CASE REPORT
A fatal case of primary cutaneous gamma–delta T-cell lymphoma complicated by HLH and cardiac amyloidosis Juliet Fraser Gibson1, Lucy Kapur2, Joseph Sokhn1, Mina Xu2 & Francine M. Foss3 1
Department of Medicine, Yale University School of Medicine, New Haven, Connecticut Hematopathology, Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 3 Hematology and Bone Marrow Transplantation, Yale University School of Medicine, New Haven, Connecticut 2
Correspondence Juliet Fraser Gibson, Hematology and Bone Marrow Transplantation, Yale University School of Medicine, New Haven, CT. Tel: 203-200-4363; Fax: 203-200-6385; E-mail:
[email protected] Funding Information No funding for this project. Received: 10 February 2014; Revised: 1 April 2014; Accepted: 20 May 2014 Clinical Case Reports 2015; 3(1): 34–38
Key Clinical Message Gamma–delta T-cell lymphomas (GD-TCL) are rare and rapidly fatal neoplasms that are often associated with Hemophagocytic Lymphohistiocytosis (HLH), a syndrome of fevers, cytopenias, and multiorgan failure that often leads to a rapid death. We report the first case demonstrating an association between GD-TCL, HLH, and cardiac amyloidosis, presenting a novel mechanism for rapid deterioration in these patients. Keywords Amyloidosis, cutaneous T-cell lymphoma, gamma–delta, hemophagocytic lymphohistiocytosis, primary cutaneous gamma–delta T-cell lymphoma.
doi: 10.1002/ccr3.142
Background Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms, representing only 10–15% of non-Hodgkin’s lymphoma, with an incidence of approximately 1.79/ 100,000 persons per year [1]. Gamma–delta T-cell lymphomas (cd TCLs) arise from immature T cells with cd T-cell receptors (TCR). cd T-cells comprise only 2–5% of peripheral T-cells, but appear to have a role in both the innate and adaptive immune systems, with both cytotoxic and regulatory properties [2, 3, 4]. cd TCLs represent a minority of these lymphomas, though they have been shown to have an aggressive course and portend a very poor prognosis [5]. The most recent WHO-EORTC classification identifies two major subtypes of cd TCLs, primary cutaneous gamma–delta Tcell lymphoma (PCGD-TCL), and hepatosplenic T-cell lymphoma (HSTL) [6]. PCGD-TCL is estimated to represent approximately 1% of cutaneous T-cell lymphomas (CTCLs) and HSTL is estimated to represent
