PAMI (PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome) is a hereditary syndrome that may present with neutropenia during childhood. Misdiagnosis and delayed diagnosis are common.
PSTPIP1 = proline-serine-threonine phosphatase-interacting protein 1.
Patient selection: unexplained childhood neutropenia
Features that can be seen with PAMI:
(1) history of recurrent infections
(5) growth retardation
(1) variable anemia and/or thrombocytopenia
(3) elevated serum calprotectin
(4) elevated serum CRP
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