KEGG   DISEASE: Acquired pure red cell aplasia
Entry
H01586                      Disease                                
Name
Acquired pure red cell aplasia
Description
Pure red cell aplasia (PRCA) is a rare condition characterised by selective inhibition and the absence of red cell precursors in the bone marrow with consequent anaemia and reticulocytopenia in the presence of normal granulocytic and megakaryocytic lineages. RPCA can be classified into congenital and acquired. Congenital PRCA is also known as Diamond-Blackfan anemia [DS:H00237] and is a disease of infancy and early childhood. The aetiology of acquired PRCA can be subclassified into primary and secondary. Primary PRCA is defined when no aetiology can be identified using available investigations. The causes of secondary PRCA are again diverse and can be due to infections (viral, bacterial, etc.), drugs (erythropoietin, carbamazepine, etc.), collagen vascular disorders (rheumatoid arthritis, systemic lupus erythematosus, etc.), malignancies (e.g. thymoma), ABO-incompatible haematopoietic stem cell transplantation, and pregnancy. Depending on the cause, the course can be acute and self-limiting or chronic with rare spontaneous remissions. PRCA can be easily diagnosed when isolated anaemia, in the presence of normal white cell and platelet counts, is associated with a marrow of normal cellularity in which there is an almost complete absence of erythroblasts but normal myeloid cells and megakaryocytes. Primary, or secondary PRCA not responding to treatment of the underlying diseases, is treated as an immunologically-mediated disease. The therapeutic plan usually focuses on the sequential use of various immunosuppressive therapies until a remission is obtained.
Category
Hematologic disease; Immune system disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Hematologic diseases
   H01586  Acquired pure red cell aplasia
Human diseases in ICD-11 classification [BR:br08403]
 03 Diseases of the blood or blood-forming organs
  Anaemias or other erythrocyte disorders
   Pure red cell aplasia
    3A61  Acquired pure red cell aplasia
     H01586  Acquired pure red cell aplasia
Drug
Triamcinolone acetonide [DR:D00983]
Dexamethasone [DR:D00292]
Hydrocortisone sodium succinate [DR:D00978]
Prednisolone sodium phosphate [DR:D00981]
Prednisone [DR:D00473]
Methylprednisolone sodium succinate [DR:D00751]
Comment
See also H00237.
Other DBs
ICD-11: 3A61
ICD-10: D60
MeSH: D012010
Reference
  Authors
Malhotra P, Muralikrishna GK, Varma N, Kumari S, Das R, Ahluwalia J, Jain S, Varma S
  Title
Spectrum of pure red cell aplasia in adult population of north-west India.
  Journal
Hematology 13:88-91 (2008)
DOI:10.1179/102453308X315979
Reference
  Authors
Sawada K, Fujishima N, Hirokawa M
  Title
Acquired pure red cell aplasia: updated review of treatment.
  Journal
Br J Haematol 142:505-14 (2008)
DOI:10.1111/j.1365-2141.2008.07216.x
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