Hemophagocytic lymphohistiocytosis secondary to T-cell Acute Lymphoblastic Leukemia with membranous tonsillitis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation, which is characterized by fever, hepatosplenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, and/or hypofibrinogenemia, and evidence of hemophagocytosis. Secondary HLH is often seen in adults and categorized based on autoimmune, infections-related, and malignancy-associated etiologies such as A-HLH, I-HLH, and M-HLH, respectively. This study presented a rare case of HLH developing concurrently at the time of diagnosis of T-cell Acute Lymphoblastic Leukemia (T- ALL) with a unique presentation of membranous tonsillitis in a 10-year-old boy. In all of the cases of T-ALL reported in the pediatric age group, HLH develops post-therapy or at the relapse. The first presentation of leukemia as membranous tonsillitis and concurrent clinic laboratory findings of HLH is rare and can mislead the diagnosis. Therefore, prompt diagnosis is the mainstay of therapy and can considerably improve the prognosis.