[CIS-PAGID] Puzzling Patient #2

[Church, Joseph]

I was just asked to consult on a 9yo boy with a 1 month history of progressive bilateral parotitis associated with spiking fevers.  Multiple features of his case suggested macrophage activation syndrome (MAS) although he did not have organomegaly.  Bone marrow showed hypocellularity with no evidence of malignancy.  Lymph node biopsy showed non-granulomatous necrotizing adenitis with preserved T and B cell populations, and lots of macrophages.

Serologic studies demonstrated strongly positive IgG and IgM against mumps virus.

His past medical records document that he was fully immunized (x2) with MMR.

Has anyone seen

*         mumps virus-associated MAS?

*         familial hemophagocytic lymphohistiocytosis presenting with mumps?

Why did this kid get mumps?

Joe Church
Children's Hospital Los Angeles




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