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August 2016

Cutaneous Hemophagocytic LymphohistiocytosisBean Bags From the Bone

Author Affiliations
  • 1Department of Dermatology, University of California–Davis, Sacramento
  • 2Department of Pathology, University of California–Davis, Sacramento
JAMA Dermatol. 2016;152(8):950-952. doi:10.1001/jamadermatol.2016.0978

We report a case of hemophagocytic lymphohistiocytosis (HLH) in which the histologic features of “bean bag cells” found in the skin biopsy specimen facilitated the diagnosis of this rare disorder.

A 15-year-old previously healthy boy presented with fever, nausea, and right upper quadrant abdominal pain, all of 1 week’s duration. He was found to have thrombocytopenia and leukopenia.

Magnetic resonance imaging and computed tomography (CT) scans showed multiple focal enhancing lesions throughout the lungs, kidneys, and liver. Subsequent positron emission tomography–CT scans revealed abnormal foci of increased uptake in the bone marrow, mandible, scapulae, ribs, pelvis, liver, lung, and kidneys (Figure 1A). Levels of lactate dehydrogenase, alkaline phosphatase, aspartate transaminase, direct bilirubin, triglycerides, ferritin, and D-dimer were elevated. The fibrinogen level was low. Findings for hepatitis A, B, and C, Epstein-Barr virus, coccidioidomycosis, cytomegalovirus, and human immunodeficiency virus were negative. Likewise, findings of viral respiratory panel, aspergillus galactomannan enzyme immunoassay, blood cultures, and bacterial, mycobacterial, and fungal tissue cultures were all negative.

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