Subcutaneous T-cell lymphoma (also known as a "panniculitis-like T-cell lymphoma") is a cutaneous condition that most commonly presents in young adults, and is characterized by subcutaneous nodules.[1]: 739 Common symptoms include fever, fatigue, and pancytopenia.
Subcutaneous panniculitis-like T-cell lymphoma is a subtype of peripheral T-cell lymphoma. Peripheral T-cell lymphoma (PTCL) is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called T-cells and natural killer cells (NK cells) (see figure for an overview of PTCL subtypes). PTCL is a type of non-Hodgkin's lymphoma (NHL).[2] NHL affects two particular types of white blood cells: B-cells and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally.
Subcutaneous panniculitis-like T-cell lymphoma is a rare type of lymphoma that infiltrates the subcutaneous fat but does not involve the skin. There are two subtypes – alpha-beta and gamma-delta. Patients with the gamma-delta subtype have a more aggressive clinical course.[3]
Historically, subcutaneous panniculitis-like T-cell lymphoma, as with other types of T-cell lymphoma, was treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no drugs approved by the U.S. Food and Drug Administration (FDA) specifically for PTCL. There are two drugs, Pralatrexate and Romidepsin, which are both approved by the FDA for relapsed or refractory T cell lymphoma. Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy. Some patients may receive an autologous or allogeneic stem cell transplant.[5][6][7][8][9][10][11][12] Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.
Pralatrexate is one compound currently under investigation to add to the frontline treatment of PTCL. It is currently approved by the FDA for relapsed or refractory T cell lymphoma. Side effects include mouth sores, which can be severe.[citation needed]
^James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN978-0-7216-2921-6.
^Swerdlow SH, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2008
^Vose JM (October 2008). "Peripheral T-cell non-Hodgkin's lymphoma". Hematol. Oncol. Clin. North Am. 22 (5): 997–1005, x. doi:10.1016/j.hoc.2008.07.010. PMID 18954748.
^Kong, YY.; Dai, B.; Kong, JC.; Zhou, XY.; Lu, HF.; Shen, L.; Du, X.; Shi, DR. (Oct 2008). "Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathologic, immunophenotypic, and molecular study of 22 Asian cases according to WHO-EORTC classification". Am J Surg Pathol. 32 (10): 1495–502. doi:10.1097/PAS.0b013e31817a9081. PMID 18708940.
^Reimer P, Rüdiger T, Geissinger E, et al. (January 2009). "Autologous stem-cell transplantation as first-line therapy in peripheral T-cell lymphomas: results of a prospective multicenter study". J. Clin. Oncol. 27 (1): 106–13. doi:10.1200/JCO.2008.17.4870. PMID 19029417.
^Mercadal S, Briones J, Xicoy B, et al. (May 2008). "Intensive chemotherapy (high-dose CHOP/ESHAP regimen) followed by autologous stem-cell transplantation in previously untreated patients with peripheral T-cell lymphoma". Ann. Oncol. 19 (5): 958–63. doi:10.1093/annonc/mdn022. PMID 18303032.
^Rodríguez J, Conde E, Gutiérrez A, et al. (July 2007). "Frontline autologous stem cell transplantation in high-risk peripheral T-cell lymphoma: a prospective study from The Gel-Tamo Study Group". Eur. J. Haematol. 79 (1): 32–8. doi:10.1111/j.1600-0609.2007.00856.x. PMID 17598836.
^Corradini P, Tarella C, Zallio F, et al. (September 2006). "Long-term follow-up of patients with peripheral T-cell lymphomas treated up-front with high-dose chemotherapy followed by autologous stem cell transplantation". Leukemia. 20 (9): 1533–8. doi:10.1038/sj.leu.2404306. PMID 16871285.
^Gisselbrecht C, Lepage E, Molina T, et al. (May 2002). "Shortened first-line high-dose chemotherapy for patients with poor-prognosis aggressive lymphoma". J. Clin. Oncol. 20 (10): 2472–9. doi:10.1200/JCO.2002.02.125. PMID 12011124.
^Deconinck E, Lamy T, Foussard C, et al. (June 2000). "Autologous stem cell transplantation for anaplastic large-cell lymphomas: results of a prospective trial". Br. J. Haematol. 109 (4): 736–42. doi:10.1046/j.1365-2141.2000.02098.x. PMID 10929023.
^Haioun C, Lepage E, Gisselbrecht C, et al. (August 2000). "Survival benefit of high-dose therapy in poor-risk aggressive non-Hodgkin's lymphoma: final analysis of the prospective LNH87-2 protocol—a groupe d'Etude des lymphomes de l'Adulte study". J. Clin. Oncol. 18 (16): 3025–30. doi:10.1200/JCO.2000.18.16.3025. PMID 10944137.
subcutaneous, cell, lymphoma, also, known, panniculitis, like, cell, lymphoma, cutaneous, condition, that, most, commonly, presents, young, adults, characterized, subcutaneous, nodules, common, symptoms, include, fever, fatigue, pancytopenia, specialtyoncology. Subcutaneous T cell lymphoma also known as a panniculitis like T cell lymphoma is a cutaneous condition that most commonly presents in young adults and is characterized by subcutaneous nodules 1 739 Common symptoms include fever fatigue and pancytopenia Subcutaneous T cell lymphomaSpecialtyOncology cutaneous oncologyComplicationsHPS hemophagocytic syndrome Diagnostic methodRequires biopsy confirmation by expert hemopathologistDifferential diagnosisLupus panniculitis cutaneous gamma delta T Cell lymphoma Contents 1 Diagnosis 1 1 Classification 2 Treatment 3 Research 4 See also 5 References 6 External linksDiagnosis editClassification edit Subcutaneous panniculitis like T cell lymphoma is a subtype of peripheral T cell lymphoma Peripheral T cell lymphoma PTCL is defined as a diverse group of aggressive lymphomas that develop from mature stage white blood cells called T cells and natural killer cells NK cells see figure for an overview of PTCL subtypes PTCL is a type of non Hodgkin s lymphoma NHL 2 NHL affects two particular types of white blood cells B cells and T cells PTCL specifically affects T cells and results when T cells develop and grow abnormally nbsp Subcutaneous panniculitis like T cell lymphoma is a rare type of lymphoma that infiltrates the subcutaneous fat but does not involve the skin There are two subtypes alpha beta and gamma delta Patients with the gamma delta subtype have a more aggressive clinical course 3 It is described as CD3 CD4 CD8 with CD30 and CD56 usually negative 4 Treatment editHistorically subcutaneous panniculitis like T cell lymphoma as with other types of T cell lymphoma was treated similarly to B cell lymphomas However in recent years scientists have developed techniques to better recognize the different types of lymphomas such as PTCL It is now understood that PTCL behaves differently from B cell lymphomas and therapies are being developed that specifically target these types of lymphoma Currently however there are no drugs approved by the U S Food and Drug Administration FDA specifically for PTCL There are two drugs Pralatrexate and Romidepsin which are both approved by the FDA for relapsed or refractory T cell lymphoma Anthracycline containing chemotherapy regimens are commonly offered as the initial therapy Some patients may receive an autologous or allogeneic stem cell transplant 5 6 7 8 9 10 11 12 Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation CHOP or CHOP like regimens have been used 13 Research editPralatrexate is one compound currently under investigation to add to the frontline treatment of PTCL It is currently approved by the FDA for relapsed or refractory T cell lymphoma Side effects include mouth sores which can be severe citation needed See also editCutaneous T cell lymphoma Lennert lymphoma Skin lesionReferences edit James William D Berger Timothy G et al 2006 Andrews Diseases of the Skin clinical Dermatology Saunders Elsevier ISBN 978 0 7216 2921 6 Swerdlow SH WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues 2008 Vose JM October 2008 Peripheral T cell non Hodgkin s lymphoma Hematol Oncol Clin North Am 22 5 997 1005 x doi 10 1016 j hoc 2008 07 010 PMID 18954748 Kong YY Dai B Kong JC Zhou XY Lu HF Shen L Du X Shi DR Oct 2008 Subcutaneous panniculitis like T cell lymphoma a clinicopathologic immunophenotypic and molecular study of 22 Asian cases according to WHO EORTC classification Am J Surg Pathol 32 10 1495 502 doi 10 1097 PAS 0b013e31817a9081 PMID 18708940 Reimer P Rudiger T Geissinger E et al January 2009 Autologous stem cell transplantation as first line therapy in peripheral T cell lymphomas results of a prospective multicenter study J Clin Oncol 27 1 106 13 doi 10 1200 JCO 2008 17 4870 PMID 19029417 Mercadal S Briones J Xicoy B et al May 2008 Intensive chemotherapy high dose CHOP ESHAP regimen followed by autologous stem cell transplantation in previously untreated patients with peripheral T cell lymphoma Ann Oncol 19 5 958 63 doi 10 1093 annonc mdn022 PMID 18303032 Rodriguez J Conde E Gutierrez A et al July 2007 Frontline autologous stem cell transplantation in high risk peripheral T cell lymphoma a prospective study from The Gel Tamo Study Group Eur J Haematol 79 1 32 8 doi 10 1111 j 1600 0609 2007 00856 x PMID 17598836 Corradini P Tarella C Zallio F et al September 2006 Long term follow up of patients with peripheral T cell lymphomas treated up front with high dose chemotherapy followed by autologous stem cell transplantation Leukemia 20 9 1533 8 doi 10 1038 sj leu 2404306 PMID 16871285 d Amore F et al Blood 2006 108 A401 Gisselbrecht C Lepage E Molina T et al May 2002 Shortened first line high dose chemotherapy for patients with poor prognosis aggressive lymphoma J Clin Oncol 20 10 2472 9 doi 10 1200 JCO 2002 02 125 PMID 12011124 Deconinck E Lamy T Foussard C et al June 2000 Autologous stem cell transplantation for anaplastic large cell lymphomas results of a prospective trial Br J Haematol 109 4 736 42 doi 10 1046 j 1365 2141 2000 02098 x PMID 10929023 Haioun C Lepage E Gisselbrecht C et al August 2000 Survival benefit of high dose therapy in poor risk aggressive non Hodgkin s lymphoma final analysis of the prospective LNH87 2 protocol a groupe d Etude des lymphomes de l Adulte study J Clin Oncol 18 16 3025 30 doi 10 1200 JCO 2000 18 16 3025 PMID 10944137 Rojnuckarin P Nakorn TN Assanasen T Wannakrairot P Intragumtornchai T Mar 2007 Cyclosporin in subcutaneous panniculitis like T cell lymphoma Leuk Lymphoma 48 3 560 3 doi 10 1080 10428190601078456 PMID 17454599 External links edit Retrieved from https en wikipedia org w index php title Subcutaneous T cell lymphoma amp oldid 1186712657, wikipedia, wiki, book, books, library,
