TY - JOUR
T1 - Malignant osteopetrosis manifested as juvenile chronic myeloid leukemia
AU - Toren, A.
AU - Neumann, Y.
AU - Meyer, J. J.
AU - Mandel, M.
AU - Schiby, G.
AU - Kende, G.
AU - Bassat, I.
AU - Rechavi, G.
PY - 1993
Y1 - 1993
N2 - 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
AB - 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
UR - http://www.scopus.com/inward/record.url?scp=0027197223&partnerID=8YFLogxK
U2 - 10.3109/08880019309016556
DO - 10.3109/08880019309016556
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AN - SCOPUS:0027197223
SN - 0888-0018
VL - 10
SP - 187
EP - 189
JO - Pediatric Hematology and Oncology
JF - Pediatric Hematology and Oncology
IS - 2
ER -