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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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.

OR

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

OR

[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.

OR

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

OR

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

OR

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

There is no racial predilection to [disease name].

OR

[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

There are no established risk factors for [disease name].

OR

The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.

Screening

There is insufficient evidence to recommend routine screening for [disease/malignancy].

OR

According to the [guideline name], screening for [disease name] is not recommended.

OR

According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

In one of the largest available series, 5 of 37 patients (14 percent) with PRCA had spontaneously-remitting disease (eg, pregnancy, some cases of parvovirus infection, drugs), requiring no specific therapy other than transfusion for symptomatic anemia and cessation of possible offending drugs [21-23].

For the remaining patients, acquired pure red cell aplasia is most often a chronic condition. Although remissions can be attained with the use of immunosuppressive or cytotoxic agents, there is a high propensity for relapse following discontinuation of treatment. Relapse-free survivals (ie, survival without the need for red cell transfusion) can be as short as three months or longer than eight years, depending upon the treatment program employed and whether or not maintenance treatment is given [24].

●In one study reported in 1984, overall survival in patients with idiopathic PRCA was >10 years, whereas it was four years in those with PRCA secondary to another condition (eg, leukemia, lymphoma) [21]. Infection was the most common cause of morbidity and mortality, likely due to the side effects of treatment with agents such as glucocorticoids and cyclophosphamide.

●A 2007 report in 62 patients with primary (idiopathic) PRCA indicated a 10-year overall survival of 95 percent, with no significant difference between those treated with cyclosporine or those treated with glucocorticoids [25]. In a separate 2008 report on 41 patients with thymoma and PRCA, median overall survival was 12 years [2

Diagnosis

Diagnostic Study of Choice

• Complete blood count, peripheral smear, reticulocyte count
• Hepatic function test
• Renal function test

●CT or MRI of the chest to rule out thymoma and other neoplasms

●Viral studies as appropriate, including parvovirus

●Bone marrow aspiration and biopsy

●Autoimmune/antinuclear antibody studies as appropriate

●Specialized tests (when an underlying hematologic condition is suspected) may include karyotype, T cell receptor clonality studies, peripheral blood immunophenotyping

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

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.

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

The diagnosis of PRCA is established when all of the following are present:

●Normocytic, normochromic anemia; rarely, macrocytic

●A very low (or zero) reticulocyte percentage, and an absolute reticulocyte count <10,000/microL

●Normal white blood cell and platelet counts

●A normocellular bone marrow that shows normal myelopoiesis, lymphopoiesis, and megakaryocytopoiesis, but few if any erythroid precursors.

Anemia is often severe and is normocytic and normochromic. Spherocytes, red cell fragments, macrocytes, and polychromatophilic red cells are not seen on routine peripheral blood smears. An exception is parvovirus-associated transient suppression of erythropoiesis seen in chronic hemolytic anemia, in which cells typical of the underlying anemia (eg, spherocytes in the case of hereditary spherocytosis) are evident on the smear. Reticulocytes are markedly decreased and are often absent.

●There are no changes in white blood cell or platelet counts; abnormal circulating white blood cells are not present in idiopathic cases, but may be seen in patients with an underlying hematologic disorder (eg, chronic lymphocytic leukemia, large granular lymphocyte leukemia).

●As ineffective erythropoiesis and hemolytic anemia are not seen in PRCA, levels of indirect bilirubin, haptoglobin, and lactate dehydrogenase are normal, and the direct antiglobulin (Coombs) test is negative.

●Once the bone marrow stops making red cells, the transfer of iron from plasma to the bone marrow also ceases, resulting in an increase in serum iron concentration, often to the level of the total iron binding capacity, such that transferrin saturation approaches 100 percent and the unsaturated iron binding capacity approaches zero.

●Bone marrow examination shows normal overall cellularity, with complete or virtually complete absence of red cell precursors. In patients with PRCA due to parvovirus infection, characteristic giant proerythroblasts can be seen (picture 1

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

An x-ray may be helpful in the diagnosis of thymoma and other neoplasms. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

Chest CT scan may be helpful in the diagnosis of thymoma and other neoplasms. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

MRI

Chest MRI may be helpful in the diagnosis of thymoma and other neoplasms. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

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].

OR

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

OR

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].

OR

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].

OR

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]

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