to treat people with cutaneous T-cell lymphoma. […] Whole-body medications may cause several side effects, including fatigue; low blood cell levels; elevated blood levels of triglycerides, a form of fat found in the blood; nausea; and diarrhea. […] Our doctors are studying the use of several new targeted medications, which home in on cancer cells and spare healthy tissue, in the treatment of cutaneous T-cell lymphoma, as well as combinations of chemotherapy and radiation therapy.

• #1 Cutaneous B-Cell Lymphoma: Symptoms, Treatment, and Diagnosis | MyLymphomaTeam

  https://www.mylymphomateam.com/resources/cutaneous-b-cell-lymphoma

  While these treatments are often effective, tumors also return for many people. It is important to continue to have regular follow-up visits with your doctor after completing treatment. […] Clinical trials to test the safety and effectiveness of newly developed interventions for CBCL are ongoing and may provide access to experimental treatments for those living with cutaneous B-cell lymphoma.

• #1 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  Primary cutaneous B-cell lymphomas are rare entities that develop primarily in the skin. They constitute a heterogeneous group that represents around a quarter of primary cutaneous lymphomas. The broad spectrum of clinical presentations and the disease course marked by frequent relapses are diagnostic and therapeutic challenges. The classification of these diseases has been refined in recent years, which allows to better define their immunopathogenesis and specific management. In the present article, we review the main clinico-biological characteristics and the current therapeutic options of these three main subsets. Based on the recent therapeutic advances in nodal B-cell lymphomas, we focus on the development of novel treatment options applicable to primary cutaneous B-cell lymphomas, including targeted therapies, combination treatments and immunotherapeutic approaches, and cover basic, translational and clinical aspects aiming to improve the treatment of cutaneous B-cell lymphomas.

• #1

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  Primary cutaneous B-cell lymphomas (pCBCLs) represent 25-30% of all primary cutaneous lymphomas. […] To date, treatment recommendations for cutaneous B-cell lymphomas have been largely based on small retrospective studies and institutional experience. […] Recently, the pharmacotherapeutic landscape has expanded to include drugs that may modify the underlying disease pathology of pCBCLs, representing new therapeutic modalities for this rare group of diseases. […] Novel therapies used for other systemic B-cell lymphomas show promise for the treatment of pCBCLs and are being increasingly considered. […] These new therapies are divided into five main groups: monoclonal antibodies, immune checkpoint inhibitors, small-molecule inhibitors, bispecific T-cell engaging, and chimeric antigen receptor T cell.

• #1 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  Optimal management of PCBCL requires multi-disciplinary collaboration between dermatologists, hematologists, pathologists and radiation oncologists. Guidelines for the treatment of PCBCL have been published by the EORTC. […] In localized disease, first-line therapies are usually local radiation or surgical excision. […] Complete response (CR) rate is close to 100% with local radiation, although relapses and acute adverse events (AE) occur in almost half of the cases. […] Disseminated lesions may be treated with intravenous rituximab, an anti-CD20 monoclonal antibody. […] When lesions are difficult to treat by radiotherapy or surgery, such as the face and the scalp, treatment with intralesional rituximab has been considered. […] In disseminated disease, another option is subcutaneous interferon-alpha.

• #2 Cutaneous B-Cell Lymphoma: Types, Diagnosis, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22925-cutaneous-b-cell-lymphoma

  Cutaneous B-cell lymphoma (CBCL) is a rare type of cancer that starts in your skin. Treatment ranges from watch and wait to radiation, chemotherapy or surgical removal. […] Most cases of CBCL can be treated and cured, but the condition may come back (recur). […] Treatment for CBCL depends on the subtype and stage. Treatment options include: Active surveillance, or watch and wait, which involves monitoring the condition until treatment is necessary. Chemotherapy. Medications rubbed on your skin (topical) or injected, such as corticosteroids. Monoclonal antibodies such as rituximab. Radiation therapy. Surgical removal.

• #2 Cutaneous B-cell Lymphoma: Treatment Options – LRF

  https://lymphoma.org/understanding-lymphoma/aboutlymphoma/nhl/cbcl/cbcltreatment/

  First-line treatment options for cutaneous B-cell lymphoma depend on the type of CBCL, the patients symptoms and overall health, and the stage of the disease. […] Treatment options for primary cutaneous follicle center lymphoma and cutaneous marginal zone B-cell lymphoma, depending on the number of lesions present, include: Observation without active treatment which is known as active surveillance (also known as watch and wait or watchful waiting). During this period, regular follow-up with your healthcare team is required. Radiation Therapy. Surgery. […] There is no known standard treatment strategy for the more aggressive types of CBCL, such as cutaneous diffuse large B-cell lymphoma, leg type. For single tumors, radiation with or without chemotherapy is recommended. Similar to other B-cell NHLs, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with or without radiation therapy is commonly used to treat CBCL that has spread and become a more generalized lymphoma. Clinical trials are also often recommended. Although all of these treatments usually result in disease remission, relapse is common.

• #2 Skin (cutaneous) B-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-b-cell-lymphoma

  If just a few areas of your skin are affected, you usually have radiotherapy on its own. Surgery is not usually used for more than one site. […] Sometimes the lesions associated with low-grade B-cell skin lymphomas can resolve themselves spontaneously. […] If your lymphoma is more widespread, localised radiotherapy or surgery are generally not suitable. Treatment depends on how much of your skin is affected and whether the lymphoma has spread anywhere else. […] If you have lymphoma in several areas of your skin but you are not experiencing any troublesome symptoms, you might not need treatment straightaway. Instead, your doctor might monitor your progress. This is called active monitoring, or watch and wait. It does not mean there is no treatment for you, but that you would not benefit from starting treatment straightaway. This saves you experiencing side effects of unnecessary treatment. You have treatment when you need it.

• #2 Primary Cutaneous B-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/primary-cutaneous-b-cell-lymphoma

  Primary cutaneous follicle center lymphoma and cutaneous marginal zone lymphoma are among the most slow growing or indolent lymphomas: […] Depending on the number of lesions present, solitary or few lesions may be treated locally with either radiation or surgery. […] Topical steroid cream, topical chemotherapy, or injections may also be used. […] Sometimes no treatment is needed. Most often these slow-growing lymphomas recur over time, usually only on the skin. As many as 50 percent of people with single lesions that get radiation or excision will never get another lesion. […] Numerous and highly bothersome lesions may be eligible for treatment with rituximab. […] Primary cutaneous diffuse large-B cell lymphoma-leg type, often require treatment chemotherapy, radiation, or a combination of both.

• #2 Skin (cutaneous) B-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-b-cell-lymphoma

  If you are not well enough to have chemotherapy, you might have radiotherapy on its own to help control your symptoms. Some people have rituximab on its own. […] If the lymphoma comes back (relapses), your doctor will probably suggest more chemotherapy. […] There is more information on our page on treatment of skin lymphoma.

• #2 Treatment Options for Cutaneous B-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/treatment-options-cutaneous-b-cell-lymphoma

  Examples of topical therapies used to treat CBCL include topical corticosteroids, imiquimod, topical nitrogen mustard and bexarotene. […] Radiation therapy is also highly effective, with most patients having a complete response (100% clearance of skin lesions) in the area treated. […] When indolent CBCL is present, a small amount of a drug usually corticosteroid, interferon, or the antibody drug rituximab (Rituxan) can be injected directly into the lesion. […] Systemic (internal) therapies for CBCL may be used when there are widespread lesions, when there is spread outside the skin, when skin directed therapy isn’t feasible for cosmetic reasons, or in the case of primary cutaneous diffuse large-B cell lymphoma. […] The most common systemic therapies used for CBCLs are rituximab, combination chemotherapies such as CHOP, and combinations of rituximab and chemotherapy (R-CHOP). […] Most patients with CBCL will respond to chemotherapy, but the rates of relapse are high.

• #2 Unveiling Primary Cutaneous B-Cell Lymphomas: New Insights into Diagnosis and Treatment Strategies

  https://www.mdpi.com/2072-6694/17/7/1202

  In patients with multiple disseminated lesions, or in select cases (i.e., PCFCL with extensive scalp lesions, where IRST may lead to long-standing alopecia), intravenous rituximab (325 mg/m² weekly for 4–8 infusions) has proven effective, with reported 98% overall response rates (ORRs), 64% CRs, a median progression free survival (PFS) of 58 months, and a median time to next treatment (TTNT) of 60 months in the largest series of 25 cases of PCMZL/LPD. […] Chemotherapy for indolent/low-grade PCBCL has failed to show reduced relapse rates compared to less aggressive therapies and should only be considered as a last-resort option for patients with extensive cutaneous involvement, or extracutaneous spread, and in PCFCL involving the lower extremities, which carries an unfavorable prognosis.

• #2

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  Patients with generalized disease are treated as nodal diffuse large B-cell lymphoma with the current first-line treatment being rituximab plus combination chemotherapy with R-CHOP. […] Local therapy demonstrates good initial efficacy, but relapses are common. […] Thus, local RT and surgical excision may be used as palliative treatment or in combination with R-CHOP.

• #2 Treatments for skin lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/treatments-skin-lymphoma

  Topical treatments include: emollients (moisturising creams, lotions or ointments), topical steroids, topical chemotherapy, other topical treatments. […] Topical steroids are safe if used carefully. If you use them for a long time, particularly the more potent ones, you may get side effects such as thinning of the skin, dilated (widened) blood vessels or an acne-like rash. […] Potent or very potent topical steroids can be very effective at clearing patches of skin lymphoma after 4 to 6 weeks of use. The effect may last for weeks or months after a course of treatment. However, the lymphoma usually comes back eventually. […] Topical chemotherapy drugs kill cells that are dividing rapidly, such as cancer cells. In early-stage skin lymphomas some chemotherapy drugs can be applied directly to the skin as gels.

• #2 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Photodynamic therapy (PDT) is the combination of a photosensitizer, e.g., 5-aminolevulinic acid (5-ALA, or its methyl ester), light, and oxygen. […] Rituximab is the first chimeric monoclonal antibody (mAb) that showed positive results in systemic B cell malignancies and also cutaneous B-cell lymphomas. […] In advanced pCBCL and nearly all DLCBCL, LT cases, a combination of immunochemotherapy with R-CHOP is the first-line treatment. […] A new generation of higher affinity, lower immunogenicity, and increased efficacy anti-CD20 antibodies are being investigated. […] Dacetuzumab (SNG-40) is a humanized IgG1 anti-CD40-Mab. […] Immunotherapies are playing an increasing role in modern cancer therapy. […] Ibrutinib is a first-in-class irreversible BTK inhibitor, which binds to the kinase by a covalent bond and inhibits continuous signaling, thus inhibiting cell proliferation.

• #2 Treatments for skin lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/treatments-skin-lymphoma

  Chlormethine (also known as mechlorethamine or nitrogen mustard) is a topical chemotherapy that has been used to treat skin lymphoma for over 50 years. […] Fluorouracil (also known as Efudix) is a chemotherapy cream that is used to treat some types of skin cancer and pre-cancerous skin conditions. Studies suggest it may help some people with skin lymphoma. […] Retinoids are related to vitamin A. They disrupt the growth and division of lymphoma cells. Topical retinoids, such as adapalene cream or gel (Differin), are licensed to treat psoriasis or acne, but may also help people with skin lymphoma. […] Rather than fighting cancer directly, immunotherapy stimulates your immune system to attack cancer cells. […] Interferon-alfa stimulates the immune system to fight cancer cells and stops cancer cells dividing.

• #2 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Primary cutaneous B-cell lymphomas (pCBCL) include an infrequent group of non-Hodgkin lymphomas that are limited to skin sites at the time of diagnosis. They comprise roughly 2025% of all cutaneous lymphomas and are subdivided into primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large cell B cell lymphoma, leg type (PCDLCBCL, LT). The first two show a rather indolent course while PCDLCBCL, LT carries a worse prognosis. […] For solitary, single-site PCMZL and PCFCL, several topical treatment options exist. They include, but are not limited to, excision, radiotherapy, and intralesional therapies, discussed in this review. However, in selected cases, even watchful waiting is reasonable. […] Indolent types of pCBCL rarely require systemic treatment. However, in extended cases and more importantly DLCBCL, LT, systemic treatment is the first choice. Monoclonal anti-CD20-antibody rituximab is often used as monotherapy in PCMZL and PCFCL or combined with chemotherapy in PCDLBCL, LT. Newer options are monoclonal anti-CD40 antibody dacetuzumab, anti-PD-1 and anti-PD-L1 checkpoint inhibitors, and Bruton tyrosine kinase inhibitors.

• #2 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Lenalidomide is an immunomodulatory drug used in multiple myeloma. […] Venetoclax shows remarkable clinical activity in chronic lymphocytic leukemia; however, despite high-level expression of BCL-2 in systemic DLBCL, responses in this malignancy are seldom. […] Localized pCBCL (PCMZL, PCFCL) shows high 5-year disease-specific survival rates (99 vs. 95%, respectively); hence, an active wait and see approach is often applicable. […] Systemic treatments almost exclusively play a role in PCDLBCL, LT, and systemic (extracutaneous) lymphoma. […] For topical regimens, there is a new approach with the intralesional application of T-VEC, currently investigated in a phase I trial. […] The field of systemic treatment is getting more interesting with a growing number of monoclonal antibodies, checkpoint inhibitors (PD1), Bruton kinase inhibitors and other small molecules.

• #2 Cutaneous B-cell lymphoma

  https://dermnetnz.org/topics/cutaneous-b-cell-lymphoma

  Patient with multiple lesions can be observed or given radiation, topical agents, cryotherapy, intralesional steroids or systemic therapy. Where disseminated skin lesions or large tumours occur, chemotherapy may be required such as ie, CHOP or R-CHOP. […] Immune-based therapies like rituximab, interferon and imiquimod are being explored. […] The recommended therapies for PCMZL are similar to PCFCL. […] Radiotherapy is highly effective and potentially curative for solitary or few contiguous lesions, but the relapse rates of PCMZL can be higher than PCFCL. […] Primary surgical excision can be effective for solitary or localised PCMZL. […] Local treatments include topical corticosteroids, intralesional steroids, topical nitrogen mustard, intralesional rituximab and cryotherapy. […] Multifocal skin lesions are treated with chemotherapy agents such as chlorambucil, interferon-alpha and anti-CD20 antibody (rituximab).

• #2 Cutaneous B-cell Lymphoma: Treatment Options – LRF

  https://www.lymphoma.org/understanding-lymphoma/aboutlymphoma/nhl/cbcl/cbcltreatment/

  First-line treatment options for cutaneous B-cell lymphoma depend on the type of CBCL, the patients symptoms and overall health, and the stage of the disease. […] Treatment options for primary cutaneous follicle center lymphoma and cutaneous marginal zone B-cell lymphoma, depending on the number of lesions present, include: […] Observation without active treatment which is known as active surveillance (also known as watch and wait or watchful waiting). During this period, regular follow-up with your healthcare team is required. […] Radiation Therapy […] Surgery […] There is no known standard treatment strategy for the more aggressive types of CBCL, such as cutaneous diffuse large B-cell lymphoma, leg type. For single tumors, radiation with or without chemotherapy is recommended. […] Similar to other B-cell NHLs, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with or without radiation therapy is commonly used to treat CBCL that has spread and become a more generalized lymphoma. […] Clinical trials are also often recommended. Although all of these treatments usually result in disease remission, relapse is common.

• #2 Cutaneous marginal zone lymphoma

  https://dermnetnz.org/topics/cutaneous-marginal-zone-lymphoma

  Spontaneous resolution of cutaneous marginal zone lymphoma can occasionally occur, particularly in the first few months. A solitary plaque or localised disease can be treated with radiotherapy or surgical excision, with curative intent. […] Multifocal disease can be treated with: Observation, Radiotherapy, Class 1 topical corticosteroid (eg, clobetasol propionate), Nitrogen mustard, Imiquimod cream, Cryotherapy, Intralesional steroids, Interferon-, Rituximab, Intravenous rituximab, Chlorambucil. […] Patients who develop disseminated skin lesions are treated with multi-agent chemotherapy. […] Antibiotic therapy may be indicated in patients who are positive for Borrelia burgdorferi antibodies.

• #2 Cutaneous B-cell lymphoma

  https://dermnetnz.org/topics/cutaneous-b-cell-lymphoma

  Rarely, disseminated skin lesions are treated with chemotherapy (eg, CHOP). […] Localised or solitary leg-type B-cell lymphoma is usually are treated with local radiotherapy alone or in conjunction with R-CHOP. […] Generalised PCDLBCL-LT can be treated with R-CHOP and/or local radiotherapy. Response rates are high but relapse rates are 58% Around 30% develop extracutaneous disease. […] Trials are assessing the efficacy of biological agents like ofatumumab, lumilixumab, dacetuzumab and intralesional TG1042 in the treatment of PCDLBCL-LT.

• #2

  https://link.springer.com/article/10.1007/s40257-022-00704-0

  First-line treatment for a solitary lesion or a single site of involvement is surgical excision with curative intent or radiation therapy (RT). […] Both have response rates around 90%. […] For a few localized lesions, RT is a better treatment option. […] However, the side effects of RT therapy are common including acute toxicities (local erythema, pain, edema, pruritus, anesthesia, and immediate pigmentation) and long-term side effects (alopecia, pruritus, pigmentation changes, atrophy, and telangiectasia). […] While the recommended total dose for PCMZL ranges between 4 and 36 Gy, multiple recent studies recommend the use of low-dose RT (4 Gy) because of improved efficacy and decreased toxicities. […] Antibiotic treatment is indicated in cases positive for Borrelia burgdorferi infection.

• #2 Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7426470/

  Indolent pCBCL are treated with a risk-adapted strategy using intralesional steroids, RT, and interferon- as first-line treatments. Relapsing cases may profit from rituximab. In aggressive PCDLCBCL, LT, rituximab with polychemotherapy is recommended. Innovative therapies include intralesional oncolytic virotherapy, systemic monoclonal antibodies, and small molecules. […] For localized low-grade lymphomas, skin-directed therapies with surgery, intralesional corticosteroids or interferon-, and radiotherapy are vivid treatment options. […] In generalized, relapsing, and advanced cases, systemic chemotherapy or immunotherapy is required. In aggressive cases, such as PCDLBCL, LT, a combination chemotherapy with monoclonal antibodies, i.e., R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) is the most common implemented therapy.

• #3 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  Optimal management of PCBCL requires multi-disciplinary collaboration between dermatologists, hematologists, pathologists and radiation oncologists. Guidelines for the treatment of PCBCL have been published by the EORTC. […] In localized disease, first-line therapies are usually local radiation or surgical excision. […] Complete response (CR) rate is close to 100% with local radiation, although relapses and acute adverse events (AE) occur in almost half of the cases. […] Disseminated lesions may be treated with intravenous rituximab, an anti-CD20 monoclonal antibody. […] When lesions are difficult to treat by radiotherapy or surgery, such as the face and the scalp, treatment with intralesional rituximab has been considered. […] In disseminated disease, another option is subcutaneous interferon-alpha.

• #3 Treatment Options for Cutaneous B-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/treatment-options-cutaneous-b-cell-lymphoma

  Examples of topical therapies used to treat CBCL include topical corticosteroids, imiquimod, topical nitrogen mustard and bexarotene. […] Radiation therapy is also highly effective, with most patients having a complete response (100% clearance of skin lesions) in the area treated. […] When indolent CBCL is present, a small amount of a drug usually corticosteroid, interferon, or the antibody drug rituximab (Rituxan) can be injected directly into the lesion. […] Systemic (internal) therapies for CBCL may be used when there are widespread lesions, when there is spread outside the skin, when skin directed therapy isn’t feasible for cosmetic reasons, or in the case of primary cutaneous diffuse large-B cell lymphoma. […] The most common systemic therapies used for CBCLs are rituximab, combination chemotherapies such as CHOP, and combinations of rituximab and chemotherapy (R-CHOP). […] Most patients with CBCL will respond to chemotherapy, but the rates of relapse are high.

• #3 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  To increase the efficacy of immunotherapy, antibodies have been combined with radioisotopes. […] Recently rituximab has also been combined with lenalidomide. […] Other immunotherapeutic approaches include intralesional adenovirus-interferon gamma (TG1042), a non-replicating human adenovirus vector allowing the intracellular transfer of human interferon (IFN)-gamma cDNA. […] Topical imiquimod monotherapy has been recently studied in CBCL in a retrospective monocentric cohort in the United States. […] PCDLBCL, LT is an aggressive lymphoma affecting elderly people and its management is thus challenging. […] Currently, first line treatment is rituximab and combination chemotherapy, most commonly cyclophosphamide, doxorubicine, vincristine and prednisone (CHOP)-like regimens if the general condition of the patient allows it.

• #3 Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7352758/

  The prognosis may be further improved using age-adapted rituximab-polychemotherapy. […] PCDLBCL, LT have the phenotype and gene expression profile of ABC-type DLBCL and the ESMO guidelines recommend that they should be treated as other ABC-type DLBCLs. […] The discovery of a subset of PCDLBCL, LT with recurrent genetic alterations in PD-L1/PD-L2 suggests that the use of immune checkpoint inhibitors could be explored.

• #4 Primary cutaneous B cell lymphoma: Clinical features, diagnosis and treatment

  https://www.wjgnet.com/2218-6190/full/v4/i1/50.htm

  Primary cutaneous B cell lymphoma (PCBCL) is defined as B cell lymphomas that presents in the skin without any evidence of extra-cutaneous involvement at diagnosis. PCBCL is a heterogeneous group of diseases comprising different B cell lymphomas with distinct treatment and prognosis. Local therapies like radiotherapy, surgery or intralesional steroids are options for localized disease in indolent forms. More disseminated disease may be treated with a systemic therapy like single agent rituximab. However combination chemotherapies which are used in systemic lymphomas are also required for aggressive PCBCL. Although indolent forms have relatively better prognosis, early relapses and disseminated diseases are mostly observed in aggressive form with a consequent poor prognosis. […] Treatment depends on the symptoms of the patient, stage of the disease and the number of the lesions. The treatment options include antibiotics, rituximab, chemotherapy, intralesional interferon alfa, radiotherapy and excision.

• #4 Unveiling Primary Cutaneous B-Cell Lymphomas: New Insights into Diagnosis and Treatment Strategies

  https://www.mdpi.com/2072-6694/17/7/1202

  Regarding ISRT, there is no consensus on the optimal radiation doses for indolent PCBCL, with treatment regimens varying widely. Success has been reported from very low-dose ISRT (VLD-IRST; 2–4 Gy in 2 fractions) to standard-dose ISTR (SD-IRST; 24–40 Gy in 12–20 fractions). Both regimens have demonstrated similar efficacy (CRs), but VLD-ISRT is associated with a significantly lower incidence of side effects (15.7% vs. 78.4%, p < 0.0001), making it an attractive option for treating indolent PCBCL. [...] Alternative treatment strategies for solitary or limited lesions of indolent PCBCL include intralesional corticosteroids, particularly for small lesions of PCMZL/LPD, though the CR rate is suboptimal at approximately 44% and multiple treatment cycles are often required. Systemic antibiotics such as cephalosporins and tetracyclines may be effective for lesions associated with Borrelia burgdorferi infection (especially in endemic regions of Europe). Other therapies include intralesional interferon-alpha (though not available in many countries), and intralesional rituximab, a chimeric monoclonal antibody targeting CD20.

• #4 Rituximab in the Treatment of Primary Cutaneous B-Cell Lymphoma: A Review | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-rituximab-in-treatment-primary-cutaneous-articulo-S1578219014001073

  The recommendations for management of LF and MZL include withholding treatment (watchful waiting), RT, surgery, or intralesional interferon alfa. Sometimes, however, large, painful, disfiguring, or itchy multiple lesions may be present; watchful waiting is not an option and treatment should be administered. Moreover, in these situations, local skin interventions, such as surgery or RT, are not appropriate, particularly in the case of multiple lesions or lesions on sites such as the face or neck where the interventions might leave substantial esthetic sequelae. In these circumstances, other treatment alternatives, among them systemic or intralesional rituximab, have been used. […] In most studies, intravenous rituximab is considered the treatment of choice for multifocal lesions and also for lesions on the face and scalp, as RT may cause alopecia and irreversible cutaneous effects, such as poichyloderma or atrophy.

• #4 Skin (cutaneous) B-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-b-cell-lymphoma

  If you have lymphoma in several areas of your skin and you are experiencing symptoms, you might have a chemotherapy drug called chlorambucil or an antibody treatment called rituximab. This can be given through a drip into a vein (intravenously) or as an injection directly into the affected skin. […] If your lymphoma has spread to your lymph nodes or other parts of your body, you might have rituximab plus a chemotherapy regimen (combination of treatment) such as bendamustine or CVP (cyclophosphamide, vincristine and prednisolone). […] Primary cutaneous diffuse large B-cell lymphoma, leg type, is usually treated with chemotherapy, often followed by radiotherapy to the affected area. The chemotherapy regimen (combination of treatment) most commonly used is called CHOP and is usually given alongside an antibody treatment called rituximab (R-CHOP).

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