Pure red cell aplasia

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

Synonyms and keywords: Acquired pure megakaryocytic aplasia, pure red cell aplasia, erythroblastopenia

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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

Historical Perspective

Pure red cell aplasia was first discovered by Paul Kaznelson in 1922.

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

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.The numbers of white blood cells and platelets are normal..In autoimmune disorders, IgG fraction in serum inhibit the growth of normal erythroid progenitors. In some cases of autoimmune PRCA, T lymphocytes suppress erythropoiesis.

Causes

• Autoimmune disease
  • Autoimmune hemolytic anemia
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
• Thymoma
• Viral infections
  • HIV
  • Herpes
  • Parvovirus B19 (Fifth disease)
  • Hepatitis such as hepatitis C
• Lymphoproliferative disorders
  • T-cell large granular lymphocyte leukemia, especially in china
  • Chronic lymphocytic leukemia
  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma

• Myeloid malignancies such as Chronic myeloid leukemia
• Myelodysplastic syndrome
• Idiopathic
• Drugs
  • Phenytoin
  • Chloramphenicol
  • Azathioprine
  • Isoniazid
  • Valproic acid
  • Erythropoietin
  • Trimethoprim-sulfamethoxazole
  • Zidovudine
  • Chlorpropamide
• ABO- incompatible hematopoietic cell transplantation
• Anti- erythropoietin antibodies due to treatment with recombinant human erythropoietin
• Plasma cell disorders
• Pregnancy

The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

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.

• Transient erythroblastopenia of childhood: Itr is self-limited condition during first years of life.

• Diamond-Blackfan anemia (DBA): congenital form of red cell aplasia. It is associated with some malignancies and it does not respond to prednisone.
• Aplastic anemia: It affects other bone marrow cells as well.

Epidemiology and Demographics

• The incidence of Diamond-Blackfan anemia (DBA) is approximately 6.6 per 100,000 individuals in Erope. 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.
• The incidence of thymoma in patients with pure red cell aplasia is about 5%.

Risk Factors

Common risk factors 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.

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 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.

Diagnosis

Diagnostic Study of Choice

• Complete blood count, peripheral smear, reticulocyte count
• Hepatic function test
• Renal function test
• Bone marrow aspiration and biopsy

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:

• Normocytic, normochromic anemia; rarely, macrocytic anemia may be seen.
• Very low or zero reticulocyte percentage and an absolute reticulocyte count <10,000/microL
• Normal white blood cell
• Normal platelet counts
• Bone marrow bipsy: normal myelopoiesis, lymphopoiesis, and megakaryocytopoiesis, but few erythroid precursors

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

• Viral studies for hepatitis c and parvovirus B19
• Autoimmune antibody studies
• karyotype
• T cell receptor clonality studies
• Peripheral blood immunophenotyping

Treatment

Medical Therapy

• Symptomatic anemia: Red blood cell transfusions
• Cessation of offending drugs
• Investigation for associated condition
• Pure red cell aplasia due to ABO incompatible hematopoietic cell transplantation is usually self limited.
• Intravenous immune globulin (IVIG): single infusion 400 mg/kg over 2-3 hours if spontaneous resolution does not occur during 2-3 weeks. IVIG, 400 mg/kg daily for five day can be considered in resistant pure red cell aplasia.
• Immunosuppressive therapy in idiopathic pure red cell aplasia such as:
  • Glucocorticoids: Prednisone, oral dose ( 60 mg/day in divided doses ). It is considerd as a initial treatment.
  • Glucocorticoids plus cyclosporine: If no response to glucocorticoids occur after one to two months. Cyclosporine oral dosage can be considered 200 to 600 mg/day.
  • Glucocorticoids plus cyclophosphamide: Cyclophosphamide oral dosage can be considered 2 to 3 mg/kg per day.
• Refractory cases:
  • Azathioprine (2 to 3 mg/kg per day)
  • Antilymphocyte globulin
  • Antithymocyte globulin
  • Rituximab: Anti-CD20 monoclonal antibody
  • Alemtuzumab: Anti-CD52 monoclonal antibody
  • Daclizumab: Anti-interleukin monoclonal receptor antibody

Surgery

Thymectomy: Surgery is usually reserved for patients with thymoma.

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 [disease name].

References

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Ongoing Trials on Pure red cell aplasia at Clinical Trials.gov Trial results on Pure red cell aplasia Clinical Trials on Pure red cell aplasia at Google
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US National Guidelines Clearinghouse on Pure red cell aplasia NICE Guidance on Pure red cell aplasia NHS PRODIGY Guidance FDA on Pure red cell aplasia CDC on Pure red cell aplasia
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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
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International
Pure red cell aplasia en Espanol Pure red cell aplasia en Francais
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Pure red cell aplasia in the Marketplace Patents on Pure red cell aplasia
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List of terms related to Pure red cell aplasia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]Mahda Alihashemi M.D. [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24] [25] [26] [27] [28] [29] [30] [31] [32] [33] [34] [35] [36]

Synonyms and keywords: Acquired pure megakaryocytic aplasia, pure red cell aplasia, erythroblastopenia

Overview

Acquired pure red cell aplasia (or PRCA) refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells.

Causes

Pure red cell aplasia is regarded as an autoimmune disease. It may also be a manifestation of thymoma. It may also be as a result of viral infections such as HIV, herpes, parvovirus B19 (Fifth disease), or hepatitis. Association of pure red cell aplasia with T large granular lymphocyte leukemia is also well recognized, especially in China (http://jcp.bmj.com/cgi/content/abstract/51/9/672). Many cases of PRCA are considered idiopathic in that there is no discernable cause detected.

It can be associated with the administration of erythropoietin.

Treatment

Medical Therapy

Drug Side Effect

• Mycophenolate

PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as ciclosporin. It has also been also been shown to respond to treatments with Rituxan.

Contraindicated medications

Pure red cell aplasia is considered an absolute contraindication to the use of the following medications:

• Darbepoetin Alfa
• Methoxy polyethylene glycol-epoetin beta

See also

• Diamond-Blackfan anemia (genetic red cell aplasia)
• aplastic anemia (aplasia affecting other bone marrow cells as well)

External links

• Aplastic Anemia & MDS International Foundation
• NEJM