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What is 4yr old boy presenting with cryptorchidism, sho stature & on auscultation, harsh ejection systolic murmur is heard in pulmonary area. What is the hematological abnormality found in this condition? and explain in detail?
The history given in the question is suggestive of Noonan syndrome which is associated with Juvenile Myelomelanocytic leukemia; JMML. JMML BCR/ABL negative entity Regarded as MDS/MPN syndrome Seen in children aged <= 4 years An aggressive disease characterized by proliferation of granulocytic and monocytic series. Peripheral blood: Leukocytosis with neutrophilia, monocytosis, nucleated red blood cells Bone marrow: Hypercellular with granulocytic hyperplasia; Monocytes, 5-30%; Megakaryocyte decreased; Blasts + promonocytes <20% Dysplasia, not prominent; Reticulin fibrosis Associated with Neurofibroma type 1 (NF 1) Noonan syndrome Trisomy 8 mosaicism