LB959 Hemophagocytic lymphohistiocytosis in cytotoxic cutaneous T-cell lymphoma: Delayed diagnosis of a life-threatening complication

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation. Numerous cases of HLH in patients with cutaneous T-cell lymphoma (CTCL) have been reported; yet, a standardized treatment regimen for these patients has not been established. The aim of this study was to assess the clinical response of patients with CTCL and HLH who were managed at our institution. We performed a retrospective review of patients with CTCL who developed HLH. From the medical records of qualifying patients, we obtained clinical and laboratory data. Six patients were selected from 1370 CTCL patients. Five developed HLH after the diagnosis of CTCL and 1 presented with CTCL and HLH simultaneously. The most frequent subtypes of CTCL had a cytotoxic phenotype (including primary cutaneous gamma-delta; subcutaneous panniculitis-like; and CD4-/CD8- CTCL, not otherwise specified). The most frequent presenting HLH symptoms were fever and fatigue. Median time from first HLH symptoms to diagnosis of HLH was 1 month (range 0.1-3.6 months). Steroids, cyclosporine, and etoposide were the most common treatments. The outcomes of these patients are: 3 dead of disease, 2 alive with disease, and 1 alive without disease (AWOD). Our study highlights the association of HLH with certain cytotoxic lymphomas and the variability in treatment regimens used to manage HLH in patients with CTCL, and stresses the need for a standardized regimen. Of note, the patient who is AWOD received an allogeneic stem cell transplant; this outcome has been seen in other published cases of CTCL and HLH managed with a transplant. Given that HLH is often fatal and that there were delays in diagnosing HLH in our patients, any clinical suspicion of HLH, especially in patients with fever and fatigue, may warrant a comprehensive workup.