Subcutaneous Panniculitis-Like T-cell Lymphoma: A Clinical and Pathologic Study of 14 Korean Patients
2
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive skin lymphoma characterized by neoplastic T-cell infiltration of the subcutaneous tissue,mimicking panniculitis. Objective: To describe the clinical and pathologic features of SPTL in Korean patients. Methods:Fourteen SPTL patients evaluated over 15 years were retrospectively reviewed. Results: The mean patient age was 35 years (range: 7∼73 years), with male predominance (2.5:1). Most patients presented with either nodules or plaques, occurring most commonly on the trunk, with two patients (14%) having hemophagocytic syndrome. Histopathologically,all patients showed infiltrates of small-tomedium pleomorphic cells mimicking panniculitis, with some also showing rimming, bean-bag cells, and fat necrosis. Most patients were positive for CD3 (14/14), CD8 (12/13),TIA-1 (9/9) and βf1 (5/5), but were negative for CD4 (11/12),CD20 (8/8), CD56 (14/14) and Epstein-Barr virus (8/8). Ten patients (71%) received chemotherapy and 2 (14%) died due to the disease, with an average survival time of 4 months. Survival analysis did not reveal any significant prognostic factors. Conclusion: This is the first series of patients with SPTL in Korea. Due to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous γδ T-cell lymphoma. (Ann Dermatol 23(3)329∼337, 2011)
Abstract
Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive skin lymphoma characterized by neoplastic T-cell infiltration of the subcutaneous tissue,mimicking panniculitis. Objective: To describe the clinical and pathologic features of SPTL in Korean patients. Methods:Fourteen SPTL patients evaluated over 15 years were retrospectively reviewed. Results: The mean patient age was 35 years (range: 7∼73 years), with male predominance (2.5:1). Most patients presented with either nodules or plaques, occurring most commonly on the trunk, with two patients (14%) having hemophagocytic syndrome. Histopathologically,all patients showed infiltrates of small-tomedium pleomorphic cells mimicking panniculitis, with some also showing rimming, bean-bag cells, and fat necrosis. Most patients were positive for CD3 (14/14), CD8 (12/13),TIA-1 (9/9) and βf1 (5/5), but were negative for CD4 (11/12),CD20 (8/8), CD56 (14/14) and Epstein-Barr virus (8/8). Ten patients (71%) received chemotherapy and 2 (14%) died due to the disease, with an average survival time of 4 months. Survival analysis did not reveal any significant prognostic factors. Conclusion: This is the first series of patients with SPTL in Korea. Due to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous γδ T-cell lymphoma. (Ann Dermatol 23(3)329∼337, 2011)
참고문헌 (17)
- Takeshita M, "Clinicopathologic differences between 22 cases of CD56-negative and CD56-positive subcutaneous panniculitis-like lymphoma in Japan", Hum Pathol, 35, pp.231~239 (2004)
- Willemze R, "WHO-EORTC classification for cutaneous lymphomas", Blood, 105, pp.3768~3785 (2005)
- van Dongen JJ, "Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98-3936", Leukemia, 17, pp.2257~2317 (2003)
- Willemze R, "Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases", Blood, 111, pp.838~845 (2008)
- Kong YY, "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, pp.1495~1502 (2008)
- Hahtola S, "Clinicopathological characterization and genomic aberrations in subcutaneous panniculitis-like T-cell lymphoma", J Invest Dermatol, 128, pp.2304~2309 (2008)
- Massone C, "Lupus erythematosus panniculitis (lupus profundus): clinical, histopathological, and molecular analysis of nine cases", J Cutan Pathol, 32, pp.396~404 (2005)
- Magro CM, "Atypical lymphocytic lobular panniculitis", J Cutan Pathol, 31, pp.300~306 (2004)
- Pincus LB, "Subcutaneous panniculitis-like T-cell lymphoma with overlapping clinicopathologic features of lupus erythematosus: coexistence of 2 entities", Am J Dermatopathol, 31, pp.520~526 (2009)
- Hoque SR, "Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients", Br J Dermatol, 148, pp.516~525 (2003)
- Massone C, "Subcutaneous, blastic natural killer (NK), NK", T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunophenotypic, and molecular study of 50 patients, 2004, pp.28, 719~735
- Gallardo F, "Subcutaneous panniculitic-like T-cell lymphoma and other primary cutaneous lymphomas with prominent subcutaneous tissue involvement", Dermatol Clin, 26, pp.529~540 (2008)
- Romero LS, "Subcutaneous T-cell lymphoma with associated hemophagocytic syndrome and terminal leukemic transformation", J Am Acad Dermatol, 34, pp.904~910 (1996)
- Magro CM, "Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia", J Cutan Pathol, 28, pp.235~247 (2001)
- Reimer P, "Subcutaneous panniculitis-like T-cell lymphoma during pregnancy with successful autologous stem cell transplantation", Ann Hematol, 82, pp.305~309 (2003)
- Mukai HY, "Successful treatment of a patient with subcutaneous panniculitis-like T-cell lymphoma with high-dose chemotherapy and total body irradiation", Eur J Haematol, 70, pp.413~416 (2003)
- Weenig RH, "Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature", Am J Dermatopathol, 23, pp.206~215 (2001)