Clinical outcome of patients with subcutaneous panniculitis-like T-cell lymphoma

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cytotoxic T-cell lymphoma. The objective of this study was to characterize the clinical presentation, treatment, and prognosis of patients with SPTCL. Twenty-one patients with SPTCL were seen at Mayo Clinic (Rochester, Minnesota, USA) between July 1973 and June 2004. The median age at diagnosis was 42 years (range 23-80 years) and 15 (71%) were women. Constitutional symptoms occurred in 14 (67%) patients, including fever, serositis, arthralgias and myalgias. The Eastern Cooperative Oncology Group performance score was poor (3-4) in 3 (15%) patients. Liver enzymes (at least 2 enzymes, Aspartate aminotransferase (AST), alkaline phosphatase and/or lactate dehydrogenase) were elevated in 11 (52%) patients. Therapy consisted of chemotherapy in 13 (62%) patients, or other therapeutic interventions in 8 (38%) patients, including surgical excision, corticosteroids alone or in combination with either plaquenil, colchicine, hydroxychoroquine, or azathioprine. Bone marrow transplantation was performed in 5 (24%) patients, 3 autologous and 2 allogeneic. The median overall survival from diagnosis was 15 months (range 0.1-104 months). Two groups of patients were identified and categorized as having a favorable or unfavorable disease course. The factors associated with an unfavorable disease course were a low white blood cell count or elevated lactate dehydrogenase. Patients treated aggressively with stem cell transplantation appeared to have an improved overall survival.

Bone marrow involvement by subcutaneous Panniculitis-like T-cell lymphoma: a report of three cases

“…However, it was unclear whether this positive case was of the ab phenotype as their series included both ab and gd type cases, and the case described was published prior to the 2008 WHO classification. 10 In this study, we reported three cases of subcutaneous panniculitis-like T-cell lymphoma with focal bone marrow infiltration by lymphoma. None of the three bone marrow core biopsies exhibited large lymphocytic infiltrates.…”

Complete remission of subcutaneous panniculitic T-cell lymphoma after allogeneic transplantation

“…6,7 Reports in the literature describe remission with schedules such as CHOP and FMD. 2,3 However, our patient progressed in the third week after MEGACHOD and, although an initial response to FMD was observed, early progression occurred.…”

“…1 Characteristically, delay and difficulty in making a diagnosis are common features of SPTCL. [2][3][4][5] Misdiagnosis is not uncommon owing to the indolent course of the disease, which can mimic an unspecific panniculitis. 6 In our report three biopsies and 6 months were required before the final diagnosis could be made, by which time the lymphoma had become aggressive.…”

“…3,8,9 In an effort to overcome disease refractoriness, we used antiCD52 (Campath 1H) 10 immediately before the transplant (days À24 to À10) and TBI as conditioning, substituting VP16 for the cyclophosphamide because of the lack of sensitivity to cyclophosphamide observed with the MegaCHOD scheme. The patient achieved clinical remission and she is in CR 31 months post transplant.…”

Subcutaneous Panniculitis-like T-cell Lymphoma: Review of Therapies

“…Cutaneous lesions can occur with systemic symptoms such as fever, malaise, anorexia, and weight loss; they can also cause lipodystrophy after resolution. While extra-cutaneous involvement is uncommon, there have been reports of bone marrow, lymph node, liver, spleen, lung, peripheral blood involvement, and even spontaneous regression [2][3][4][5][6][7][8][9][10]. The average age of presentation is mid to late thirty with a female predominance (male: female=0.5) [2].…”