Autosomal dominant hereditary disorder
Hereditary cancer-predisposing syndrome
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DEAD-box helicase 41-related hematologic malignancy predisposition syndrome (disorder)
DDX41-related hematologic malignancy predisposition syndrome
DEAD-box helicase 41-related hematologic malignancy predisposition syndrome
A rare inherited cancer-predisposing syndrome characterized by adult onset of hematologic malignancies mainly affecting the myeloid lineage (such as myelodysplastic syndrome and/or acute myeloid leukemia), less frequently lymphoid malignancies. Some patients have been reported to develop granulomatous or immune disorders (including sarcoidosis, systemic lupus erythematosus, asthma, eczema, or juvenile arthritis) before or in the absence of hematologic malignancies.
syndroom van DDX41-gerelateerde predispositie voor hematologische maligniteit
syndroom van 'DEAD-box helicase 41'-gerelateerde predispositie voor hematologische maligniteit
Id1172625004
StatusPrimitive
ICD-10 complex map reference set
TargetC96.7
RuleTRUE
AdviceALWAYS C96.7 | 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