TCL-026: Subcutaneous Panniculitis–Like T-Cell Lymphoma: A Rare Case

Abstract

Context: Subcutaneous panniculitis-like T-cell lymphoma (SCPTCL), is an uncommon variant and poorly differentiated type of T-cell lymphoma with nonspecific clinicopathologic features and associated with an aggressive clinical course. CHOP-like chemotherapy is generally used as an initial therapy with different outcomes. Objective: To describe the clinical course and manifestations of one patient. Design: Case report Setting: Hematology Department at Hospital Calderon Guardia. Patients or other participants: A young adult male diagnosed with SCPTCL associated hemophagocytic syndrome. Interventions: A 19-year-old healthy male presented an acute history of fever, constitutional symptoms and diffused swelling of the right arm. Once hospitalized, a complete test for FUO was negative. Bicytopenia and nephrotic syndrome documented. Bone marrow studies, kidney and muscle biopsy were performed. No regional lymph node involvement, only hepatosplenomegaly was registered by CT scans. Results: Patient got worse. Both his eyes and right arm swelled drastically, and fever continued. Biopsy of the muscle showed an atypical cell infiltrate composed by intermedium size cells with scarce cytoplasm, irregular and hyperchromatic nuclei. Neoplastic cells produced a septal and interstitial panniculitis with fat necrosis. Macrophages and hemophagocytosis were also identified. Immunohistochemistry analysis showed that tumor cells expressed CD3 and CD8 but were negative for CD20, CD4, and CD56. Ki-67 80%. These findings were consistent with the diagnosis of SCPTCL (α/β subtype). Bone marrow studies reveled lymphoma infiltration and hemophagocytosis. Paraneoplastic membranous glomerulonephritis was documented in kidney biopsy. CHOP protocol was initiated. Fever disappeared. Eyes and arm swelling decreased. Nevertheless, patient had a short response with progression of the symptoms, so the protocol was changed to ESHAP. After one cycle the patient had a remarkable response. Right arm edema disappeared completely and eye swelling had a significant improvement. All studies for peripheral blood stem cell transplantation (PBSCT) were initiated. Currently, patient is receiving his second cycle of ESHAP. Our initial approach is to achieve a full response to consequently proceed with PBSCT. Conclusions: SCPTCL is an uncommon disease and 2 subtypes have been described, TCR α/β and TCR γ/δ. The clinical manifestations are different but not specific to either. Outcomes depend on clinical and pathology factors. There is no standardized treatment.