Pure red cell aplasia: Difference between revisions

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{{SK}} Acquired pure megakaryocytic aplasia, pure red cell aplasia, erythroblastopenia
{{SK}} Pure red cell aplasia; Erythroblastopenia.


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==Overview==
==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 blood group system|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 tests|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 anemia|normocytic]], [[Normochromic anemia|normochromic]] [[anemia]], very low or zero [[reticulocyte]] percentage and an [[absolute reticulocyte count]] <10,000/microL, normal [[White blood cells|white blood cell]] and [[platelets]]. An [[x-ray]] may be helpful in the diagnosis of [[thymoma]] and other [[Neoplasm|neoplasms]]. Medical therapy include [[red blood cell]] [[Blood transfusion|transfusion]] in symptomatic [[anemia]], cessation of offending drugs, Intravenous [[immune globulin]] ([[Intravenous immunoglobulin|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==
==Historical Perspective==
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>
 
* A [[congenital]] form of PRCA was described by Diamond and Blackfan in 1938.  
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==
==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.
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]].
 
●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==
==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]].
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.<ref name="pmid17591682">{{cite journal |vauthors=Dessypris EN |title=The biology of pure red cell aplasia |journal=Semin. Hematol. |volume=28 |issue=4 |pages=275–84 |date=October 1991 |pmid=1759168 |doi= |url=}}</ref>.In autoimmune disorders, IgG fraction in serum inhibit the growth of normal erythroid progenitors. <ref name="pmid17591683">{{cite journal |vauthors=Dessypris EN |title=The biology of pure red cell aplasia |journal=Semin. Hematol. |volume=28 |issue=4 |pages=275–84 |date=October 1991 |pmid=1759168 |doi= |url=}}</ref> In some cases of autoimmune PRCA, T lymphocytes suppress erythropoiesis. <ref name="pmid8639922">{{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>
* The numbers of [[white blood cells]] and [[platelets]] are normal.<ref name="pmid17591682">{{cite journal |vauthors=Dessypris EN |title=The biology of pure red cell aplasia |journal=Semin. Hematol. |volume=28 |issue=4 |pages=275–84 |date=October 1991 |pmid=1759168 |doi= |url=}}</ref>  
 
* In [[autoimmune disorders]], IgG fraction in serum inhibit the growth of normal erythroid progenitors.<ref name="pmid17591683">{{cite journal |vauthors=Dessypris EN |title=The biology of pure red cell aplasia |journal=Semin. Hematol. |volume=28 |issue=4 |pages=275–84 |date=October 1991 |pmid=1759168 |doi= |url=}}</ref>  
 
* In some cases of [[autoimmune]] PRCA, [[T lymphocytes]] suppress [[erythropoiesis]].<ref name="pmid8639922">{{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>


==Causes==
==Causes==
* Autoimmune disease
* [[Autoimmune disease]]
** Autoimmune hemolytic anemia
** [[Autoimmune hemolytic anemia]]
** Systemic lupus erythematosus
** [[Systemic lupus erythematosus]]
** Rheumatoid arthritis
** [[Rheumatoid arthritis]]
* Thymoma<ref name="pmid18166782">{{cite journal |vauthors=Hirokawa M, Sawada K, Fujishima N, Nakao S, Urabe A, Dan K, Fujisawa S, Yonemura Y, Kawano F, Omine M, Ozawa K |title=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 |journal=Haematologica |volume=93 |issue=1 |pages=27–33 |date=January 2008 |pmid=18166782 |doi=10.3324/haematol.11655 |url=}}</ref>
* [[Thymoma]]<ref name="pmid18166782">{{cite journal |vauthors=Hirokawa M, Sawada K, Fujishima N, Nakao S, Urabe A, Dan K, Fujisawa S, Yonemura Y, Kawano F, Omine M, Ozawa K |title=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 |journal=Haematologica |volume=93 |issue=1 |pages=27–33 |date=January 2008 |pmid=18166782 |doi=10.3324/haematol.11655 |url=}}</ref>
* Viral infections  
* [[Viral]] infections  
** HIV  
** [[HIV]]
** Herpes  
** [[Herpes]]
** Parvovirus B19 (Fifth disease)<ref name="pmid11127313">{{cite journal |vauthors=Geetha D, Zachary JB, Baldado HM, Kronz JD, Kraus ES |title=Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature |journal=Clin Transplant |volume=14 |issue=6 |pages=586–91 |date=December 2000 |pmid=11127313 |doi= |url=}}</ref>
** [[Parvovirus B19]] ([[Fifth disease]])<ref name="pmid11127313">{{cite journal |vauthors=Geetha D, Zachary JB, Baldado HM, Kronz JD, Kraus ES |title=Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature |journal=Clin Transplant |volume=14 |issue=6 |pages=586–91 |date=December 2000 |pmid=11127313 |doi= |url=}}</ref>
** Hepatitis such as hepatitis C<ref name="pmid9258213">{{cite journal |vauthors=al-Awami Y, Sears DA, Carrum G, Udden MM, Alter BP, Conlon CL |title=Pure red cell aplasia associated with hepatitis C infection |journal=Am. J. Med. Sci. |volume=314 |issue=2 |pages=113–7 |date=August 1997 |pmid=9258213 |doi= |url=}}</ref>
** [[Hepatitis]] such as [[hepatitis C]]<ref name="pmid9258213">{{cite journal |vauthors=al-Awami Y, Sears DA, Carrum G, Udden MM, Alter BP, Conlon CL |title=Pure red cell aplasia associated with hepatitis C infection |journal=Am. J. Med. Sci. |volume=314 |issue=2 |pages=113–7 |date=August 1997 |pmid=9258213 |doi= |url=}}</ref>
* Lymphoproliferative disorders  
* [[Lymphoproliferative disorders]]
** T-cell large granular lymphocyte leukemia, especially in china <ref name="pmid9930071">{{cite journal |vauthors=Kwong YL, Wong KF |title=Association of pure red cell aplasia with T large granular lymphocyte leukaemia |journal=J. Clin. Pathol. |volume=51 |issue=9 |pages=672–5 |date=September 1998 |pmid=9930071 |pmc=500904 |doi= |url=}}</ref>
** [[T-cell large granular lymphocyte leukemia]], especially in china <ref name="pmid9930071">{{cite journal |vauthors=Kwong YL, Wong KF |title=Association of pure red cell aplasia with T large granular lymphocyte leukaemia |journal=J. Clin. Pathol. |volume=51 |issue=9 |pages=672–5 |date=September 1998 |pmid=9930071 |pmc=500904 |doi= |url=}}</ref>
** Chronic lymphocytic leukemia
** [[Chronic lymphocytic leukemia]]
** Hodgkin lymphoma
** [[Hodgkin lymphoma]]
** Non-Hodgkin lymphoma
** [[Non-Hodgkin lymphoma]]


* Myeloid malignancies such as Chronic myeloid leukemia
* [[Myeloid]] malignancies such as [[chronic myeloid leukemia]]
* Myelodysplastic syndrome<ref name="pmid1759168">{{cite journal |vauthors=Dessypris EN |title=The biology of pure red cell aplasia |journal=Semin. Hematol. |volume=28 |issue=4 |pages=275–84 |date=October 1991 |pmid=1759168 |doi= |url=}}</ref>
* [[Myelodysplastic syndrome]]<ref name="pmid1759168">{{cite journal |vauthors=Dessypris EN |title=The biology of pure red cell aplasia |journal=Semin. Hematol. |volume=28 |issue=4 |pages=275–84 |date=October 1991 |pmid=1759168 |doi= |url=}}</ref>
* Idiopathic<ref name="pmid18576941">{{cite journal |vauthors=Miller AC, Rashid RM |title=Three episodes of acquired pure red cell aplasia restricted to pregnancy |journal=J Perinat Med |volume=36 |issue=3 |pages=270–1 |date=2008 |pmid=18576941 |doi=10.1515/JPM.2008.041 |url=}}</ref>
* [[Idiopathic]]<ref name="pmid18576941">{{cite journal |vauthors=Miller AC, Rashid RM |title=Three episodes of acquired pure red cell aplasia restricted to pregnancy |journal=J Perinat Med |volume=36 |issue=3 |pages=270–1 |date=2008 |pmid=18576941 |doi=10.1515/JPM.2008.041 |url=}}</ref>
* Drugs <ref name="pmid18089975">{{cite journal |vauthors=Macdougall IC |title=Epoetin-induced pure red cell aplasia: diagnosis and treatment |journal=Curr. Opin. Nephrol. Hypertens. |volume=16 |issue=6 |pages=585–8 |date=November 2007 |pmid=18089975 |doi=10.1097/MNH.0b013e3282f0c4bf |url=}}</ref><ref name="pmid18353161">{{cite journal |vauthors=Bartakke S, Abdelhaleem M, Carcao M |title=Valproate-induced pure red cell aplasia and megakaryocyte dysplasia |journal=Br. J. Haematol. |volume=141 |issue=2 |pages=133 |date=April 2008 |pmid=18353161 |doi=10.1111/j.1365-2141.2008.06979.x |url=}}</ref><ref name="pmid8947971">{{cite journal |vauthors=Thompson DF, Gales MA |title=Drug-induced pure red cell aplasia |journal=Pharmacotherapy |volume=16 |issue=6 |pages=1002–8 |date=1996 |pmid=8947971 |doi= |url=}}</ref>
* [[Drugs]] <ref name="pmid18089975">{{cite journal |vauthors=Macdougall IC |title=Epoetin-induced pure red cell aplasia: diagnosis and treatment |journal=Curr. Opin. Nephrol. Hypertens. |volume=16 |issue=6 |pages=585–8 |date=November 2007 |pmid=18089975 |doi=10.1097/MNH.0b013e3282f0c4bf |url=}}</ref><ref name="pmid18353161">{{cite journal |vauthors=Bartakke S, Abdelhaleem M, Carcao M |title=Valproate-induced pure red cell aplasia and megakaryocyte dysplasia |journal=Br. J. Haematol. |volume=141 |issue=2 |pages=133 |date=April 2008 |pmid=18353161 |doi=10.1111/j.1365-2141.2008.06979.x |url=}}</ref><ref name="pmid8947971">{{cite journal |vauthors=Thompson DF, Gales MA |title=Drug-induced pure red cell aplasia |journal=Pharmacotherapy |volume=16 |issue=6 |pages=1002–8 |date=1996 |pmid=8947971 |doi= |url=}}</ref>
** Phenytoin  
** [[Phenytoin]]
** Chloramphenicol
** [[Chloramphenicol|Chloramphenico]]<nowiki/>l
** Azathioprine
** [[Azathioprine]]
** Isoniazid
** [[Isoniazid]]
** Valproic acid
** [[Valproic acid]]
** Erythropoietin
** [[Erythropoietin]]
** Trimethoprim-sulfamethoxazole
** [[Sulfamethoxazole-Trimethoprim|Trimethoprim-sulfamethoxazole]]
** Zidovudine
** [[Zidovudine]]
** Chlorpropamide
** [[Chlorpropamide]]
**  
**  
* ABO- incompatible hematopoietic cell transplantation
* [[ABO blood group system|ABO]]- incompatible [[hematopoietic cell]] [[transplantation]]
* Anti- erythropoietin antibodies due to treatment with recombinant human erythropoietin<ref name="pmid24120841">{{cite journal |vauthors=Rossert J, Yue S, Smirnakis K, Mytych DT, Johnson L, Kouchakji E, Casadevall N |title=Risk of pure red cell aplasia in patients with hepatitis C receiving antiviral therapy and an erythropoiesis-stimulating agent |journal=Clin. Gastroenterol. Hepatol. |volume=12 |issue=2 |pages=341–5 |date=February 2014 |pmid=24120841 |doi=10.1016/j.cgh.2013.09.065 |url=}}</ref>
* Anti-[[erythropoietin]] antibodies due to treatment with recombinant human [[erythropoietin]]<ref name="pmid24120841">{{cite journal |vauthors=Rossert J, Yue S, Smirnakis K, Mytych DT, Johnson L, Kouchakji E, Casadevall N |title=Risk of pure red cell aplasia in patients with hepatitis C receiving antiviral therapy and an erythropoiesis-stimulating agent |journal=Clin. Gastroenterol. Hepatol. |volume=12 |issue=2 |pages=341–5 |date=February 2014 |pmid=24120841 |doi=10.1016/j.cgh.2013.09.065 |url=}}</ref>
* Plasma cell disorders<ref name="pmid26999424">{{cite journal |vauthors=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 |title=Monoclonal gammopathy-associated pure red cell aplasia |journal=Br. J. Haematol. |volume=173 |issue=6 |pages=876–83 |date=June 2016 |pmid=26999424 |pmc=5549779 |doi=10.1111/bjh.14012 |url=}}</ref>
* [[Plasma cell disorders]]<ref name="pmid26999424">{{cite journal |vauthors=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 |title=Monoclonal gammopathy-associated pure red cell aplasia |journal=Br. J. Haematol. |volume=173 |issue=6 |pages=876–83 |date=June 2016 |pmid=26999424 |pmc=5549779 |doi=10.1111/bjh.14012 |url=}}</ref>
* Pregnancy
* [[Pregnancy]]
*  
*  
*  
*  
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The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
==Differentiating Pure Red Cell Aplasia from Other Diseases==
Pure red cell aplasia must be differentiated from [[transient erythroblastopenia of childhood]], [[Diamond-Blackfan anemia]] ([[Diamond-Blackfan anemia|DBA]]) and [[Aplastic anemia]]:
* [[Transient erythroblastopenia of childhood]]: It is self-limited condition during first years of life.


==Differentiating ((Page name)) from Other Diseases==
* [[Diamond-Blackfan anemia]] ([[DBA]]): [[congenital]] form of red cell [[aplasia]]. It is associated with some [[malignancies]] and it does not respond to [[prednisone]].  
Pure red cell aplasia must be differentiated from Transient erythroblastopenia of childhood, Diamond-Blackfan anemia (DBA) and Aplastic anemia.
* [[Aplastic anemia]]: It affects other [[bone marrow cells]] as well.  
* 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==
==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 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.<ref name="pmid185106824">{{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
* 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>
 
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==
There are no established risk factors for [disease name].
Common risk factor in the development of pure red cell aplasia include strong [[family history]].  
 
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==
==Screening==
There is insufficient evidence to recommend routine screening for [disease/malignancy].
There is insufficient evidence to recommend routine screening for pure red cell aplasia.
 
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==
==Natural History, Complications, and Prognosis==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
* 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>
 
* 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>
OR
* 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>
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==
==Diagnosis==
===Diagnostic Study of Choice===
===Diagnostic Study of Choice===
n all cases of PRCA, a search should be made for an underlying condition, including certain drugs, thymoma, hypoplastic myelodysplastic syndrome, large granular lymphocyte (LGL) leukemia, and viral infection (table 1) (see 'Presentation'above). Appropriate '''initial''' studies for such patients include:
* [[Complete blood count]], [[peripheral smear]], [[reticulocyte count]]<ref name="pmid185106825">{{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>
 
* [[Liver function tests|Hepatic function test]]
●Complete blood count, examination of the peripheral smear, reticulocyte count
* [[Renal function tests|Renal function test]]
 
* [[Bone marrow aspiration]] and [[biopsy]]
●Routine tests of hepatic and renal function
 
●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===
===History and Symptoms===
The majority of patients with [disease name] are asymptomatic.
Common symptoms of pure red cell aplasia include [[fatigue]] and [[lethargy]].
 
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===
===Physical Examination===


Common physical examination findings of pure red cell aplasia include fast heart beat and pale apperance.
Common physical examination findings of pure red cell aplasia include fast heart beat and [[Pale skin|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===
===Laboratory Findings===
 
Laboratory findings consistent with the diagnosis of pure red cell aplasia include:<ref name="pmid185106822">{{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 diagnosis of PRCA is established when '''all''' of the following are present:
* [[Normocytic anemia|Normocytic]], [[Normochromic anemia|normochromic]] [[anemia]]; rarely, [[macrocytic anemia]] may be seen
 
* Very low or zero [[reticulocyte]] percentage and an [[absolute reticulocyte count]] <10,000/microL
●Normocytic, normochromic anemia; rarely, macrocytic
* Normal [[White blood cells|white blood cell]]
 
* Normal [[platelet]] counts
●A very low (or zero) reticulocyte percentage, and an absolute reticulocyte count <10,000/microL
* [[Bone marrow biopsy]]: normal myelopoiesis, [[lymphopoiesis]], and megakaryocytopoiesis, but few [[erythroid]] precursors
 
●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===
===Electrocardiogram===
There are no ECG findings associated with [disease name].
There are no ECG findings associated with pure red cell aplasia.
 
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===
===X-ray===
There are no x-ray findings associated with [disease name].
OR
An x-ray may be helpful in the diagnosis of [disease name]. 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].
An [[x-ray]] may be helpful in the diagnosis of [[thymoma]] and other [[Neoplasm|neoplasms]].


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound findings associated with [disease name].
There are no echocardiography/ultrasound findings associated with pure red cell aplasia.  
 
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===
===CT scan===
There are no CT scan findings associated with [disease name].


OR
[[Chest]] CT scan may be helpful in the diagnosis of thymoma and other neoplasms.  
 
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===MRI===
===MRI===
There are no MRI findings associated with [disease name].


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


[Location] MRI may be helpful in the diagnosis of [disease name]. 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 pure red cell aplasia.  
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===Other 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===
===Other Diagnostic Studies===
There are no other diagnostic studies associated with [disease name].
* [[Viral]] studies for [[hepatitis C]] and [[parvovirus B19]]
* [[Autoimmune]] [[antibody]] studies
* [[Karyotype]]
* [[T cell]] [[receptor]] clonality studies
* [[Peripheral blood]] [[immunophenotyping]]


OR
==Treatment ==
 
[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===
===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]] [[Blood transfusion|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
 
* Self limited if due to [[ABO blood group system|ABO]] incompatible [[hematopoietic cell]] [[transplantation]]
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
* Intravenous [[immune globulin]] ([[Intravenous immunoglobulin|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].
[[Thymectomy]]: 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 pure red cell aplasia.
 
OR
 
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].


==References==
==References==
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{{WikiDoc Sources}}
{{WikiDoc Sources}}


<nowiki>}}</nowiki>
{{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]
{{SK}} 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 simplex virus|herpes]], [[parvovirus B19]] ([[Fifth disease]]), or [[Hepatitis#Viral|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====
* [[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====
{{MedCondContrAbs
|MedCond = Pure red cell aplasia|Darbepoetin Alfa|Methoxy polyethylene glycol-epoetin beta}}
===Surgery===
===Prevention===


==See also==
==See also==

Latest revision as of 03:50, 16 February 2019

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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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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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