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Leukemia, monoblastic/monocytic , acute

Orpha number ORPHA514
Prevalence of rare diseases -
Inheritance -
Age of onset Childhood
ICD 10 code
  • C92.7
MIM number
Synonym(s) Acute myeloblastic leukemia, type 5

Summary

Acute myeloblastic leukemia (AML) (see this term) is a group of malignant bone marrow neoplasms of myeloid precursors of white blood cells. Acute monocytic leukemia (AML-M5) is one of the most common type of AML in young children (< 2 years). However, the condition is rare and represents approximately 2.5% of all leukemias during childhood and has an incidence of 0.8-1.1 per million per year. The symptoms may be aspecific: asthenia, pallor, fever, dizziness and respiratory symptoms. More specific symptoms are bruises and/or (excessive) bleeding, coagulation disorders (DIC), neurological disorders and gingival hyperplasia. Diagnostic methods include blood analysis, bone marrow aspirate for cytochemical, immunological and cytogenetical analysis, and cerebrospinal fluid (CSF) investigations. A characteristic translocation observed in AML-M5 is t(9;11). Treatment includes intensive multidrug chemotherapy and in selected cases allogeneic bone marrow transplantation. Nevertheless, outcome of AML remains poor with an overall survival of 35-60%. New therapeutics are required to increase the probability of cure in this serious disorder. *Author: Dr A. C. Verschuur (May 2004)*.

Detailed information

Review article
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