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IMAGES IN HEMATOLOGY

Hemophagocytic lymphohistiocytosis and

disseminated histoplasmosis

c Alina M. Huang, Brown University

A 25-year-old Guatemalan man presented to the hospital with abdominal distension and fever. A computed
tomography scan showed marked hepatosplenomegaly and bulky abdominal adenopathy. A complete blood
count showed pancytopenia: white blood count, 7.7 3 109/L; hemoglobin, 7.0 g/dL; platelets, 48 3 109/L. White
blood cell inclusions were present. Further work-up revealed AIDS with a CD4 count of 4 cells/mL. Evaluation of his
anemia revealed a ferritin level of 85 000 ng/mL, which was a level that prompted consideration of hemophagocytic
lymphohistiocytosis (HLH) as a unifying diagnosis. He fulfilled 5 of 8 diagnostic criteria: fever $38.5°C,
splenomegaly, $2 cytopenias, ferritin level .500 ng/mL, and evidence of hemophagocytosis in the bone marrow.
Prominent clusters of Histoplasma capsulatum were seen disseminated throughout the marrow aspirate (panel A).
Serologic testing for Histoplasma antigen was positve. Hemophagocytosis was seen (panel B), in which a histiocyte is
engulfing a red blood cell and several Histoplasma organisms.
HLH is a syndrome of pathologic immune activation characterized as primary or secondary in etiology. This patient
had HLH secondary to disseminated histoplasmosis in the setting of severe immune deficiency. He received
dexamethasone for treatment of HLH, as well as highly active antiretroviral therapy and antifungal treatment to treat
the underlying infectious etiology. He recovered without incident and is doing well.

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BLOOD, 17 APRIL 2014 x VOLUME 123, NUMBER 16 2449

 From www.bloodjournal.org by guest on September 9, 2019. For personal use only.

2014 123: 2449

doi:10.1182/blood-2014-01-550657

Hemophagocytic lymphohistiocytosis and disseminated histoplasmosis

Alina M. Huang

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