Unusual case of subcutaneous panniculitis‐like T‐cell lymphoma

Abstract

An unusual case of subcutaneous panniculitis-like T-cell lymphoma is presented involving multiple organ systems, which eventually culminated in rapid demise from the haemophagocytic syndrome, after an initial protracted course. A 44-year-old man presented in April 2001 with bronchiolitis obliterans organising pneumonia that initially responded well to corticosteroids. However, the condition relapsed on attempted prednisone withdrawal in January 2002 and the patient was noted to have developed truncal subcutaneous nodules. Initial skin biopsy revealed lobular panniculitis, with negative microbiological culture. In July 2002, mononeuritis multiplex was diagnosed after the patient presented with paresthesiae and was treated with pulse cyclophosphamide therapy. By November 2002 there was ulceration of the subcutaneous nodules. Repeat skin biopsy revealed subcutaneous panniculitis-like T-cell lymphoma. The clinical manifestations were supportive of an unifying diagnosis of malignancy involving pulmonary, cutaneous and nervous systems. Combination chemotherapy with fludarabine, mitoxantrone and dexamethasone was commenced. However, the patient deteriorated, with fevers, weight loss, pancytopenia and laboratory features consistent with the haemophagocytic syndrome. Despite maximal supportive therapy the patient succumbed to his disease.

Subcutaneous Panniculitislike T-Cell Lymphoma Appearing as a Breast Mass

“…6 Subcutaneous panniculitislike T-cell lymphoma is an uncommon type of CTCL with fewer than 100 cases reported worldwide. 7 It affects a broad age group, including children and young adults, with variable outcome. The mean survival has been reported as less than 3 years, with most cases having an aggressive course.…”

Use of Fludarabine-Containing Chemotherapeutic Regimen Results in Durable Complete Remission of Subcutaneous Panniculitis-Like T-Cell Lymphoma

“…[11] In another study, however, the efficacy of fludarabine was notype should be distinguished from those expressing the γ/δ reduced when used as salvage treatment for refractory SPTCL. [14] phenotype, as the latter is classified provisionally as cutaneous γ/δ Whether the efficacy of fludarabine was related to the TCR T-cell lymphoma by the WHO-EORTC criteria. [5] However, pracphenotype of α/β or γ/δ remains unclear.…”

“…[6] The occurthat fludarabine might be effective in SPTCL (table I). [11][12][13][14] To rence of systemic symptoms or hemophagocytosis does not always date, four patients with SPTCL have been reported to have been differentiate benign cases from lymphomatous cases. [6] In uncertreated with fludarabine as first-line treatment.…”

Subcutaneous Panniculitis‐Like T‐Cell Lymphoma With Hemophagocytic Syndrome Successfully Treated With Cyclosporin A

“…SPTCL is a rare form of peripheral T‐cell lymphoma 3 . It is being increasingly recognized, having now been incorporated as a distinct entity in the recent World Health Organization classification of lymphomas, but it remains an uncommon type of cutaneous T‐cell lymphoma, with fewer than 100 cases reported worldwide 4 . Most cases arise in young adults, and there is a slight preponderance in women (1.5:1) compared with men 3 .…”

“…If cyclosporine fails, or if disease progression occurs while on cyclosporine, or if there is clear evidence of SPTCL without HPS, however, then chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone [CHOP] or a CHOP‐based regimen) appears to be the best treatment option. High‐dose chemotherapy with autologous peripheral stem cell transplantation should be considered if relapse occurs following chemotherapy 4 . Most patients with SPTCL follow an aggressive clinical course due to HPS; however, some patients follow a protracted or indolent course 2 .…”