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Pure red cell aplasia was first discovered by Paul Kaznelson in 1922.<ref name="pmid18510682">{{cite journal |vauthors=Sawada K, Fujishima N, Hirokawa M |title=Acquired pure red cell aplasia: updated review of treatment |journal=Br. J. Haematol. |volume=142 |issue=4 |pages=505–14 |date=August 2008 |pmid=18510682 |pmc=2592349 |doi=10.1111/j.1365-2141.2008.07216.x |url=}}</ref>
Pure red cell aplasia was first discovered by Paul Kaznelson in 1922.<ref name="pmid18510682">{{cite journal |vauthors=Sawada K, Fujishima N, Hirokawa M |title=Acquired pure red cell aplasia: updated review of treatment |journal=Br. J. Haematol. |volume=142 |issue=4 |pages=505–14 |date=August 2008 |pmid=18510682 |pmc=2592349 |doi=10.1111/j.1365-2141.2008.07216.x |url=}}</ref>


The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
A congenital form of PRCA was described by Diamond and Blackfan in 1938.  
 
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==
==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.
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.  
 
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'.)
 
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==
==Pathophysiology==
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==Epidemiology and Demographics==
==Epidemiology and Demographics==
The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
* 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 incidence of thymoma in patients with pure red cell aplasia is about 5%. <ref name="pmid65818393">{{cite journal |vauthors=Clark DA, Dessypris EN, Krantz SB |title=Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients |journal=Blood |volume=63 |issue=2 |pages=277–86 |date=February 1984 |pmid=6581839 |doi= |url=}}</ref>
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==
==Risk Factors==
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If left untreated, 14% of patients with pure red cell aplasia may have spontaneously remitting disease.<ref name="pmid6581839">{{cite journal |vauthors=Clark DA, Dessypris EN, Krantz SB |title=Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients |journal=Blood |volume=63 |issue=2 |pages=277–86 |date=February 1984 |pmid=6581839 |doi= |url=}}</ref>
If left untreated, 14% of patients with pure red cell aplasia may have spontaneously remitting disease.<ref name="pmid6581839">{{cite journal |vauthors=Clark DA, Dessypris EN, Krantz SB |title=Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients |journal=Blood |volume=63 |issue=2 |pages=277–86 |date=February 1984 |pmid=6581839 |doi= |url=}}</ref>


Common complications of pure red cell aplasia include infection due to side effects of some treatments such as glucocorticoids and cyclophosphamide.  
Pure red cell aplasia due to parvovirus infection usually resolve within 2-3 weeks. <ref name="pmid2548098">{{cite journal |vauthors=Kurtzman G, Frickhofen N, Kimball J, Jenkins DW, Nienhuis AW, Young NS |title=Pure red-cell aplasia of 10 years' duration due to persistent parvovirus B19 infection and its cure with immunoglobulin therapy |journal=N. Engl. J. Med. |volume=321 |issue=8 |pages=519–23 |date=August 1989 |pmid=2548098 |doi=10.1056/NEJM198908243210807 |url=}}</ref>
 
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. <ref name="pmid65818392">{{cite journal |vauthors=Clark DA, Dessypris EN, Krantz SB |title=Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients |journal=Blood |volume=63 |issue=2 |pages=277–86 |date=February 1984 |pmid=6581839 |doi= |url=}}</ref>
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. <ref name="pmid65818392">{{cite journal |vauthors=Clark DA, Dessypris EN, Krantz SB |title=Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients |journal=Blood |volume=63 |issue=2 |pages=277–86 |date=February 1984 |pmid=6581839 |doi= |url=}}</ref>
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==Treatment ==
==Treatment ==
===Medical Therapy===
===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.
* Symptomatic anemia: Red blood cell transfusions
 
* Cessation of offending drugs
It has also been shown to respond to treatments with rituximab and tacrolimus.<sup>[''citation needed'']</sup>
* Investigation for associated condition
 
* Pure red cell aplasia due to ABO incompatible hematopoietic cell transplantation is usually self limited.
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
* Intravenous immune globulin (IVIG): single infusion 400 mg/kg  over 2-3 hours if spontaneous resolution does not occur during 2-3 weeks. <ref name="pmid23243178">{{cite journal |vauthors=Crabol Y, Terrier B, Rozenberg F, Pestre V, Legendre C, Hermine O, Montagnier-Petrissans C, Guillevin L, Mouthon L |title=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 |journal=Clin. Infect. Dis. |volume=56 |issue=7 |pages=968–77 |date=April 2013 |pmid=23243178 |doi=10.1093/cid/cis1046 |url=}}</ref> IVIG, 400 mg/kg daily for five day can be considered in resistant pure red cell aplasia. <ref name="pmid1509289">{{cite journal |vauthors=Ballester OF, Saba HI, Moscinski LC, Nelson R, Foulis P |title=Pure red cell aplasia: treatment with intravenous immunoglobulin concentrate |journal=Semin. Hematol. |volume=29 |issue=3 Suppl 2 |pages=106–8 |date=July 1992 |pmid=1509289 |doi= |url=}}</ref>
 
* Immunosuppressive therapy in idiopathic pure red cell aplasia such as: <ref name="pmid185106823">{{cite journal |vauthors=Sawada K, Fujishima N, Hirokawa M |title=Acquired pure red cell aplasia: updated review of treatment |journal=Br. J. Haematol. |volume=142 |issue=4 |pages=505–14 |date=August 2008 |pmid=18510682 |pmc=2592349 |doi=10.1111/j.1365-2141.2008.07216.x |url=}}</ref>
OR
** Glucocorticoids: Prednisone, oral dose ( 60 mg/day in divided doses ). It is considerd as a initial treatment. <ref name="pmid86399222">{{cite journal |vauthors=Lacy MQ, Kurtin PJ, Tefferi A |title=Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities |journal=Blood |volume=87 |issue=7 |pages=3000–6 |date=April 1996 |pmid=8639922 |doi= |url=}}</ref>
 
** 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.<ref name="pmid1698487">{{cite journal |vauthors=Raghavachar A |title=Pure red cell aplasia: review of treatment and proposal for a treatment strategy |journal=Blut |volume=61 |issue=2-3 |pages=47–51 |date=1990 |pmid=1698487 |doi= |url=}}</ref>
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
** Glucocorticoids plus cyclophosphamide: Cyclophosphamide oral dosage can be considered 2 to 3 mg/kg per day. <ref name="pmid86399223">{{cite journal |vauthors=Lacy MQ, Kurtin PJ, Tefferi A |title=Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities |journal=Blood |volume=87 |issue=7 |pages=3000–6 |date=April 1996 |pmid=8639922 |doi= |url=}}</ref>
 
* Refractory cases:
OR
** Azathioprine (2 to 3 mg/kg per day)
 
** Antilymphocyte globulin
The majority of cases of [disease name] are self-limited and require only supportive care.
** Antithymocyte globulin<ref name="pmid3098093">{{cite journal |vauthors=Abkowitz JL, Powell JS, Nakamura JM, Kadin ME, Adamson JW |title=Pure red cell aplasia: response to therapy with anti-thymocyte globulin |journal=Am. J. Hematol. |volume=23 |issue=4 |pages=363–71 |date=December 1986 |pmid=3098093 |doi= |url=}}</ref>
 
** Rituximab: Anti-CD20 monoclonal antibody <ref name="pmid118070202">{{cite journal |vauthors=Ghazal H |title=Successful treatment of pure red cell aplasia with rituximab in patients with chronic lymphocytic leukemia |journal=Blood |volume=99 |issue=3 |pages=1092–4 |date=February 2002 |pmid=11807020 |doi= |url=}}</ref>
OR
** Alemtuzumab: Anti-CD52 monoclonal antibody<ref name="pmid145319092">{{cite journal |vauthors=Ru X, Liebman HA |title=Successful treatment of refractory pure red cell aplasia associated with lymphoproliferative disorders with the anti-CD52 monoclonal antibody alemtuzumab (Campath-1H) |journal=Br. J. Haematol. |volume=123 |issue=2 |pages=278–81 |date=October 2003 |pmid=14531909 |doi= |url=}}</ref>
 
** Daclizumab: Anti-interleukin monoclonal receptor antibody <ref name="pmid164619622">{{cite journal |vauthors=Sloand EM, Scheinberg P, Maciejewski J, Young NS |title=Brief communication: Successful treatment of pure red-cell aplasia with an anti-interleukin-2 receptor antibody (daclizumab) |journal=Ann. Intern. Med. |volume=144 |issue=3 |pages=181–5 |date=February 2006 |pmid=16461962 |doi= |url=}}</ref>
[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===
===Surgery===
Surgical intervention is not recommended for the management of [disease name].
Surgery is usually reserved for patients with thymoma. <ref name="pmid65818394">{{cite journal |vauthors=Clark DA, Dessypris EN, Krantz SB |title=Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients |journal=Blood |volume=63 |issue=2 |pages=277–86 |date=February 1984 |pmid=6581839 |doi= |url=}}</ref>
 
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===
===Primary Prevention===
There are no established measures for the primary prevention of [disease name].
There are no established measures for the primary prevention of pure red cell aplasia
 
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===
===Secondary Prevention===
There are no established measures for the secondary prevention of [disease name].
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==
==References==
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{{SI}}
{{SI}}
{{CMG}}{{MA}} [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu]
{{CMG}}{{MA}} [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu]


{{SK}} Acquired pure megakaryocytic aplasia, pure red cell aplasia, erythroblastopenia
{{SK}} Acquired pure megakaryocytic aplasia, pure red cell aplasia, erythroblastopenia

Revision as of 19:50, 11 September 2018

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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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Overview

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.

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.[2].In autoimmune disorders, IgG fraction in serum inhibit the growth of normal erythroid progenitors. [3] In some cases of autoimmune PRCA, T lymphocytes suppress erythropoiesis. [4]


Causes

  • Autoimmune disease
    • Autoimmune hemolytic anemia
    • Systemic lupus erythematosus
    • Rheumatoid arthritis
  • Thymoma[5]
  • Viral infections
    • HIV
    • Herpes
    • Parvovirus B19 (Fifth disease)[6]
    • Hepatitis such as hepatitis C[7]
  • Lymphoproliferative disorders
    • T-cell large granular lymphocyte leukemia, especially in china [8]
    • Chronic lymphocytic leukemia
    • Hodgkin lymphoma
    • Non-Hodgkin lymphoma
  • Myeloid malignancies such as Chronic myeloid leukemia
  • Myelodysplastic syndrome[9]
  • Idiopathic[10]
  • Drugs [11][12][13]
    • 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[14]
  • Plasma cell disorders[15]
  • 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 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 incidence of thymoma in patients with pure red cell aplasia is about 5%. [16]

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.[17]

Pure red cell aplasia due to parvovirus infection usually resolve within 2-3 weeks. [18]

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. [19]

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:[20]

  • 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. [21] IVIG, 400 mg/kg daily for five day can be considered in resistant pure red cell aplasia. [22]
  • Immunosuppressive therapy in idiopathic pure red cell aplasia such as: [23]
    • Glucocorticoids: Prednisone, oral dose ( 60 mg/day in divided doses ). It is considerd as a initial treatment. [24]
    • 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.[25]
    • Glucocorticoids plus cyclophosphamide: Cyclophosphamide oral dosage can be considered 2 to 3 mg/kg per day. [26]
  • Refractory cases:
    • Azathioprine (2 to 3 mg/kg per day)
    • Antilymphocyte globulin
    • Antithymocyte globulin[27]
    • Rituximab: Anti-CD20 monoclonal antibody [28]
    • Alemtuzumab: Anti-CD52 monoclonal antibody[29]
    • Daclizumab: Anti-interleukin monoclonal receptor antibody [30]

Surgery

Surgery is usually reserved for patients with thymoma. [31]

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

  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. ↑ 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.
  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. ↑ 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.
  19. ↑ 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.
  20. ↑ 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.
  21. ↑ 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.
  22. ↑ 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.
  23. ↑ 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.
  24. ↑ 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.
  25. ↑ Raghavachar A (1990). "Pure red cell aplasia: review of treatment and proposal for a treatment strategy". Blut. 61 (2–3): 47–51. PMID 1698487.
  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. ↑ 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.
  28. ↑ 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.
  29. ↑ 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.
  30. ↑ 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.
  31. ↑ 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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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]

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.

Historical Perspective

Classification

Pathophysiology

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.

Differentiating [Disease] from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Drug Side Effect

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:

Surgery

Prevention

See also

External links

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