Materiały źródłowe

• #1

  https://link.springer.com/article/10.1007/s13555-023-00895-2

  Cutaneous T-cell lymphoma (CTCL) is a rare, heterogeneous group of non-Hodgkin lymphomas characterized by various clinical, molecular, and histopathologic features of the skin. Variants of CTCL share many clinical features with common inflammatory skin diseases such as atopic dermatitis and psoriasis, making accurate and early diagnosis challenging in clinical settings. Inappropriate treatment or a delay in diagnosis can lead to increased morbidity and mortality. […] An electronic survey of ten questions was developed and approved by physician experts in CTCL to assess experiences in diagnosing and treating CTCL among healthcare providers (HCPs). […] Most respondents (91%) reported that they include CTCL in their differential diagnoses after patients do not respond to treatment of more common conditions. Patients with CTCL were frequently diagnosed with other inflammatory dermatosesmost commonly dermatitis and psoriasisbefore a CTCL diagnosis, and many were treated with ineffective therapies for years.

• #2

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8484943/

  Cutaneous T-cell lymphoma (CTCL) encompasses a group of low-grade, non-Hodgkin lymphoma, including mycosis fungoides and Szary syndrome. Diagnosis of CTCL can be challenging given the prolonged, gradual onset and shared characteristics with many benign inflammatory skin diseases. […] In particular, we highlight clinical pearls to aid in distinguishing CTCL from inflammatory dermatoses and describe the diagnostic strategy to help dermatologists arrive at the diagnosis of CTCL at earlier stages of the disease. […] This case highlights the importance of repeat biopsies if initial biopsies indicate parapsoriasis and/or the presence of lymphocytic exocytosis. Moreover, the case demonstrates the importance of waiting 2 weeks from the last topical corticosteroid use to biopsy dermatitis properly and identify any potential cases of CTCL.

• #3 Skin lymphoma | Lymphoma Action

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

  Skin lymphoma is difficult to diagnose as it can develop slowly, sometimes over decades, and often resembles more common skin conditions. […] The most important test for diagnosing a skin lymphoma is a skin biopsy. The doctor numbs an affected area of your skin with a local anaesthetic and removes a small sample. This is sent to the laboratory to be examined under a microscope and for specialised genetic tests. […] Diagnosing skin lymphoma is a bit like solving a jigsaw puzzle. A team of people from different hospital departments are involved in identifying the pieces and putting them together. […] Even if your doctor suspects a skin lymphoma, you may need to have tests repeated several times before the diagnosis is confirmed. On average it takes around three years to get a confirmed diagnosis of skin lymphoma. However, it might take considerably more or less time.

• #4 Meeting Key Challenges in the Diagnosis and Treatment of Cutaneous T-Cell Lymphoma – Personalized Medicine in Oncology

  https://personalizedmedonc.com/supplements/meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma-highlights-from-an-expert-roundtable/15227:meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma

  Cutaneous T-cell lymphomas (CTCLs) are a rare group of heterogeneous non-Hodgkin lymphomas comprising several different types of malignancies that present with diverse characteristics, clinical courses, and prognoses. The definitive diagnosis of CTCL is often challenging because of nonspecific clinical and pathologic features, requiring integration of clinical, histopathologic, immunophenotyping, and molecular data. The expert panel identified several challenges in diagnosing CTCL in the real-world setting. The panel indicated that the length of time between a patients first visit to a physician and the diagnosis of CTCL is variable and depends on the type of CTCL, the stage of disease, and the responsible physician (ie, a dermatologist, primary care physician [PCP], or infrequently, an oncologist). Making a diagnosis of CTCL typically takes approximately 3 years, largely because early-stage CTCL often resembles benign inflammatory conditions, such as eczema or parapsoriasis.

• #5 Cutaneous T-cell lymphoma | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/cutaneous-t-cell-lymphoma

  To diagnose cutaneous T-cell lymphoma, a healthcare professional might start with an exam to look for signs of cancer. Tests and procedures might include blood tests, skin biopsies and imaging tests. Cutaneous T-cell lymphoma may be hard to diagnose because the symptoms are like those of other skin conditions, such as eczema. And early tests may not show cancer cells in the skin. Getting the right diagnosis may take time. […] A healthcare professional may do a physical exam to look over your skin for scaly areas or growths. The healthcare professional may check for other signs of cutaneous T-cell lymphoma, such as swollen lymph nodes. […] Blood tests such as a complete blood count can give information about your condition. Sometimes blood tests show cancer cells in the blood. This is more common with one type of cutaneous T-cell lymphoma called Sezary syndrome.

• #5 Cutaneous T-cell lymphoma | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/cutaneous-t-cell-lymphoma

  A skin biopsy is a procedure to remove cells from the surface of the body so that they can be tested in a lab. The tests can show whether cancer cells are present in the skin. […] Skin biopsies don’t always detect cancer cells, even when cancer is present. You might need more than one skin biopsy over time. […] If there’s concern that the cancer cells have spread to other parts of the body, your healthcare professional might suggest imaging tests. These might include a computerized tomography scan, also called a CT scan, or a positron emission tomography scan, also called a PET scan.

• #6 Meeting Key Challenges in the Diagnosis and Treatment of Cutaneous T-Cell Lymphoma – Personalized Medicine in Oncology

  https://www.personalizedmedonc.com/supplements/meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma-highlights-from-an-expert-roundtable/meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma

  Cutaneous T-cell lymphomas (CTCLs) are a rare group of heterogeneous non-Hodgkin lymphomas comprising several different types of malignancies that present with diverse characteristics, clinical courses, and prognoses. The definitive diagnosis of CTCL is often challenging because of nonspecific clinical and pathologic features, requiring integration of clinical, histopathologic, immunophenotyping, and molecular data. The expert panel deliberated on key challenges in making a timely and accurate diagnosis of CTCL, issues related to CD30 testing, and treatment approaches for patients with CTCL who require systemic therapy. The expert panel identified several challenges in diagnosing CTCL in the real-world setting. Making a diagnosis of CTCL typically takes approximately 3 years, largely because early-stage CTCL often resembles benign inflammatory conditions, such as eczema or parapsoriasis. The panel emphasized the importance of documenting a clinicopathologic correlation for the accurate diagnosis of CTCL. Indeed, European Society for Medical Oncology (ESMO) clinical practice guidelines on the diagnosis and treatment of primary cutaneous lymphomas (PCLs) indicate that the diagnosis and classification of PCLs should always be based on a combination of clinical, histologic, immunophenotypic, and genetic data. The major problems in diagnosing CTCL, particularly in the early stages, are that in the majority of cases there is not a clinically specific interpretation of the lesion. The most important thing is to have a team that includes the clinician and the dermatopathologist, who work closely together to see the complete clinical picture and then do the right biopsy from the right edges of the lesion. The panel agreed that early diagnoses may have very important treatment implications, such as avoiding incorrect treatment and negatively affecting eventual clinical outcomes. The treatment goal continues to be establishing the diagnosis as early as possible and preventing the disease from progressing. Early diagnosis is essential to prevent treatment delay, mistreatment, and a deleterious effect on quality of life due to itching, pain, and disfigurement. The panel emphasized the importance of making oncologists, dermatologists, and pathologists aware of the need for CD30 testing and its critical role in the diagnosis and treatment selection for CTCL.

• #7

  https://link.springer.com/article/10.1007/s13555-023-00895-2

  Two-thirds of HCPs agreed that further education surrounding CTCL is needed. […] Given the infrequency of CTCL and its similar presentation to other common dermatologic conditions, increased education of CTCL is needed in the dermatology community to improve patient outcomes. […] Early detection of CTCL requires extensive experience, and accurate diagnosis does not rely on any one clinical, biologic, or histopathologic factor. […] It is critical that patients with CTCL receive an early diagnosis to ensure an optimal treatment regimen; however, difficulty with differentiating CTCL from other inflammatory diseases with similar symptomatology can lead to delays in diagnosis and poor patient outcomes. […] To shorten the time to diagnosis, improved education and greater experience are needed among dermatologists and other healthcare providers (HCPs) in the dermatology field.

• #8 Cutaneous T-Cell Lymphoma | UMass Memorial Health

  https://www.ummhealth.org/health-library/cutaneous-t-cell-lymphoma

  The most common way to find CTCL is when a person sees a healthcare provider because of an itchy patch or rash on their skin that doesnt get better or keeps getting bigger. The provider will ask about your health history, symptoms, risk factors, and family history of disease. They will do a physical exam and check for enlarged lymph nodes and look at all of your skin. […] You likely will be sent to a dermatologist. This is a healthcare provider with special training to treat skin problems. The healthcare provider may use a special light, magnifying lens, or camera to get a very close look at the changed skin. […] You will very likely need one or more skin biopsies. This is when the healthcare provider takes small pieces of tissue from the affected skin. These samples can be removed with a sharp blade or scalpel and looked at under a microscope by a pathologist to check for cancer cells. Special tests on the tissue will be done. This is the only way to know for sure that the changes are because of CTCL.

• #9 Cutaneous T-Cell Lymphoma (CTCL)

  https://my.clevelandclinic.org/health/diseases/17940-cutaneous-t-cell-lymphoma

  Cutaneous T-cell lymphoma may affect lymph nodes, spleen, large intestine, small intestine and inguinal lymph nodes In 10% of cases, cutaneous T-cell lymphoma may affect your lymph nodes (top), spleen, large intestine, small intestine (middle) and inguinal lymph nodes (bottom). […] Many people have symptoms like skin rash, itchiness or discoloration for years before obtaining a diagnosis. […] A healthcare provider will review your medical history and do a physical examination, focusing on any patches or plaques (thickened areas) on your skin. Theyll ask you about your symptoms and review your health history, including any prior or current health conditions. […] Your healthcare provider will need to run tests to confirm a cutaneous T-cell lymphoma diagnosis. These tests may include: Blood tests. Imaging tests like CT scans or PET scans. Lymph node biopsy. Skin biopsy.

• #9 Cutaneous T-Cell Lymphoma (CTCL)

  https://my.clevelandclinic.org/health/diseases/17940-cutaneous-t-cell-lymphoma

  Healthcare providers use staging systems to categorize, diagnose and treat cancers. The TNM classification system is the most common for staging CTCLs. […] The overall 10-year survival rate for people with early-stage (Stage I or II) CTCL is 90%. That means that 9 out of 10 people with this condition are still alive 10 years later. People with late-stage CTCL (Stage III or IV) have an overall 10-year survival rate of 53%. […] Its important to let a healthcare provider know any time you notice skin changes. They can determine the cause. Maybe its not cancer. But if it is, treatment is key. The sooner you start it, the better your overall outlook.

• #10 Cutaneous Lymphoma Cancer Types, Symptoms, and Diagnosis

  https://www.ahn.org/services/cancer/types/cutaneous-lymphoma

  Your doctor will ask about your symptoms, family history of cancer, and any relevant medical conditions. They will examine your skin, lymph nodes, and other areas to assess the extent of the disease. Tests are performed to get a whole picture of your whole health. Each cancer case is different, and your care and treatment plan are unique to your needs. […] Common screening options include: Skin biopsy: A small sample of skin is taken and examined under a microscope. Blood tests: A blood draw can be used to check for cancerous cells in the blood. Imaging tests: CT scans or MRI scans, may be used to assess the extent of the lymphoma in the body. […] The prognosis for cutaneous lymphoma varies depending on the type and stage. Regular screening, especially for those at higher risk is important. Your doctor can help determine what screening looks like for you.

• #11 OBTAINING A PROPER CUTANEOUS LYMPHOMA DIAGNOSIS | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/obtaining-proper-cutaneous-lymphoma-diagnosis

  One of the most difficult things about uncommon diseases for both patients and physicians is reaching a correct diagnosis. […] A definitive diagnosis will help inform treatment decisions and potentially yield better patient-related outcomes over time. […] In the case of cutaneous lymphoma, a definitive diagnosis sometimes takes years. […] Since reactive processes and other types of inflammation can trigger symptoms similar to those of cutaneous lymphomas, evaluation by a provider with experience in diagnosing skin lymphoma is important if skin lymphoma is suspected. […] A definitive diagnosis cannot be obtained without a biopsy, and multiple biopsies are often necessary to confirm the diagnosis of cutaneous lymphoma. […] It is very important to confirm any diagnosis of cutaneous lymphoma by a specialized type of pathologist – dermatopathologist or a hematopathologist – who has expertise in diagnosing cutaneous lymphomas.

• #12 Tests for Lymphoma of the Skin | American Cancer Society

  https://www.cancer.org/cancer/types/skin-lymphoma/detection-diagnosis-staging/how-diagnosed.html

  Because this type of lymphoma affects the skin, it is often noticed fairly quickly. But the actual diagnosis of skin lymphoma might be delayed because the symptoms often resemble other, more common skin problems. The diagnosis of skin lymphoma can only be confirmed with a skin biopsy. Other tests might be needed as well. […] A biopsy is a procedure in which a doctor removes a sample of body tissue for viewing under a microscope or other lab tests. A biopsy is needed to diagnose lymphoma of the skin. […] Many of the more common forms of skin cancer (and other skin diseases) can be diagnosed just by looking at the biopsy samples. But diagnosing and classifying lymphomas of the skin often requires one or more special lab tests. […] Diagnosing some forms of skin lymphoma can be very challenging. Sometimes, especially if the diagnosis is unclear, the skin samples may need to be sent to a dermatopathologist, a dermatologist or a pathologist with additional training in diagnosing skin samples. Even with this expertise, in some cases several biopsies may be need to be done in different areas of abnormal skin and/or at different times before the diagnosis is confirmed.

• #12 Tests for Lymphoma of the Skin | American Cancer Society

  https://www.cancer.org/cancer/types/skin-lymphoma/detection-diagnosis-staging/how-diagnosed.html

  Skin lymphomas often spread to lymph nodes, so your doctor may recommend a lymph node biopsy to help confirm the diagnosis or to help determine how widespread the lymphoma is. […] Removing a lymph node almost always provides enough tissue to diagnose the exact type of lymphoma. Most doctors prefer this type of biopsy, if it can be done without too much discomfort to the patient. […] Lab tests are done on the biopsy samples (and in some cases, blood samples) to help diagnose lymphoma and determine what type it is. Pathologists can sometimes tell which kind of lymphoma a person has by just looking at the cells under a microscope, but usually these other types of tests are needed to confirm the diagnosis. […] Blood tests measure the amounts of certain types of cells and chemicals in the blood. They are not used to diagnose lymphoma usually, but they can sometimes help determine how advanced the lymphoma is. […] Imaging tests aren’t always needed for people with skin lymphomas who have only a few skin lesions, but they are often done if a lot of the skin is affected, or if lymphoma cells are found in the lymph nodes or blood.

• #13

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8484943/

  This case highlights the fact that dermatitis in adults can be multifactorial, and thorough diagnosis with patch testing and repetitive biopsies can lead to the most expeditious path to effective therapy. […] The pearls included in this report may help dermatologists arrive at a diagnosis of CTCL in the earlier stages of the disease.

• #14 Diagnosing Cutaneous T-Cell Lymphoma | NYU Langone Health

  https://nyulangone.org/conditions/cutaneous-t-cell-lymphoma/diagnosis

  If cutaneous T-cell lymphoma is found with a skin biopsy, doctors at NYU Langone may use a full-body PET scan to see whether cancer has spread beyond the skin to other soft tissues of the body, such as the lymph nodes, causing them to enlarge and forming tumors there. […] Our doctors may perform a lymph node biopsy to help confirm a diagnosis if a skin biopsy does not provide enough information. […] Often, our doctors choose to remove one lymph node during a surgical procedure. […] If your doctors believe surgery poses a risk to your health, they may try to obtain a smaller tissue sample using a needle, which is less invasive. […] After diagnosis, our doctors may use bone marrow aspiration and biopsy to see if cutaneous T-cell lymphoma has spread to bone marrow.

• #15

• #16 Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/2139720-overview

  Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of lymphoproliferative disorders characterized by localization of neoplastic T lymphocytes to the skin, with no evidence of extracutaneous disease at the time of diagnosis. […] In most cases of mycosis fungoides, the diagnosis is reached owing to its clinical features, disease history, and histomorphologic and cytomorphologic findings. An additional diagnostic criterion to distinguish CTCL from inflammatory dermatoses is demonstration of a dominant T-cell clone in skin biopsy specimens by a molecular assay (ie, Southern blot, polymerase chain reaction [PCR]). Genetic testing may also be considered. […] The following laboratory tests are included in the diagnostic workup of mycosis fungoides: Complete blood count with differential; review the buffy coat smear for Szary cells, Liver function tests: Look for liver-associated enzyme abnormalities, Uric acid and lactate dehydrogenase levels: These are markers of bulky and/or biologically aggressive disease, Flow cytometric study of the blood (include available T-cell related antibodies): To detect a circulating malignant clone and to assess immunocompetence by quantifying the level of CD8-expressing lymphocytes, Human immunodeficiency virus (HIV) and human T-lymphotropic virus type 1 (HTLV-I) testing.

• #16 Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/2139720-overview

  For a diagnosis of Szary syndrome, one or more of the following criteria should be met: Absolute Szary cell count of at least 1000 cells/L, Immunophenotypic abnormalities (expanded CD4+ T-cell population resulting in CD4/CD8 ratio of 10; loss of any or all of T-cell antigens CD2, CD3, CD4, and CD5; or loss of both CD4 and CD5), T-cell clone in the peripheral blood shown by molecular or cytogenetic methods: Flow cytometry may be useful for differential diagnosis of precursor and peripheral T-cell and NK-cell lymphomas. […] Although mycosis fungoides and Szary syndrome are types of NHL, a different staging system is used, based on the particular skin findings and findings of extracutaneous disease.

• #17 Lab Tests in Staging Cutaneous Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/lab-tests

  Flow cytometry of the blood tests for circulating lymphoma cells in the blood (also called Szary cells), and is often done for newly diagnosed patients with CTCL. […] Your providers might recommend evaluating your bone marrow for involvement by cutaneous lymphoma if there are any unexplained abnormal blood counts.

• #18 Cutaneous T-Cell Lymphoma | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/cutaneous-t-cell-lymphoma

  A series of detailed pictures, taken from different angles, are created by a computer linked to an X-ray machine. […] A large magnet, radio waves and a computer are used to produce pictures of the affected area. […] This imaging test uses a radioactive substance called a tracer to look for disease in the body.

• #19 Cutaneous T-cell Lymphoma – Newcastle Hospitals NHS Foundation Trust

  https://www.newcastle-hospitals.nhs.uk/services/dermatology/patient-dermatology-information-leaflets/cutaneous-t-cell-lymphoma/

  You have been diagnosed with a type of skin lymphoma. In the north east of England we have a specialist clinic for patients with this rare type of skin cancer which is held on a monthly basis in Newcastle-upon-Tyne. This information sheet provides a summary of the condition and provides links to other information and local service contacts. […] Skin lymphoma is diagnosed by a skin biopsy. However the diagnosis is not always easy and sometimes patients need more than one biopsy over a period of time before the diagnosis can be established. […] Skin lymphomas are normally assessed by examination of the skin, feeling for lymph glands, routine blood tests and the results of the skin biopsy. It is rare for skin lymphomas to involve internal organs and therefore scans may not be required in early stage disease. Patients with more advanced stage disease (such as raised skin lumps or tumours, swollen lymph glands or abnormal blood tests) will require scans to exclude involvement of other organs.

• #20 Primary cutaneous lymphomas: diagnosis and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4692822/

  Immunophenotyping of the abnormal cells should be pursued in every case, either by means of immunohistochemical evaluation of the biopsy specimen, or by flow cytometric analysis of the cell suspension (immunocytochemistry). […] The diagnosis must be complemented with precise staging of the disease. […] Identification of the prognostic factors following the International Prognostic Index (IPI) is of pivotal importance for the further choice of the best treatment method as well as for the evaluation of the treatment results. […] The treatment response and monitoring of the disease can, in most cases of primary CL, be based on the skin examination according to the modified Severity Weighted Assessment Tool (mSWAT). […] Clinical evaluation of the therapy response is sufficient for IA grade (withdrawal of the erythema), but the advanced stages require histopathological evaluation to assess the recession of the skin lesions. […] The prognosis in MF depends on the phase of the disease, its type, extent of the skin lesions and lymph node and/or internal organ involvement.

• #20 Primary cutaneous lymphomas: diagnosis and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4692822/

  Primary cutaneous lymphomas (CLs) are a heterogeneous group of lymphoproliferative neoplasms, with lymphatic proliferation limited to the skin with no involvement of lymph nodes, bone marrow or viscera at the diagnosis. […] Histopathological evaluation including immunophenotyping of the skin biopsy specimen is the basis of the diagnosis, which must be complemented with a precise staging of the disease and identification of prognostic factors, to allow for the choice of the best treatment method as well as for the evaluation of the treatment results. […] In most of the cases, the initial diagnosis is put forward by a dermatologist in cooperation with a pathologist. Histopathological evaluation of the skin biopsy forms the basis for the diagnosis of the primary cutaneous lymphomas, which may be also sometimes diagnosed from a lymph node specimen or a biopsy specimen from another infiltrated organ.

• #21 Cutaneous T-cell Lymphomas (CTCL) – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/lymphomas/cutaneous-t-cell-lymphomas-ctcl

  Mycosis fungoides (cutaneous T-cell lymphoma) can be difficult to distinguish from nonmalignant chronic dermatoses. Accurate diagnosis can be made only by a detailed clinical history (no progression of nonmalignant lesions over time) and biopsy with microscopic evaluation. […] Diagnosis is based on skin biopsy, but histology may be equivocal early in the course because of insufficient quantities of lymphoma cells. The malignant cells are mature CD4+ T cells that may have lost common T-cell markers such as CD7. […] Characteristic Pautrier microabscesses may be present in the epidermis on skin punch biopsies. In some cases, a leukemic phase is characterized by the appearance of malignant T cells with serpentine nuclei in the peripheral blood (Szary cells). These malignant T cells in the blood can be detected on a routine Wright-stained smear or by flow cytometry.

• #21 Cutaneous T-cell Lymphomas (CTCL) – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/lymphomas/cutaneous-t-cell-lymphomas-ctcl

  After diagnosis, stage is determined to guide therapy. The commonly used ISCL/EORTC (International Society of Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer) staging system incorporates physical examination findings, histopathology results, and results of imaging tests.

• #22 Cutaneous T-cell lymphoma: Symptoms, Classification, and Treatment – DermNet

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

  Cutaneous T-cell lymphoma is the most common type of primary cutaneous lymphoma. It is a form of non-Hodgkin lymphoma in which malignant T-cells are initially localised to the skin with no evidence of extracutaneous disease at the time of diagnosis. […] The diagnosis of cutaneous T-cell lymphoma is often delayed by months or years due to the indolent course, clinical similarity to inflammatory dermatoses, and subtle changes on histopathology. […] Diagnosis of cutaneous T-cell lymphoma involves careful clinicopathological correlation. Multiple skin biopsies are often required to detect the characteristic histopathology changes, especially in the early patch stages. Further investigations may be performed on skin biopsy specimens including immunohistochemistry. […] EBV, T-cell receptor (TCR) gene rearrangements, ALK, DUSP22-IRF4, and TP63 gene studies may be performed to confirm the diagnosis and predict the clinical course. […] Formal TNMB staging is recommended in some forms of CTCL.

• #23 A deep dive into rare diseases: cutaneous T-cell lymphoma | VJHemOnc

  https://www.vjhemonc.com/a-deep-dive-into-rare-diseases-cutaneous-t-cell-lymphoma/

  In CTCL, the type and stage of disease significantly impact prognosis, meaning that accurate diagnosis and staging are critical to ensure patients receive the most appropriate treatment. Diagnosing CTCL can be challenging due to shared characteristics with several common skin conditions. Therefore, repeated skin biopsies are necessary, alongside blood tests, imaging, bone marrow and lymph node biopsies, to allow for clinicopathological correlation. To accurately differentiate between different sub-types of CTCL, a biopsy of each type of skin lesion should be performed. […] As the management of CTCL requires a risk-adapted approach, patients are risk stratified using the TNMB (tumor, node, metastasis, blood) staging system, which was most recently updated in 2022 by the International Society for Cutaneous Lymphomas (ISCL), the United States Cutaneous Lymphoma Consortium (USCLC), and the Cutaneous Lymphoma Task Force of the European Organization for the Research and Treatment of Cancer (EORTC). Using this system, a clinical stage of disease is determined, and this informs the therapeutic strategy selected for the patient.

• #24 Cutaneous T-cell lymphoma – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/1025

  Cutaneous T-cell lymphomas (CTCL) diagnosis is based on a combination of clinical, histological, immunophenotypical, and genetic data, and usually requires specialist expertise. […] Establishing a diagnosis can be challenging, as the condition can take many different forms in the skin: flat patches, raised plaques, large tumours, and/or marked erythroderma (intense and widespread reddening of the skin). […] Diagnostic investigations include skin biopsy, clonal T-cell receptor rearrangement, and flow cytometry. […] The choice of skin-directed therapy or systemic treatment is dependent on both doctor and patient preference; no one treatment option has been shown to be superior to another. […] Patients diagnosed with early-stage disease have a higher rate of survival.

• #25 Meeting Key Challenges in the Diagnosis and Treatment of Cutaneous T-Cell Lymphoma – Personalized Medicine in Oncology

  https://personalizedmedonc.com/supplements/meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma-highlights-from-an-expert-roundtable/15227:meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma

  Optimally, the diagnosis of CTCL involves a collaborative effort between the (dermato)pathologist and the treating physician and requires taking a careful history; performing a physical examination; obtaining skin biopsies for histopathology, immunohistochemistry, and molecular analysis; and, in the case of erythroderma, an assessment of peripheral blood for the presence of malignant T-cells. The panel emphasized the importance of documenting a clinicopathologic correlation for the accurate diagnosis of CTCL. Indeed, European Society for Medical Oncology (ESMO) clinical practice guidelines on the diagnosis and treatment of primary cutaneous lymphomas (PCLs), which were released in June 2018, indicate that the diagnosis and classification of PCLs should always be based on a combination of clinical, histologic, immunophenotypic, and genetic data.

• #26 The Pathology of Cutaneous T-Cell Lymphoma

  https://www.cancernetwork.com/view/pathology-cutaneous-t-cell-lymphoma

  The diagnosis of cutaneous T-cell lymphoma (CTCL) requires accurate histopathology, including immunocytochemistry, as well as careful clinical appraisal and analysis for T-cell clonality. […] Therefore, clinical appraisal plus the presence of characteristic histopathologic features are needed to ensure accurate diagnosis. […] Clinical information is particularly important in the diagnosis of LyP, as the disease appears malignant histologically, but has a benign clinical course. […] Successful diagnosis requires not only accurate histopathology, including immunocytochemistry, but also good clinical appraisal and analysis for T-cell clonality. […] An accurate diagnosis is of paramount importance not only to distinguish between neoplastic and inflammatory processes, but also to determine the precise type of lymphoma. These judgements require careful attention to the clinical presentation, critical histopathologic/immunocytochemical appraisal, and use of molecular data.

• #27 Cutaneous T-cell lymphoma: Diagnosis & treatment

  https://www.aad.org/public/diseases/a-z/ctcl-treatment

  How do dermatologists diagnose cutaneous T-cell lymphoma? Because the first noticeable signs often appear on the skin, many people see a dermatologist to find out whats causing their skin problem. Its important to understand that it takes time to diagnose cutaneous T-cell lymphoma, also called CTCL. If your dermatologist believes that you may have a type of this cancer, your dermatologist will: Give you a complete skin exam, checking your skin for signs like rashes and plaques. Run blood tests. Perform a skin biopsy, to check for cancer. Ask what medications you take. […] Early cutaneous T-cell lymphoma can be difficult to find because: A skin biopsy often doesnt show cancer cells. This type of cancer can be difficult to find with one biopsy. To get an accurate diagnosis, you may need multiple skin biopsies over time. Blood tests may not show cancer cells. These cancers can look like a common skin condition, such as eczema or psoriasis, so your dermatologist will have to rule out these conditions.

• #28 Cutaneous T-Cell Lymphoma Differential Diagnoses

  https://emedicine.medscape.com/article/2139720-differential

  Cutaneous T-cell lymphomas are T-cell proliferative disorders. Primary cutaneous lymphomas require distinction from histologically similar primary nodal ones because their clinical behavior, prognosis, and therapy are often different. […] At times, disseminated infections such as leishmaniasis, leprosy, South American blastomycosis, coccidioidomycosis, and other deep fungal infections may mimic and require distinction from cutaneous T-cell lymphoma. […] Granulomatous mycosis fungoides with hypohidrosis may mimic lepromatous leprosy. […] Other conditions to consider in the differential diagnosis of cutaneous t-cell lymphoma include nonlymphomatous erythroderma and erythema neurolyticum migrans. […] Diagnosing erythrodermic cutaneous T-cell lymphoma. […] Consistency and distribution of reflectance confocal microscopy features for diagnosis of cutaneous T cell lymphoma.

• #29

  https://link.springer.com/article/10.1007/s13555-023-00895-2

  The survey was developed to assess HCP experiences in diagnosing and treating CTCL and to determine if further education and collaboration are needed to improve early detection. […] Results support the premise that CTCL is uncommon, with most respondents reporting that they diagnosed ten or fewer cases in the last 5 years. […] Additionally, patients may be treated for other diseases and skin conditions with similar symptomatology for up to 5 years before being ultimately diagnosed with CTCL, suggesting that late detection of CTCL is a common issue and that increased education is needed regarding differential diagnoses. […] This study emphasizes an increased need for education among dermatologists and HCPs involved in the diagnosis of CTCL to ensure proper treatment during the early phases of CTCL to prevent disease progression.

• #30 Cutaneous T-Cell Lymphoma Presenting as Benign Dermatoses | AAFP

  https://www.aafp.org/pubs/afp/issues/1999/0515/p2809.html

  Cutaneous T-cell lymphoma, also known as mycosis fungoides, is a malignancy of the T helper (CD4+) cells. Diagnosis is difficult early in the course of this disease because it mimics several benign skin disorders, including eczema, psoriasis and contact dermatitis. Cutaneous T-cell lymphoma is also difficult to identify histologically, and multiple biopsies may be necessary to confirm the diagnosis. […] The diagnosis may be missed and the patient left untreated for years because of the benign appearance of this disorder. Close follow-up with multiple biopsies over time may help the physician make the diagnosis. […] The diagnosis of CTCL is confirmed by skin biopsy. Several biopsies may be needed over time to make the diagnosis, since the early patch stage may mimic chronic inflammatory dermatoses. Immunophenotyping and T-cell receptor gene arrangement analysis confirming a malignant clone are sometimes helpful in diagnosis. […] Maintaining an index of suspicion for the disease, performing a skin biopsy and vigilant patient follow-up are essential to effectively treat this disorder in its early stages and prevent progression to a life-threatening malignancy.

• #31 Meeting Key Challenges in the Diagnosis and Treatment of Cutaneous T-Cell Lymphoma – Personalized Medicine in Oncology

  https://personalizedmedonc.com/supplements/meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma-highlights-from-an-expert-roundtable/15227:meeting-key-challenges-in-the-diagnosis-and-treatment-of-cutaneous-t-cell-lymphoma

  According to Dr Quaglino, the major problems in diagnosing CTCL, particularly in the early stages, are that in the majority of cases there is not a clinically specific interpretation of the lesion. If I send the pathologist a skin biopsy with dermatitis or patches, it could be difficult for the pathologist to make a correct diagnosis without a clinical correlation. The panel agreed that early diagnoses may have very important treatment implications, such as avoiding incorrect treatment and negatively affecting eventual clinical outcomes. […] Dr Scarisbrick explained, Early diagnosis is essential to prevent treatment delay, mistreatment, and a deleterious effect on quality of life due to itching, pain, and disfigurement. For patients with advanced-stage disease, rapid diagnosis is critical, particularly for SS and for stage IVA2-IVB patients who have a median survival of just 12 to 36 months from the time of diagnosis.

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