Malignant osteopetrosis manifested as juvenile chronic myeloid leukemia

A. Toren*, Y. Neumann, J. J. Meyer, M. Mandel, G. Schiby, G. Kende, I. Bassat, G. Rechavi

*Corresponding author for this work

Research output: Contribution to journalLetterpeer-review

14 Scopus citations

Abstract

Juvenile chronic myelogenous leukemia (JCML) is a clonal panmyelopathy associated with an elevated leukocyte count with a relatively high proportion of myelomonoblasts, normoblasts, lymphocytes and monocytes, splenomegaly, and a decreased or normal LAP score. Bone marrow shows erythroid and myeloid hyperplasia. JCML is distinguished from adult chronic myelogenous leukemia (CML), both clinically and by laboratory tests. Clinically, the patients suffer from typical rashes, organomegaly, including the liver, spleen and lymph nodes, bleeding phenomena, and a relatively rapid course. The typical laboratory findings are elevated fetal hemoglobin, immunodeficiency, thrombocytopenia, and leukocytosis with prominent involvement of the monocytic series and an absence of Ph chromosome or bor rearrangement.1,2 A selective increased response to granulocyte macrophage colony stimulating factor (GM-CSF) by hematopoietic cells appears to be involved in the pathogenesis of the disease.3

Original languageEnglish
Pages (from-to)187-189
Number of pages3
JournalPediatric Hematology and Oncology
Volume10
Issue number2
DOIs
StatePublished - 1993

Fingerprint

Dive into the research topics of 'Malignant osteopetrosis manifested as juvenile chronic myeloid leukemia'. Together they form a unique fingerprint.

Cite this