Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative, in a 5-Month Baby - ARare Presentation.

J Coll Physicians Surg Pak

Department of Haematology and Transfusion Medicine, The Children's Hospital and Institute of Child Health, Lahore.

Published: November 2016

AI Article Synopsis

  • Atypical chronic myeloid leukemia (aCML) is a rare and serious blood cancer with a poor outlook, marked by abnormal blood cell production and lack of the BCR-ABL1 gene.
  • The disease usually develops in older adults, but there have been rare instances in children, including a reported case of a 5-month-old baby.
  • The diagnosis in this case was confirmed using the World Health Organization's criteria from 2008, highlighting the need for proper identification of the disease.

Article Abstract

Atypical chronic myeloid leukemia (aCML) is a neoplasm with poor prognosis, characterized by myeloid hyperplasia, dysmyelopoiesis and absence of BCR-ABL1 gene. Clinically, the disease course may be similar to chronic myeloid leukemia (CML), BCR-ABL1 positive. It presents in seventh to eighth decade of life with few cases of paediatric aCML being reported. Here, we report a case of aCMLin a 5-month baby who presented with massive splenomegaly. The diagnosis was in accordance with the WHO criteria established in 2008.

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