Pure red cell aplasia

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Acquired pure red cell aplasia
ICD-10 D60
ICD-9 284.8
DiseasesDB 29063
MeSH D012010

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2] Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [3]

Synonyms and Keywords: Pure red cell aplasia; Erythroblastopenia.


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Evidence Based Medicine

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Clinical Trials

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Guidelines / Policies / Govt

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Healthcare Provider Resources

Symptoms of Pure red cell aplasia

Causes & Risk Factors for Pure red cell aplasia

Diagnostic studies for Pure red cell aplasia

Treatment of Pure red cell aplasia

Continuing Medical Education (CME)

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International

Pure red cell aplasia en Espanol

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Pure red cell aplasia in the Marketplace

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Overview

Pure red cell aplasia was first discovered by Paul Kaznelson in 1922. Pure red cell aplasia may be classified into primary (idiopathic) PRCA and acquired red cell aplasia. It is thought that acquired pure red cell aplasia is the result of profound anemia due to severe reduction in number of RBC in peripheral blood and absence of erythroid precursors, proerythroblast in the bone marrow. Causes include autoimmune disease, thymoma, viral infections, lymphoproliferative disorders, idiopathic, drugs, ABO- incompatible hematopoietic cell transplantation, Anti- erythropoietin antibodies. Pure red cell aplasia must be differentiated from transient erythroblastopenia of childhood, Diamond-Blackfan anemia (DBA) and aplastic anemia. The incidence of Diamond-Blackfan anemia (DBA) is approximately 6.6 per 100,000 individuals in Europe. Pure red cell aplasia due to Diamond-Blackfan anemia (DBA) affects men and women equally. Common risk factor in the development of pure red cell aplasia include strong family history. If left untreated, 14% of patients with pure red cell aplasia may have spontaneously remitting disease. Pure red cell aplasia due to parvovirus infection usually resolve within 2-3 weeks. Common complications of pure red cell aplasia include infection due to side effects of some treatments. Prognosis is generally good. Diagnostic study of choice include complete blood count, peripheral smear, reticulocyte count, hepatic function test, renal function test, bone marrow aspiration and biopsy. Common symptoms of pure red cell aplasia include fatigue and lethargy. Laboratory findings consistent with the diagnosis of pure red cell aplasia include normocytic, normochromic anemia, very low or zero reticulocyte percentage and an absolute reticulocyte count <10,000/microL, normal white blood cell and platelets. An x-ray may be helpful in the diagnosis of thymoma and other neoplasms. Medical therapy include red blood cell transfusion in symptomatic anemia, cessation of offending drugs, Intravenous immune globulin (IVIG) in resistant pure red cell aplasia, Immunosuppressive therapy in idiopathic pure red cell aplasia. Surgery is usually reserved for patients with thymoma.

Historical Perspective

  • Pure red cell aplasia was first discovered by Paul Kaznelson in 1922.[1]
  • A congenital form of PRCA was described by Diamond and Blackfan in 1938.

Classification

There is no established system for the classification of pure red cell aplasia (PRCA). However it may be classified into primary (idiopathic) PRCA and acquired red cell aplasia. Diamond-Blackfan anemia (DBA) is a congenital form of red cell aplasia. Based on the duration of symptoms, pure red cell aplasia may be classified as either acute or chronic.

Pathophysiology

Causes

Differentiating Pure Red Cell Aplasia from Other Diseases

Pure red cell aplasia must be differentiated from transient erythroblastopenia of childhood, Diamond-Blackfan anemia (DBA) and Aplastic anemia:

Epidemiology and Demographics

  • The incidence of Diamond-Blackfan anemia (DBA) is approximately 6.6 per 100,000 individuals in Europe. Pure red cell aplasia due to Diamond-Blackfan anemia (DBA) affects men and women equally and there is no racial predilection to this disease.
  • The acquired form of pure red cell aplasia can presents as an acute self-limited disease predominantly in children or chronic illness that is more seen in adults.[16]
  • The incidence of thymoma in patients with pure red cell aplasia is about 5%.[17]

Risk Factors

Common risk factor in the development of pure red cell aplasia include strong family history.

Screening

There is insufficient evidence to recommend routine screening for pure red cell aplasia.

Natural History, Complications, and Prognosis

  • If left untreated, 14% of patients with pure red cell aplasia may have spontaneously remitting disease.[18]
  • Pure red cell aplasia due to parvovirus infection usually resolve within 2-3 weeks. [19]
  • Common complications of pure red cell aplasia include infection due to side effects of some treatments such as glucocorticoids and cyclophosphamide.
  • Prognosis is generally good. In one study in 1984, survival in idiopathic pure red cell aplasia was more than 10 years, but only four years in pure red cell aplasia secondary to leukemia and lymphoma.[20]

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Common symptoms of pure red cell aplasia include fatigue and lethargy.

Physical Examination

Common physical examination findings of pure red cell aplasia include fast heart beat and pale apperance.

Laboratory Findings

Laboratory findings consistent with the diagnosis of pure red cell aplasia include:[22]

Electrocardiogram

There are no ECG findings associated with pure red cell aplasia.

X-ray

An x-ray may be helpful in the diagnosis of thymoma and other neoplasms.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with pure red cell aplasia.

CT scan

Chest CT scan may be helpful in the diagnosis of thymoma and other neoplasms.

MRI

Chest MRI may be helpful in the diagnosis of thymoma and other neoplasms.

Imaging Findings

There are no other imaging findings associated with pure red cell aplasia.

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Thymectomy: Surgery is usually reserved for patients with thymoma. [33]

Primary Prevention

There are no established measures for the primary prevention of pure red cell aplasia.

Secondary Prevention

There are no established measures for the secondary prevention of pure red cell aplasia.

References

  1. Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC 2592349. PMID 18510682.
  2. Dessypris EN (October 1991). "The biology of pure red cell aplasia". Semin. Hematol. 28 (4): 275–84. PMID 1759168.
  3. Dessypris EN (October 1991). "The biology of pure red cell aplasia". Semin. Hematol. 28 (4): 275–84. PMID 1759168.
  4. Lacy MQ, Kurtin PJ, Tefferi A (April 1996). "Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities". Blood. 87 (7): 3000–6. PMID 8639922.
  5. Hirokawa M, Sawada K, Fujishima N, Nakao S, Urabe A, Dan K, Fujisawa S, Yonemura Y, Kawano F, Omine M, Ozawa K (January 2008). "Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group". Haematologica. 93 (1): 27–33. doi:10.3324/haematol.11655. PMID 18166782.
  6. Geetha D, Zachary JB, Baldado HM, Kronz JD, Kraus ES (December 2000). "Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature". Clin Transplant. 14 (6): 586–91. PMID 11127313.
  7. al-Awami Y, Sears DA, Carrum G, Udden MM, Alter BP, Conlon CL (August 1997). "Pure red cell aplasia associated with hepatitis C infection". Am. J. Med. Sci. 314 (2): 113–7. PMID 9258213.
  8. Kwong YL, Wong KF (September 1998). "Association of pure red cell aplasia with T large granular lymphocyte leukaemia". J. Clin. Pathol. 51 (9): 672–5. PMC 500904. PMID 9930071.
  9. Dessypris EN (October 1991). "The biology of pure red cell aplasia". Semin. Hematol. 28 (4): 275–84. PMID 1759168.
  10. Miller AC, Rashid RM (2008). "Three episodes of acquired pure red cell aplasia restricted to pregnancy". J Perinat Med. 36 (3): 270–1. doi:10.1515/JPM.2008.041. PMID 18576941.
  11. Macdougall IC (November 2007). "Epoetin-induced pure red cell aplasia: diagnosis and treatment". Curr. Opin. Nephrol. Hypertens. 16 (6): 585–8. doi:10.1097/MNH.0b013e3282f0c4bf. PMID 18089975.
  12. Bartakke S, Abdelhaleem M, Carcao M (April 2008). "Valproate-induced pure red cell aplasia and megakaryocyte dysplasia". Br. J. Haematol. 141 (2): 133. doi:10.1111/j.1365-2141.2008.06979.x. PMID 18353161.
  13. Thompson DF, Gales MA (1996). "Drug-induced pure red cell aplasia". Pharmacotherapy. 16 (6): 1002–8. PMID 8947971.
  14. Rossert J, Yue S, Smirnakis K, Mytych DT, Johnson L, Kouchakji E, Casadevall N (February 2014). "Risk of pure red cell aplasia in patients with hepatitis C receiving antiviral therapy and an erythropoiesis-stimulating agent". Clin. Gastroenterol. Hepatol. 12 (2): 341–5. doi:10.1016/j.cgh.2013.09.065. PMID 24120841.
  15. Korde N, Zhang Y, Loeliger K, Poon A, Simakova O, Zingone A, Costello R, Childs R, Noel P, Silver S, Kwok M, Mo C, Young N, Landgren O, Sloand E, Maric I (June 2016). "Monoclonal gammopathy-associated pure red cell aplasia". Br. J. Haematol. 173 (6): 876–83. doi:10.1111/bjh.14012. PMC 5549779. PMID 26999424.
  16. Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC 2592349. PMID 18510682.
  17. Clark DA, Dessypris EN, Krantz SB (February 1984). "Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients". Blood. 63 (2): 277–86. PMID 6581839.
  18. Clark DA, Dessypris EN, Krantz SB (February 1984). "Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients". Blood. 63 (2): 277–86. PMID 6581839.
  19. Kurtzman G, Frickhofen N, Kimball J, Jenkins DW, Nienhuis AW, Young NS (August 1989). "Pure red-cell aplasia of 10 years' duration due to persistent parvovirus B19 infection and its cure with immunoglobulin therapy". N. Engl. J. Med. 321 (8): 519–23. doi:10.1056/NEJM198908243210807. PMID 2548098.
  20. Clark DA, Dessypris EN, Krantz SB (February 1984). "Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients". Blood. 63 (2): 277–86. PMID 6581839.
  21. Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC 2592349. PMID 18510682.
  22. Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC 2592349. PMID 18510682.
  23. Crabol Y, Terrier B, Rozenberg F, Pestre V, Legendre C, Hermine O, Montagnier-Petrissans C, Guillevin L, Mouthon L (April 2013). "Intravenous immunoglobulin therapy for pure red cell aplasia related to human parvovirus b19 infection: a retrospective study of 10 patients and review of the literature". Clin. Infect. Dis. 56 (7): 968–77. doi:10.1093/cid/cis1046. PMID 23243178.
  24. Ballester OF, Saba HI, Moscinski LC, Nelson R, Foulis P (July 1992). "Pure red cell aplasia: treatment with intravenous immunoglobulin concentrate". Semin. Hematol. 29 (3 Suppl 2): 106–8. PMID 1509289.
  25. Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC 2592349. PMID 18510682.
  26. Lacy MQ, Kurtin PJ, Tefferi A (April 1996). "Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities". Blood. 87 (7): 3000–6. PMID 8639922.
  27. Raghavachar A (1990). "Pure red cell aplasia: review of treatment and proposal for a treatment strategy". Blut. 61 (2–3): 47–51. PMID 1698487.
  28. Lacy MQ, Kurtin PJ, Tefferi A (April 1996). "Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities". Blood. 87 (7): 3000–6. PMID 8639922.
  29. Abkowitz JL, Powell JS, Nakamura JM, Kadin ME, Adamson JW (December 1986). "Pure red cell aplasia: response to therapy with anti-thymocyte globulin". Am. J. Hematol. 23 (4): 363–71. PMID 3098093.
  30. Ghazal H (February 2002). "Successful treatment of pure red cell aplasia with rituximab in patients with chronic lymphocytic leukemia". Blood. 99 (3): 1092–4. PMID 11807020.
  31. Ru X, Liebman HA (October 2003). "Successful treatment of refractory pure red cell aplasia associated with lymphoproliferative disorders with the anti-CD52 monoclonal antibody alemtuzumab (Campath-1H)". Br. J. Haematol. 123 (2): 278–81. PMID 14531909.
  32. Sloand EM, Scheinberg P, Maciejewski J, Young NS (February 2006). "Brief communication: Successful treatment of pure red-cell aplasia with an anti-interleukin-2 receptor antibody (daclizumab)". Ann. Intern. Med. 144 (3): 181–5. PMID 16461962.
  33. Clark DA, Dessypris EN, Krantz SB (February 1984). "Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients". Blood. 63 (2): 277–86. PMID 6581839.



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