Acute myelomonocytic leukemia
Synonyms: AML M4 · AMMoL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare acute myeloid leukemia disorder characterized by increased blast cells (myeloblasts, monoblast, and/or promonoblasts), representing more than 20% of the total bone marrow (BM) or peripheral blood differential counts, with 20-80% of BM cells being of monocytic lineage. Clinical presentation is the result of bone marrow involvement and extramedullary infiltration by the leukemic cells and includes asthenia, pallor, fever, dizziness, respiratory symptoms, easy bruising, bleeding disorders, and neurological deficits. Gingival hyperplasia, organomegaly, especially hepatosplenomegaly, and lymphadenopathy may also be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute myelomonocytic leukemia | AML M4 · AMMoL |
| fr | Leucémie aiguë myélomonocytaire | AML M4 · LAM M4 · Leucémie aiguë myélo-monocytaire |
| de | Myelomonozytenleukämie, akute | AML-M4 · AMMoL |
| es | Leucemia mielomonocítica aguda | LMA M4 · LMMA |
| it | Leucemia mielomonocitica acuta | Leucemia acuta mieloblastica, tipo 4 |
| nl | Acute myelomonocytaire leukemie | = en |
| pt | Leucemia mielomonocítica aguda | Leucemia mieloblástica aguda tipo 4 |
| pl | Ostra białaczka mielomonocytowa | AMMoL · AML M4 |
| cs | Akutní myelomonocytární leukémie | AML M4 |
| uk | Гострий мієломоноцитарний лейкоз | AML M4 · АММОЛ |
| zh | 急性粒-单核细胞白血病 | AML M4 |
| tr | Akut miyelomonositik lösemi | AMMoL · AML M4 |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C92.5 | E | specific | validated |
| ICD-11 | 2A60.33 | E | specific | validated |
| MONDO | 0018871 | E | validated | |
| MeSH | D015479 | E | validated | |
| UMLS | C0023479 | E | validated | |
| GARD | 529 | E | validated | |
| MedDRA | 10000890 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:167714Unclassified acute myeloid leukemia
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 1 000 000 | — | Europe | Class only | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.17 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
9 annotations · validated 2019-05-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001903 | Anemia | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0001873 | Thrombocytopenia | |
| Frequent (79-30%) | ||
| HP:0001892 | Abnormal bleeding | |
| HP:0002094 | Dyspnea | |
| HP:0000980 | Pallor | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0000168 | Abnormality of the gingiva | |
| HP:0001880 | Increased total eosinophil count | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 18508801[PMID]_29491461[PMID]_26185647[PMID]_6577285[PMID]_28371234[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/517
- This record as JSON: /data/d/517.json