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IMAGES

IN

HEMATOLOGY

Transient myeloproliferative disorder

Bushra Moiz and Maria Shafiq, The Aga Khan University

n 11-day-old boy presented with high-grade fever since the second day of life. He was dysmorphic with Down
A
syndrome (DS) facies, pallor, irritability, tachypnea, pan-systolic murmur, and hepatosplenomegaly. Hemoglobin was
98 g/L, white cells were 254 10 /L, and platelets were 31 10 /L. Peripheral blood film (see figure) displayed 95% blast
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cells having deeply basophilic cytoplasm with blebs, multiple nucleoli, low platelets, and a few target cells. Liver enzymes
and creatinine were normal. Circulating blasts were CD34, CD36, CD117, CD13, CD33, and MPO. Trisomy 21 was
the 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 no
other 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 blasts
universally show trisomy 21 in TMD. Spontaneous remissions occur often but fatal forms are seen in 16% to 23% of cases.
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4672

BLOOD, 6 DECEMBER 2012 VOLUME 120, NUMBER 24

From www.bloodjournal.org by guest on July 29, 2015. For personal use only.

2012 120: 4672


doi:10.1182/blood-2012-07-440917

Transient myeloproliferative disorder


Bushra Moiz and Maria Shafiq

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