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==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. | |||
A [[congenital]] form of PRCA was described by Diamond and Blackfan in 1938. | |||
==Classification== | ==Classification== | ||
| Line 32: | Line 31: | ||
==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> | * 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== | ||
| Line 78: | Line 77: | ||
==Differentiating Pure Red Cell Aplasia from Other Diseases== | ==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]] | 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]]: Itr is self-limited condition during first years of life. | * [[Transient erythroblastopenia of childhood]]: Itr is self-limited condition during first years of life. | ||
| Line 85: | Line 84: | ||
==Epidemiology and Demographics== | ==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 Diamond-Blackfan anemia (DBA) is approximately 6.6 per 100,000 individuals in Erope. Pure red cell aplasia due to [[Diamond-Blackfan anemia]] ([[DBA]]) affects men and women equally and there is no racial predilection to this disease. | ||
* The acquired form of pure red cell aplasia can presents as an [[acute]] self-limited disease predominantly in children or [[chronic]] illness that is more seen in adults.<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> | * 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> | ||
* 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> | * 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> | ||
==Risk Factors== | ==Risk Factors== | ||
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==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
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> | ||
* 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> | |||
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> | |||
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> | |||
==Diagnosis== | ==Diagnosis== | ||
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===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> | 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> | ||
* [[Normocytic anemia|Normocytic]], [[Normochromic anemia|normochromic]] [[anemia]]; rarely, [[macrocytic anemia]] may be seen | * [[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 | * Very low or zero [[reticulocyte]] percentage and an [[absolute reticulocyte count]] <10,000/microL | ||
* Normal [[White blood cells|white blood cell]] | * Normal [[White blood cells|white blood cell]] | ||
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===Electrocardiogram=== | ===Electrocardiogram=== | ||
There are no ECG findings associated with pure red cell aplasia | There are no ECG findings associated with pure red cell aplasia. | ||
===X-ray=== | ===X-ray=== | ||
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===CT scan=== | ===CT scan=== | ||
[[Chest]] CT scan may be helpful in the diagnosis of thymoma and other neoplasms | [[Chest]] CT scan may be helpful in the diagnosis of thymoma and other neoplasms. | ||
===MRI=== | ===MRI=== | ||
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* Cessation of offending drugs | * Cessation of offending drugs | ||
* Investigation for associated condition | * Investigation for associated condition | ||
* | * Self limited if due to [[ABO blood group system|ABO]] incompatible [[hematopoietic cell]] [[transplantation]] | ||
* Intravenous [[immune globulin]] ([[Intravenous immunoglobulin|IVIG]]): | * 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> | * [[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> | ||
** [[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]]: [[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> | ** [[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> | ||
** [[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> | ** [[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: | * [[Refractory]] cases: | ||
** [[Azathioprine]] (2 to 3 mg/kg per day) | ** [[Azathioprine]] (2 to 3 mg/kg per day) | ||
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** [[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> | ** [[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> | ||
** [[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> | ** [[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> | ** [[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> | ||
===Surgery=== | ===Surgery=== | ||
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> | [[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> | ||
===Primary Prevention=== | ===Primary Prevention=== | ||
Revision as of 12:26, 1 November 2018
Template:DiseaseDisorder infobox
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]
Synonyms and keywords: Pure red cell aplasia; Erythroblastopenia.
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 Erope. 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
- 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
- Myeloid malignancies such as Chronic myeloid leukemia
- Myelodysplastic syndrome[9]
- Idiopathic[10]
- Drugs [11][12][13]
- ABO- incompatible hematopoietic cell transplantation
- Anti- erythropoietin antibodies due to treatment with recombinant human erythropoietin[14]
- Plasma cell disorders[15]
- Pregnancy
Differentiating Pure Red Cell Aplasia from Other Diseases
Pure red cell aplasia must be differentiated from Transient erythroblastopenia of childhood, Diamond-Blackfan anemia (DBA) and Aplastic anemia:
- Transient erythroblastopenia of childhood: Itr is self-limited condition during first years of life.
- Diamond-Blackfan anemia (DBA): congenital form of red cell aplasia. It is associated with some malignancies and it does not respond to prednisone.
- Aplastic anemia: It affects other bone marrow cells as well.
Epidemiology and Demographics
- The incidence of Diamond-Blackfan anemia (DBA) is approximately 6.6 per 100,000 individuals in Erope. Pure red cell aplasia due to Diamond-Blackfan anemia (DBA) affects men and women equally and there is no racial predilection to this disease.
- The acquired form of pure red cell aplasia can presents as an acute self-limited disease predominantly in children or chronic illness that is more seen in adults.[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
- Complete blood count, peripheral smear, reticulocyte count[21]
- Hepatic function test
- Renal function test
- Bone marrow aspiration and biopsy
History and Symptoms
Common symptoms of pure red cell aplasia include fatigue and lethargy.
Physical Examination
Common physical examination findings of pure red cell aplasia include fast heart beat and pale apperance.
Laboratory Findings
Laboratory findings consistent with the diagnosis of pure red cell aplasia include:[22]
- 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 biopsy: 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
- Self limited if due to ABO incompatible hematopoietic cell transplantation
- Intravenous immune globulin (IVIG): Single infusion 400 mg/kg over 2-3 hours if spontaneous resolution does not occur during 2-3 weeks.[23] IVIG, 400 mg/kg daily for five day can be considered in resistant pure red cell aplasia.[24]
- Immunosuppressive therapy in idiopathic pure red cell aplasia such as:[25]
- Glucocorticoids: Prednisone, oral dose ( 60 mg/day in divided doses ). It is considerd as a initial treatment.[26]
- 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.[27]
- Glucocorticoids plus cyclophosphamide: Cyclophosphamide oral dosage can be considered 2 to 3 mg/kg per day.[28]
- Refractory cases:
- Azathioprine (2 to 3 mg/kg per day)
- Antilymphocyte globulin
- Antithymocyte globulin[29]
- Rituximab: Anti-CD20 monoclonal antibody [30]
- Alemtuzumab: Anti-CD52 monoclonal antibody[31]
- Daclizumab: Anti-interleukin monoclonal receptor antibody [32]
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
- ↑ 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.
- ↑ Dessypris EN (October 1991). "The biology of pure red cell aplasia". Semin. Hematol. 28 (4): 275–84. PMID 1759168.
- ↑ Dessypris EN (October 1991). "The biology of pure red cell aplasia". Semin. Hematol. 28 (4): 275–84. PMID 1759168.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Dessypris EN (October 1991). "The biology of pure red cell aplasia". Semin. Hematol. 28 (4): 275–84. PMID 1759168.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Thompson DF, Gales MA (1996). "Drug-induced pure red cell aplasia". Pharmacotherapy. 16 (6): 1002–8. PMID 8947971.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Raghavachar A (1990). "Pure red cell aplasia: review of treatment and proposal for a treatment strategy". Blut. 61 (2–3): 47–51. PMID 1698487.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
See also
- Diamond-Blackfan anemia (genetic red cell aplasia)
- aplastic anemia (aplasia affecting other bone marrow cells as well)