Recent Findings on Juvenile Myelomonocytic Leukemia: a Case Report

Document Type : Original Article

Author

Depatment of Hematology – Oncology,Mashhad University of Medical Sciences, Mashhad, Iran.

Abstract

Background and Objective: Juvenile myelomonocytic leukemia (JMML) is a chronic, fatal and aggressive myeloproliferative neoplasm of childhood that occurs in 1.2 per million. JMML is a clonal disorder characterized by over proliferation of one or more lineages that retain the ability to differentiate. Because of rarity of the disease and the different kind of clinical findings the diagnosis of JMML is difficult for the most physicians. JMML is clinically characterized by the overproliferation of monocytic cells that can infiltrate organs, including the liver, spleen, lung. This paper tries to make a comprehensive survey on the diagnosis and treatment of the disease.
Case Report: A 4-year-old boy presented with hepatosplenomegaly, pallor, fever and respiratory distress. He also presented with myeloid precursors in peripheral blood and a white blood cell count >10Í109/L, peripheral blood monocyte count > 1 Í109 /L, bone marrow blasts < 20% with no Philadelphia chromosome was identified. Fetal haemoglobin (HbF) was within the normal range. The patient responded completely to chemotherapy, but the disease recurred later. Bone marrow transplantation (BMT) was carried out but the patient expired due to infection complications associated with transplantation..
Conclusion: Although chemotherapy protocols, which induce maturation and differentiation, are used as a temporary treatment, BMT is the definite treatment for JMML. BMT is the only treatment which able to cure JMML, but the high relapse rate is of great concern and it may be unavailable anywhere, and patient selection for this method need to be carefully undertaken. Recent advances in diagnosis and treatment of JMML will be discussed here.

Keywords

References

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Jundishapur Scientific Medical Journal
Volume 12, Issue 6 - Serial Number 87
July and August 2013
Pages 441-450

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History

  • Receive Date: 27 May 2013
  • Revise Date: 11 August 2013
  • Accept Date: 24 September 2013

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