Myelodysplastic neoplasm

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Myelodysplastic neoplasm with increased blasts type 1

ORPHA:100019Clinical subtype

Also called MDS-IB1 · RAEB-1 · Refractory anemia with excess blasts type 1

What it is

A severe type of RAEB characterized by cytopenias and the following hematological parameters: uni- or multilineage dysplasia, 5% to 9% blasts in bone marrow or 2% to 4% in peripheral blood, and no Auer rods (abnormal, needle-shaped or round inclusions in the cytoplasm of myeloblasts and promyelocytes). Median survival has been reported to be 18 months.

Key facts

Age of onset
Adult
Inheritance
Not applicable
Classified as
Clinical subtype

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (annual incidence, Europe)Myelodysplastic neoplasm with increased blasts

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Gene

TET2Disease-causing somatic mutation(s)

ICD-10 codes

D46.2filed under a broader ICD-10 category — shared with 3 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0015040UMLS C1318550

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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