Acquired isolated aplastic anemia

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Pure red cell aplasia ( Engl. : Aplasia of red blood cells, PRCA ) or isolated aplastic anemia or pure red cell aplasia is an anemia caused by destruction or missing formation of erythroblasts in the bone marrow results. It can be congenital or acquired. Today the congenital PRCA is mostly called Diamond-Blackfan-Syndrome (see there) while the abbreviation PRCA is mostly used for the acquired form.

causes

The acquired PRCA is viewed by many clinicians as an autoimmune disease . It often occurs at the same time as other autoimmune diseases, such as lupus erythematosus or thymomas . In addition, PRCA can occur as a result of a viral infection, for example with parvovirus B19 (especially in children), HIV , herpes simplex viruses and virus hepatitis . PRCA also occurs frequently associated with immune-mediated bone marrow diseases on, such as aplastic anemia and T-LGL cell - leukemia . Autoantibodies , autoimmune T cells or drugs (e.g. erythropoietin or mycophenolate mofetil ), which in turn often lead to the development of autoantibodies, are assumed to be the causes .

PRCA can also occur as an immunohematological complication of a graft-versus-host reaction after a stem cell transplant. However, as with many blood disorders, in a number of cases the cause is unknown ( idiopathic ).

treatment

PRCA patients respond well to treatment with immunosuppressants ( e.g. ciclosporin or rituximab ), which supports the hypothesis that this is an autoimmune disease.

Complications

The complications that arise in the course of the disease are bleeding and often the transition to aplastic anemia . It is believed that PRCA occurs as a symptom of (possibly not yet recognized) aplastic anemia.

History

The first description of the acquired PRCA was made by Paul Kaznelson in 1922. The first description of the innate PRCA goes back to Louis Diamond and Kenneth Blackfan .

Web links

  • Website of the Aplastic Anemia & MDS International Foundation Inc.

Individual evidence

  1. ↑ H. Frost, B. von Graffenried, R. Stoller: Erythroblastopenia (Pure Red Cell Aplasia, PRCA) by induced antibodies: New rare severe undesirable effects of recombinant erythropoietin (epoetin, also abbreviated to EPO). ( Memento of the original from December 21, 2015 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. In: Swissmedic Journal. 2002; 1, pp. 445-446, ISSN 0026-9212 . @1@ 2Template: Webachiv / IABot / www.swissmedic.ch 
  2. ↑ R. Handgretinger, A. Geiselhart, A. Moris et al .: Pure red-cell aplasia associated with clonal expansion of granular lymphocytes expressing killer-cell inhibitory receptors. In: N Engl J Med . 1999; 340, pp. 278-284. PMID 9920952
  3. ↑ K. Sawada, N. Fujishima, M. Hirokawa: Acquired pure red cell aplasia: updated review of treatment. In: Br J Haematol . 2008; 142, pp. 505-514. doi: 10.1111 / j.1365-2141.2008.07216.x . PMID 18510682 .
  4. ^ P. Kaznelson. About the development of platelets. In: Verh Dtsch Ges Inn Med. 1922; 34, pp. 557-558.
  5. ^ LK Diamond, KD Blackfan: Hypoplastic anemia. In: Am J Dis Child. 1938; 56, pp. 464-467.