#00065663

An 18-year-old male presented with high fever and jaundice for 4 days duration and anuria for 24 hrs. Examination revealed tachycardia, hypotension (BP 102/64mm Hg), pallor, icterus and splenomegaly (2 cm below left subcostal margin). Investigations revealed Hemoglobin 99g/L; Total leucocyte count: 6.5x10³/uL; Absolute neutrophil count: 4.3x10³/uL; Platelets 23x10³/uL; Serum bilirubin 11.5mg/dl; AST: 228IU/L; ALT: 125IU/L; Serum creatinine: 3.1mg/dl. C-reactive protein 10.1; Blood culture was sent, and due to declining peripheral blood counts, the bone marrow aspirate was done, which showed cellular reactive bone marrow with hemophagocytosis.

No hemoparasite/ abnormal lymphocytes/blasts were present. Further investigations revealed Fibrinogen 120mg/dL; Triglyceride 359mg/dL; Serum ferritin 2718mg/dL; The diagnosis of HLH was offered (HLH2004 score 6/8 and HScore: 294). Salmonella typhae came positive in blood culture. The patient was managed with antimicrobials, and supportive treatment. He responded well to the treatment and was discharged.

Hemophagocytosis refers to the engulfment of hematopoietic cells by histiocytes, and can be seen in the bone marrow, lymph node and spleen. Although hemophagocytosis is just morphological evidence of HLH in the marrow, it is neither specific nor sensitive for HLH diagnosis. The presence of hemophagocytosis in the bone marrow supports the diagnosis of HLH.