Juvenile myelomonocytic leukemia: report of seven cases and review of literature

Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
Latha UrsSamir B Kahwash

Abstract

Juvenile myelomonocytic leukemia (JMML) is a rare, aggressive, clonal hematopoietic disorder of childhood with features of both myelodysplasia (thrombocytopenia, anemia) and myeloproliferation (leukocytosis, monocytosis). In most cases there is marrow hypercellularity, splenomegaly, and extramedullary involvement. In 1997 an international consensus on terminology was reached and guidelines/criteria for diagnosis were proposed. A recent World Health Organization classification described the current diagnostic criteria of JMML. Although the diagnosis of JMML has been facilitated, it can be challenging, especially in the early stages or when it 1st presents as an extramedullary tumor. We report a series of 7 cases diagnosed over a period of 10 years (from January 1, 1996, to December 31, 2005). Two cases had interesting associated findings that would potentially lead to delay in diagnosis or misdiagnosis. Two other cases had extramedullary involvement with symptoms referable to the organs of involvement at presentation. Clinical and pathologic findings are summarized with a review of relevant literature.

References

Sep 30, 2004·Journal of Pediatric Hematology/oncology·Atsushi ManabeUNKNOWN MDS Committee of the Japanese Society of Pediatric Hematology
Feb 5, 2005·American Journal of Clinical Pathology·Robert W McKenna
Apr 20, 2005·Journal of Pediatric Hematology/oncology·Adam GassasYigal Dror
Sep 29, 2005·Hematology·Christian P Kratz, Charlotte M Niemeyer
Nov 24, 2005·Hematology·Catherine P M Hayward
Nov 11, 2006·Leukemia·A NencioniP Brossart
Jan 17, 2007·Current Opinion in Pediatrics·Henrik Hasle
Jul 10, 2007·Leukemia Research·Anna Janik-MoszantTomasz Szczepański
Nov 15, 2008·Blood·Soheir S AdamCharles S Greenberg
Oct 1, 2004·Journal of Pediatric Hematology/oncology·Atsushi ManabeUNKNOWN MDS Committee of the Japanese Society of Pediatric Hematology

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Citations

Nov 26, 2013·Pediatrics and Neonatology·Ming-Luen LeeWen-Jue Soong
May 17, 2011·Journal of Pediatric Hematology/oncology·Britt GustafssonHenrik Hasle
Apr 14, 2017·Neuropathology and Applied Neurobiology·Q ZhangL-C Ang

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Datasets Mentioned

BETA
GM-CSF

Methods Mentioned

BETA
biopsy
biopsies
electrophoresis
flow cytometry

Software Mentioned

JMML

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