Clinical and Pathological Characteristics and Outcomes Among Patients With Subcutaneous Panniculitis-like T-Cell Lymphoma and Related Adipotropic Lymphoproliferative Disorders.

Autor:	Guitart J; Department of Dermatology and Pathology, Northwestern University, Chicago, Illinois., Mangold AR; Department of Dermatology, Mayo Clinic Scottsdale, Scottsdale, Arizona., Martinez-Escala ME; Department of Dermatology, University of Chicago Medical Center, Chicago, Illinois., Walker CJ; Department of Dermatology and Pathology, Northwestern University, Chicago, Illinois., Comfere NI; Department of Dermatology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota., Pulitzer M; Medicine and Pathology, Memorial Sloan-Kettering Hospital, New York, New York., Rieger KE; Department of Dermatology and Pathology, Stanford University, Palo Alto, California., Torres-Cabala CA; Department of Dermatology and Pathology, University of Texas MD Anderson Cancer Center, Houston., Pincus LB; Department of Dermatology, University of California, San Francisco., Kumar ES; Medicine and Pathology, Memorial Sloan-Kettering Hospital, New York, New York., Wang EBK; Department of Dermatology and Pathology, Stanford University, Palo Alto, California., Park KE; Department of Dermatology and Pathology, University of Texas MD Anderson Cancer Center, Houston., Espinosa ML; Department of Dermatology and Pathology, Northwestern University, Chicago, Illinois., Duvic M; Department of Dermatology and Pathology, University of Texas MD Anderson Cancer Center, Houston., Kim YH; Department of Dermatology and Pathology, Stanford University, Palo Alto, California., Horwitz S; Medicine and Pathology, Memorial Sloan-Kettering Hospital, New York, New York.
Jazyk:	angličtina
Zdroj:	JAMA dermatology [JAMA Dermatol] 2022 Oct 01; Vol. 158 (10), pp. 1167-1174.
DOI:	10.1001/jamadermatol.2022.3347
Abstrakt:	Importance: There is a knowledge gap about subcutaneous panniculitis-like T-cell lymphoma (SPTCL) owing to its rarity and diagnostic difficulty, resulting in an absence of well-documented large case series published to date. Objective: To generate consensus knowledge by a joint multi-institutional review of SPTCL and related conditions. Design, Setting, and Participants: This retrospective clinical and pathological review included cases initially diagnosed as SPTCL at 6 large US academic centers. All cases were reviewed by a group of pathologists, dermatologists, and oncologists with expertise in cutaneous lymphomas. Through a process of group consensus applying defined clinical and pathological diagnostic criteria, the cohort was classified as (1) SPTCL or (2) adipotropic lymphoproliferative disorder (ALPD) for similar cases with incomplete histopathological criteria for SPTCL designation. Exposures: Cases of SPTCL diagnosed between 1998 and 2018. Main Outcomes and Measures: The main outcome was disease presentation and evolution, including response to therapy, disease progression, and development of hemophagocytic lymphohistiocytosis. Results: The cohort of 95 patients (median [range] age, 38 [2-81] years; female-to-male ratio, 2.7) included 75 cases of SPTCL and 20 cases of ALPD. The clinical presentation was similar for both groups with multiple (61 of 72 [85%]) or single (11 of 72 [15%]) tender nodules mostly involving extremities, occasionally resulting in lipoatrophy. Hemophagocytic lymphohistiocytosis (HLH) was only observed in SPTCL cases. With a mean follow-up of 56 months, 60 of 90 patients (67%) achieved complete remission with a median (range) of 3 (1-7) cumulative therapies. Relapse was common. None of the patients died of disease progression or HLH. Two patients with ALPD eventually progressed to SPTCL without associated systemic symptoms or HLH. Conclusions and Relevance: In this case series of patients initially diagnosed as having SPTCL, results showed no evidence of systemic tumoral progression beyond the adipose tissue. The SPTCL experience in this study confirmed an indolent course and favorable response to a variety of treatments ranging from immune modulation to chemotherapy followed by hematopoietic stem cell transplantation. Morbidity was primarily associated with HLH.
Databáze:	MEDLINE
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