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Hemophagocytic Lymphohistiocytosis (HLH) With B-Cell Lymphoma: A Rare Lethal Cause for Fever of Unknown Origin FREE TO VIEW

Mohamed Abdelfatah, MD; Donald Dumford, MD; Christie Murphy, DO
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Akron General Medical Center, Northeast Ohio University, Akron, OH

Chest. 2014;146(4_MeetingAbstracts):195A. doi:10.1378/chest.1990053
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: HLH is a rare lethal disease caused by mutations in genes crucial to the cytolytic secretory pathway, resulting in induced apoptosis of target cells by perforin and granzymes(1). few patients worldwide are reported to have HLH associated with B-cell lymphoma (2-3).

CASE PRESENTATION: A 55-year-old Caucasian male with fevers (39.4C), fine resting tremors, and influenza- like symptoms. Two months prior he had a febrile illness, presumed to be viral, which persisted for two weeks. On admission, the patient was found to have dehydration, fever, and jaundice Laboratory studies demonstrated pancytopenia with Renal and hepatic failure. No specific cause of hepatic failure was identified despite extensive evaluation. Abdominal ultrasound and CT and MRI of the chest and abdomen revealed mild ascites with pleural effusion and hepatosplenomegaly. Subsequent Liver biopsy demonstrated acute hepatitis. An immunological and infectious disease work-up yielded negative results. The clinical picture then raised suspicion for HLH. A bone marrow biopsy demonstrated histiocytes without hemophagocytes. The patient was transferred to a tertiary center, where a brain biopsy demonstrated B-cell lymphoma. Unfortunately, the patient died shortly after the diagnosis.

DISCUSSION: The absence of hemophagocytosis from a bone marrow biopsy does not exclude the diagnosis of HLH. This case demonstrates that B-cell lymphoma should be investigated even after the diagnosis of HLH has been made. Secondary HLH is associated with infection, rheumatologic disorders and malignancy. HLH is diagnosed with one of two criteria; molecular diagnosis or the presence of five of the eight HLH criteria; fever, splenomegaly, pancytopenia, hypertriglyceridemia >3 times normal, hypofibrinogenemia, high ferritin, soluble CD 25 >2,400 U/mL and hemophagocytosis in BM, spleen, or lymph nodes. Findings supporting a diagnosis of HLH include neurological symptoms, cerebrospinal fluid pleocytosis, conjugated hyperbilirubinemia, transaminitis, and elevated D-dimer. Table 2

CONCLUSIONS: Although the association between HLH and B-cell lymphoma is rare, given its high mortality, should intensivist should carry a high index of suspicion to make the diagnosis. Especially with patients presenting with fever, pancytopenia and multiple organ failure after ruling out other more common causes.

Reference #1: Zuzana Tothova, MD, PhD1,2 and Nancy Berliner, MD1,2 Hemophagocytic Syndrome and Critical Illness: New Insights into Diagnosis and Management. Journal of Intensive Care Medicine 1-12, 2014

Reference #2: Falini B, Pileri S, De Solas I, et al. Peripheral T-cell lymphoma asso- ciated with hemophagocytic syndrome. Blood. 1990;75(2):434-444.

Reference #3: Miyahara M, Sano M, Shibata K, et al. B-cell lymphoma- associated hemophagocytic syndrome: clinicopathological char- acteristics. Ann Hematol. 2000;79(7):378-388.

DISCLOSURE: The following authors have nothing to disclose: Mohamed Abdelfatah, Donald Dumford, Christie Murphy

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