Acute monoblastic/monocytic leukemia
Synonyms: AML M5 · Acute monoblastic or monocytic leukemia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A form of acute myeloid leukemia that is either comprised of more than 80% of monoblasts or 30-80% monoblasts with (pro)monocytic differentiation. It presents with asthenia, pallor, fever, and dizziness. Specific features include hyperleukocytosis, propensity for extramedullary infiltrates, coagulation abnormalities including disseminated intravascular coagulation and neurological disorders. Leukemia cutis and gingival infiltration can also be seen. A characteristic translocation observed is t(9;11).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute monoblastic/monocytic leukemia | AML M5 · Acute monoblastic or monocytic leukemia |
| fr | Leucémie aiguë monoblastique/monocytaire | AML M5 · LAM M5 · Leucémie aiguë myéloblastique type 5 |
| de | Leukämie, akute monoblastische | AML-M5 · Leukämie, akute monozytäre · Leukämie, akute myeloische, Typ 5 · Monoblastenleukämie |
| es | Leucemia monoblástica aguda | Leucemia mieloblástica aguda tipo 5 · Leucemia monocítica aguda · MLA-M5 |
| it | Leucemia monoblastica acuta | AML-M5 · Leucemia acuta mieloblastica, tipo 5 · Leucemia mieloblastica acuta, tipo 5 · Leucemia monocitica acuta |
| nl | Acute monoblastische leukemie | AML M5 · Acute monocytaire leukemie |
| pt | Leucemia monoblástica/monocítica aguda | Leucemia mieloblástica aguda tipo 5 |
| pl | Ostra białaczka monoblastyczna | AML-M5 · Ostra białaczka mieloblastyczna typu 5 · Ostra białaczka monocytowa |
| cs | Akutní monoblastická leukémie | AML M5 · Akutní monocytární leukémie |
| uk | Гостра монобластна/моноцитарна лейкемія | Гострий монобластний або моноцитарний лейкоз · AML M5 |
| zh | 急性单核细胞白血病 | 急性单核细胞白血病 |
| tr | Akut monoblastik / monositik lösemi | AML M5 · Akut monoblastik veya monositik lösemi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C93.0 | E | specific | validated |
| ICD-11 | 2A60.34 | E | specific | validated |
| MONDO | 0007896 | E | validated | |
| MONDO | 7896 | E | validated | |
| MeSH | D007948 | E | validated | |
| UMLS | C3831784 | E | validated | |
| GARD | 525 | 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 |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.13 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | 1-9 / 1 000 000 | — | Europe | Class only | Not yet validated | ORPHANET |
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
20 annotations · validated 2019-05-22| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0004845 | Acute monocytic leukemia | |
| Frequent (79-30%) | ||
| HP:0001903 | Anemia | |
| HP:0031020 | Bone marrow hypercellularity | |
| HP:0002875 | Exertional dyspnea | |
| HP:0012378 | Fatigue | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0100827 | Lymphocytosis | |
| Occasional (29-5%) | ||
| HP:0012145 | Abnormality of multiple cell lineages in the bone marrow | |
| HP:0001785 | Ankle swelling | |
| HP:0002039 | Anorexia | |
| HP:0011787 | Central hypothyroidism | |
| HP:0025289 | Cervical lymphadenopathy | |
| HP:0001945 | Fever | |
| HP:0001931 | Hypochromic anemia | |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0100520 | Oliguria | |
| HP:0100539 | Periorbital edema | |
| HP:0001730 | Progressive hearing impairment | |
| HP:0001482 | Subcutaneous nodule | |
| HP:0001824 | Weight loss | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 29417354[PMID]_30524762[PMID]_30631409[PMID]_28658145[PMID]_27884836[PMID]_28642361[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/514
- This record as JSON: /data/d/514.json