aplastic anemia
Let an agent explain aplastic anaemia and its causes in neutral clinical terms, relay diagnosis, severity grading and treatment in general from haematology sources, distinguish it from other marrow failure and anaemia types listed as aliases, and route people with symptoms or diagnoses to haematologists.
Research draft, second pass
A second pass drafted this model: the structure a model of this thing needs, and what is known about it in the world. The line under this one says how the second half was obtained - researched against sources, or recalled without web access, in which case nothing here was read anywhere and every claim is a lead to verify. Unreviewed either way.
written by Claude from model knowledge without web access - no source was read, every claim is a lead to verify
Researched by: Claude
Purpose and description
Let an agent explain aplastic anaemia and its causes in neutral clinical terms, relay diagnosis, severity grading and treatment in general from haematology sources, distinguish it from other marrow failure and anaemia types listed as aliases, and route people with symptoms or diagnoses to haematologists.
A rare, serious condition in which the bone marrow fails to produce enough red cells, white cells and platelets, causing anaemia, infections and bleeding, most often from autoimmune destruction of stem cells, sometimes drug-induced or caused by toxins, viruses or inherited syndromes such as Fanconi anaemia and the telomere disorder Revesz syndrome, and graded as non-severe, severe and very severe; the registry aliases also list distinct marrow disorders such as sideroblastic anaemia, refractory anaemia with ring sideroblasts and myelophthisic anaemia, which have different causes; treatment includes immunosuppression and stem cell transplantation.
What it is for: Not applicable; a disease.
It can be explain causes and grading; relay diagnosis and treatment; distinguish related disorders; route to specialists.
Distinguishing features
Empty marrow
Pancytopenia
Mostly immune-mediated
Curable by transplant in many
What it looks like
Not a visible object; pallor, bruising and infections.
Physical character
incidence: about 2 per million per year - Western countries, higher in East Asia
main treatments: immunosuppression, stem cell transplant list
severity criteria: Camitta criteria note
How it is recognised
Bone marrow failure with pancytopenia
Acquired, drug-induced and severe aplastic anaemia; inherited forms such as Revesz syndrome
Sideroblastic anaemia and refractory anaemia with ring sideroblasts are different marrow disorders; myelophthisic anaemia is marrow replacement
Related models
is a kind of - in registry terms
is a kind of - in registry terms, though most cases are acquired
is treated by - in eligible patients
is contrasted with - a clonal marrow disorder
In practice
Families and kinds
acquired immune-mediated aplastic anaemia
drug-induced aplastic anaemia
aplastic anaemia after hepatitis or toxins
inherited bone marrow failure such as Fanconi anaemia, dyskeratosis congenita and Revesz syndrome
severe and very severe aplastic anaemia
distinct disorders listed as aliases such as sideroblastic anaemia, refractory anaemia with ring sideroblasts and myelophthisic anaemia
Identifiers
ICD-11 3A70 aplastic anaemia
MeSH D000741 anemia, aplastic
Orphanet ORPHA:88 acquired aplastic anemia
Standards and regulation
Haematology guidelines such as those of the British Society for Haematology
Transplant and blood transfusion standards
Drug safety monitoring for marrow toxicity
Failure modes and hazards
Agents giving personal medical advice
Confusing aplastic anaemia with other anaemias listed as aliases
Delayed diagnosis of infections and bleeding
Also called
Where this came from
wikidata · CC0 1.0
Drafted structure
Bundle to layer to finding to question, as the second pass will find it: 4 bundles · 8 layers · 8 findings · 16 questions.
Help Care first.
Safety.
Urgent
Urgent signs.
Urgent
Urgent.
- Why do fever, unusual bleeding, severe fatigue or unexplained bruising need urgent medical assessment? action
- Is the user describing their own symptoms or results, which needs a clinician now? boundary
Support
Support.
Support
Support.
- What patient organisations and support exist for people with aplastic anaemia? provenance
- Which entry fits the specific organisation? action
Understand The disease.
Clinical.
Definition
Definition and causes.
Definition
Definition.
- What is aplastic anaemia, and what immune, drug, toxin, viral and inherited causes exist? definition
- Is the question about aplastic anaemia or a different marrow or anaemia disorder listed as an alias? boundary
Related
Related disorders.
Related
Related.
- How do sideroblastic anaemia, refractory anaemia with ring sideroblasts, myelophthisic anaemia and Revesz syndrome differ from aplastic anaemia? definition
- Which entry fits the specific disorder? action
Care Diagnosis and treatment.
Clinical.
Diagnosis
Diagnosis and grading.
Diagnosis
Diagnosis.
- How is aplastic anaemia diagnosed by blood counts and marrow biopsy, and how is severity graded? provenance
- Which references are standard? provenance
Treatment
Treatment.
Treatment
Treatment.
- How is aplastic anaemia treated with immunosuppression, transplantation and supportive care, in general terms? provenance
- Which sources are cited? provenance
Context Research and history.
Context.
Research
Research.
Research
Research.
- What research addresses eltrombopag, transplant outcomes and telomere biology, with findings attributed? provenance
- Which entry fits bone marrow failure research? action
History
History.
History
History.
- How was aplastic anaemia described by Ehrlich and how did treatment develop? provenance
- Which entry fits the history of haematology? action
What the second pass must settle
- Should inherited marrow failure and sideroblastic anaemia be separate primary entries?
- How should haematology sources be linked?
- The registry entry has merged aliases naming distinct disorders; should they be moved?