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

WikiDoc Resources for Pure red cell aplasia
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Historical Perspective

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

The association between [important risk factor/cause] and [disease name] was made in/during [year/event].

In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].

In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

There have been several outbreaks of [disease name], including -----.

In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Classification

cquired pure red cell aplasia (PRCA) is a rare, generally chronic condition of profound anemia characterized by a severe reduction in the number of reticulocytes in the peripheral blood and the virtual absence of erythroid precursors in the bone marrow. All other cell lineages are present and appear morphologically normal.

●A similar but usually self-limited condition seen during the first years of life, transient erythroblastopenia of childhood, is discussed separately. (See "Anemia in children due to decreased red blood cell production", section on 'Transient erythroblastopenia of childhood (TEC)'.)

●A congenital form of red cell aplasia is known as Diamond-Blackfan anemia (DBA). DBA is associated with a number of congenital abnormalities, risk of malignancy, and marked unresponsiveness to prednisone. (See "Anemia in children due to decreased red blood cell production", section on 'Diamond-Blackfan anemia'.)

Acquired PRCA will be discussed here. Acquired PRCA due to the presence of anti-erythropoietin antibodies secondary to treatment with recombinant human erythropoietin is discussed separately

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.

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[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

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[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].

OR

Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

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 ((Page name)) 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/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.

OR

In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.

OR

In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.

Patients of all age groups may develop [disease name].

OR

The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.

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[Disease name] commonly affects individuals younger than/older than [number of years] years of age.

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[Chronic disease name] is usually first diagnosed among [age group].

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[Acute disease name] commonly affects [age group].

There is no racial predilection to [disease name].

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[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].

[Disease name] affects men and women equally.

OR

[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.

The majority of [disease name] cases are reported in [geographical region].

OR

[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].

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.

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

The majority of patients with [disease name] are asymptomatic.

OR

The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

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

RCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as ciclosporin in many patients, though this approach is not without risk.

It has also been shown to respond to treatments with rituximab and tacrolimus.^{[citation needed]}

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

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Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

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Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

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Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

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The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

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WikiDoc Resources for Pure red cell aplasia
Articles
Most recent articles on Pure red cell aplasia Most cited articles on Pure red cell aplasia Review articles on Pure red cell aplasia Articles on Pure red cell aplasia in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Pure red cell aplasia Images of Pure red cell aplasia Photos of Pure red cell aplasia Podcasts & MP3s on Pure red cell aplasia Videos on Pure red cell aplasia
Evidence Based Medicine
Cochrane Collaboration on Pure red cell aplasia Bandolier on Pure red cell aplasia TRIP on Pure red cell aplasia
Clinical Trials
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
Guidelines / Policies / Govt
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
Books
Books on Pure red cell aplasia
News
Pure red cell aplasia in the news Be alerted to news on Pure red cell aplasia News trends on Pure red cell aplasia
Commentary
Blogs on Pure red cell aplasia
Definitions
Definitions of Pure red cell aplasia
Patient Resources / Community
Patient resources on Pure red cell aplasia Discussion groups on Pure red cell aplasia Patient Handouts on Pure red cell aplasia Directions to Hospitals Treating Pure red cell aplasia Risk calculators and risk factors for Pure red cell aplasia
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)
CME Programs on Pure red cell aplasia
International
Pure red cell aplasia en Espanol Pure red cell aplasia en Francais
Business
Pure red cell aplasia in the Marketplace Patents on Pure red cell aplasia
Experimental / Informatics
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]

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