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Bone marrow biopsy showing multinucleated histiocytes (shown by arrows) with phagocytosed hematopoietic cells and debris consistent with the diagnosis of macrophage activation syndrome.

Bone marrow biopsy showing multinucleated histiocytes (shown by arrows) with phagocytosed hematopoietic cells and debris consistent with the diagnosis of macrophage activation syndrome.

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Macrophage activation syndrome (MAS) is a rare life-threatening complication that sometimes occurs in patients with systemic lupus erythematosus (SLE) and other connective tissue diseases. This syndrome is universally fatal without treatment, and therefore, prompt diagnosis and initiation of treatment is of vital importance.

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... on pancytopenia, transaminitis, hypertriglyceridemia, and hyperferritinemia, MAS was strongly suspected. Subsequently, a bone marrow biopsy was done which was consistent with MAS showing histiocytes with phagocytized hematopoietic cells and debris ( Figure 1). Immunohistochemistry and phenotyping of the bone marrow specimen did not reveal any evidence of leukemia or lymphoma. ...