Transient myeloproliferative disorder

BushraMoiz andMaria Shafiq, TheAgaKhanUniversity

An 11-day-old boy presented with high-grade fever since the second day of life. He was dysmorphic with Downsyndrome (DS) facies, pallor, irritability, tachypnea, pan-systolic murmur, and hepatosplenomegaly. Hemoglobin was98 g/L, white cells were 254 109/L, and platelets were 31 109/L. Peripheral blood film (see figure) displayed 95% blastcells having deeply basophilic cytoplasm with blebs, multiple nucleoli, low platelets, and a few target cells. Liver enzymesand creatinine were normal. Circulating blasts were CD34, CD36, CD117, CD13, CD33, and MPO. Trisomy 21 wasthe sole cytogenetic abnormality. Supportive treatment was initiated. Leukapheresis and low-dose Ara-C were planned;however, the parents refused treatment and took the child out of the hospital. Transient myeloproliferative disorder (TMD)remained unresolved and the neonate soon succumbed to cardiorespiratory failure.TMD was the presumed diagnosis because the child was a neonate with hepatomegaly and blasts with trisomy 21 with noother chromosomal abnormalities. TMD is a unique hematologic feature seen in 10% of neonates with DS. Its clinical,morphologic, and phenotypic features are indistinguishable from acute megakaryocytic leukemia (AMKL) but the blastsuniversally show trisomy 21 in TMD. Spontaneous remissions occur often but fatal forms are seen in 16% to 23% of cases.

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workbloodI M A G E S I N H E M A T O L O G Y

4672 BLOOD, 6 DECEMBER 2012 VOLUME 120, NUMBER 24

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doi:10.1182/blood-2012-07-4409172012 120: 4672

Bushra Moiz and Maria Shafiq

Transient myeloproliferative disorder

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