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acute myeloïde leukemie met megakaryoblastair fenotype met t(1;22)(p13.3;q13.1); RBM15-MKL1 (aandoening)
acute myeloïde leukemie met megakaryoblastair fenotype met t(1;22)(p13.3;q13.1); RBM15-MKL1
megakaryoblastaire AML met t(1;22)(p13.3;q13.1); RBM15-MKL1
Megakaryoblastic acute myeloid leukemia with t(1;22)(p13;q13)
A rare subtype of acute myeloid leukemia with recurrent cytogenetic abnormalities characterized by clonal proliferation of myeloid blasts with predominantly megakaryoblastic differentiation in the bone marrow and blood, often with extensive infiltration of the abdominal organs. It occurs typically in infants and usually presents with hepatosplenomegaly, anemia, thrombocytopenia and nonspecific symptoms related to ineffective hematopoesis (fatigue, bleeding and bruising, recurrent infections). Myelofibrosis and fibrosis of other infiltrated organs is also characteristic of this disease.
Id763796007
StatusDefined
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetC92.0
TermAcute myeloblastenleukemie [AML]
SNOMED CT to Orphanet simple map402023
SNOMED CT to ICD-10 extended map
TargetC94.2
RuleTRUE
AdviceALWAYS C94.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified