Patient is a 44 year old male with no prior comorbidity, who presented with rapidly progressing cervical and axillary lymphadenopathy, fever and multiorgan failure including acute kidney injury (AKI), acute liver failure, encephalopathy, and tumor lysis syndrome. Hemogram showed neutrophilic leukocytosis of 49.2 10³/uL(3.8-10.8 10³/uL) with anemia of 10.3g/dl(13.2-17.1 g/dl) and thrombocytopenia of 18 10³/uL(140-400 10³/uL). Peripheral smear showed marked leukocytosis with prominent granulocytes, and left shift. Other labs include very high ferritin > 7500 ng/ml(23.9-336.2 ng/ml), elevated triglyceride 236 mg/dl(10-149 mg/dl), and normal NK cell function.

He underwent excisional biopsy of axillary lymph nodes which was consistent with anaplastic large cell lymphoma (ALK positive). Bone marrow biopsy showed normocellular bone marrow with maturing trilineage hematopoiesis and rare ALK-positive cells, reactive changes with hemophagocytosis. Patient was diagnosed to have hemophagocytic lymphohistiocytosis(HLH) secondary to underlying lymphoma. 

He was started on ESAPH regimen (Etoposide, Solumedrol, Cytarabine and Cisplatin), as this also contains Etoposide from HLH-94 treatment protocol, with remarkable improvement in his mental status and organ function. 

Fig: Bone marrow aspirate smear (Wright-Giemsa, 1000x, oil immersion) shows macrophages with cytoplasmic cellular debris in  a background of trilineage hematopoiesis.