Acute panmyelosis with myelofibrosis

Summary

Acute panmyelosis with myelofibrosis (MONDO:0019455) is a disease. A subtype of acute myeloid leukemia by FAB classification — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 15

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.06	Europe	Validated

Signs & symptoms

Clinical features (HPO)

15 HPO clinical features (Orphanet curated; top 15 by frequency):

HPO ID	Term	Frequency
HP:0001876	Pancytopenia	Very frequent (80-99%)
HP:0011974	Myelofibrosis	Very frequent (80-99%)
HP:0001324	Muscle weakness	Frequent (30-79%)
HP:0012129	Abnormality of bone marrow stromal cells	Frequent (30-79%)
HP:0012143	Abnormal megakaryocyte morphology	Frequent (30-79%)
HP:0012378	Fatigue	Frequent (30-79%)
HP:0031020	Bone marrow hypercellularity	Frequent (30-79%)
HP:0003419	Low back pain	Occasional (5-29%)
HP:0004808	Acute myeloid leukemia	Occasional (5-29%)
HP:0004820	Acute myelomonocytic leukemia	Occasional (5-29%)
HP:0005528	Bone marrow hypocellularity	Occasional (5-29%)
HP:0031385	Megakaryocyte nucleus hypolobulation	Occasional (5-29%)
HP:0031386	Increased micromegakaryocyte count	Occasional (5-29%)
HP:0100827	Lymphocytosis	Occasional (5-29%)
HP:0001744	Splenomegaly	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	acute panmyelosis with myelofibrosis
Mondo ID	MONDO:0019455
Orphanet	86843
ICD-10-CM	C94.4
ICD-11	1831346835, 585339631
NCIT	C4344
SNOMED CT	109991003
UMLS	C0334674
MedGen	87279
GARD	0011907
MedDRA	10000879
Is cancer (heuristic)	no

Also known as: acute (malignant) myelofibrosis · acute (malignant) myelosclerosis · acute myelodysplasia with myelofibrosis · acute myelofibrosis · acute myelosclerosis · acute panmyelosis · APMF

Disease family

This is a subtype of acute myeloid leukemia by FAB classification. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › hematopoietic and lymphoid system neoplasm › hematopoietic and lymphoid cell neoplasm › leukemia › myeloid leukemia › acute myeloid leukemia › acute myeloid leukemia by FAB classification › acute panmyelosis with myelofibrosis

Related subtypes (8): acute myeloid leukemia with minimal differentiation, acute myeloblastic leukemia without maturation, myeloid sarcoma, acute erythroid leukemia, acute myelomonocytic leukemia M4, acute megakaryoblastic leukemia, acute basophilic leukemia, acute myeloblastic leukemia with maturation

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.