A CASE OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN THE SETTING OF CLASSIC HODGKIN LYMPHOMA, HIV, AND EBV W. Gibson DO, P. Zito MD Department of Internal Medicine, LSU Health Sciences Center, New Orleans, LA
INTRODUCTION: Hemophagocytic Lymphohistiocytosis (HLH) is characterized by over activation and proliferation of CD8+ T cells, histiocytes, and macrophages. This results in systemic inflammation and phagocytosis of various cell lines. Five out of eight criteria (recurrent fevers, cytopenia, splenomegaly, hypertriglyceridemia, hemophagocytosis in bone marrow or spleen or lymph nodes, elevated ferritin (> 500), elevated soluble CD25 (>2,400 U/ml), and low or absent natural killer cell activity) are required for diagnosis. CASE: A twenty-seven-year-old man with untreated HIV presented to the Emergency Department with syncope. He also reports experiencing chills, fevers, diaphoresis, and weakness over the past month. Initial labs revealed a hemoglobin of 5.6g and platelets 81. Subsequent labs revealed a ferritin of 5,825.7, triglycerides of 370 mg/dl, soluble IL-2 at 21,540 pg/ml, and HIV viral load of 267,742. CT scan demonstratedmild enlargement of the spleen and several enlarge abdominal and pelvic lymph nodes. Recurrent fevers up to 103 0 F were documented. The patient met six out of eight criteria for the diagnosis of HLH. A bone marrow biopsy revealed an atypical CD30 positive lymphoid infiltrate, suspicious for lymphoproliferative disorder. The CD30+ cells were large-sized, most were mononuclear and some were binuclear with large nuclei and conspicuous nucleoli. Atypical, large-sized lymphoid cells were positive for Epstein Barr virus RNA. Flow cytometry was significant for a mix of T, B and NK cells with T cells predominating. A left cervical lymph node biopsy revealed classic Hodgkin lymphoma, nodular sclerosis type. DISCUSSION: HLH can be acquired genetically (primary) or environmentally (secondary). Mutations in the genes PRF1, MUNC 13–4, and STX11 are associated with primary HLH. Secondary HLH is often triggered by autoimmune disorders or viral infections. The most common trigger for HLH is an infection with Epstein-Barr virus. HLH is often fatal making prompt diagnosis and treatment of great significance.
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