In aplastic anemia (synonyms: Aplastic anemia; Aplastic syndrome; Argeneric anemia; Panmyelopathy, Panmyelophtisis; ICD-10-GM D61.9: Aplastic anemia, unspecified) is a form of anemia (anemia) characterized by pancytopenia (synonym: tricytopenia; reduction of all three rows of cells in the blood; stem cell disease) and concomitant hypoplasia (functional impairment) of the bone marrow.
Aplastic anemia belongs to the group of hyporegenerative anemias, i.e. there is a disorder of erythropoiesis (formation of mature erythrocytes from hematopoietic stem cells of the hematopoietic bone marrow).
Typical of aplastic anemia is a normal mean hemoglobin content per erythrocyte (MCH) and normal mean single erythrocyte volume (MCV). This is referred to as normochromasia and classifies the anemia as a normocytic normochromic anemia. Furthermore, reticulocytes are normal to decreased in this clinical picture.
Classification of aplastic anemia according to severity (2 of 3 criteria must be met):
Severity | Granulocytes | Platelets | Reticulocytes |
Non-severe aplastic anemia (nSAA). | <1,500/µl | < 50,000/µl | < 60,000/µl |
Severe AA (SAA) | <500/µl | < 20,000/µl | < 20,000/µl |
Very (very) severe AA (vSAA) | <200/µl | < 20,000/µl | < 20,000/µl |
Furthermore, a distinction is made between congenital (congenital) and acquired forms of aplastic anemia. The most important representatives of the two forms include:
Congenital aplastic anemia (occurs rarely overall).
- Dyskeratosis congenita
- Fanconi anemia
Acquired aplastic anemia
- Aplastic anemia due to radiatio (radiotherapy) or cytostatic drugs.
- Aplastic anemia caused by immune diseases.
- Viral-induced aplastic anemia
In more than half of all cases of disease, the cause remains unclear.
Sex ratio: males and females are equally affected.
Frequency peaks: there are two age peaks. One is in teenagers and young adults and the other is in older individuals. A correlation to hormonal changes is suspected.
The incidence (frequency of new cases) is approximately 2 cases per 1,000,000 population per year (in Europe). In China, the incidence is 2 diseases per 100,000 inhabitants per year.
Course and prognosis: The prognosis is very poor if untreated. The lethality (mortality related to the total number of people suffering from the disease) for untreated patients is up to 70% (they die within a short time). With supportive (assisting) therapy, the lethality can be reduced to about 50%, and with immunosuppressive therapy or allogeneic bone marrow transplantation, the lethality is less than 20%. The most important prognostic parameters are granulocyte count and age at diagnosis.