Evans syndrome 1657789 226183069 2008-07-17T05:38:57Z Oda Mari 4320462 Reverted 1 edit by [[Special:Contributions/128.23.61.34|128.23.61.34]] identified as [[WP:VAND|vandalism]] to last revision by [[User:DOI bot|DOI bot]]. using [[WP:TWINKLE|TW]] {{expert}} {{Infobox_Disease | Name = {{PAGENAME}} | Image = | Caption = | DiseasesDB = 29724 | ICD10 = {{ICD10|D|69|3|d|65}} | ICD9 = {{ICD9|287.32}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = ped | eMedicineTopic = 721 | MeshID = }} '''Evans' Syndrome''' is an [[autoimmune]] disease in which an individual's [[antibodies]] attack their own [[Red blood cell|RBC]]s as well as their [[platelets]],<ref name="pmid14782741">{{cite journal |author=Evans RS, Takahashi K, Duane RT, Payne R, Liu C |title=Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology |journal=A.M.AARRAYrchives of internal medicine |volume=87 |issue=1 |pages=48–65 |year=1951 |pmid=14782741 |doi=}}</ref> both of these events may occur simultaneously or one follow on from the other.<ref name="pmid16398647">{{cite journal |author=Norton A, Roberts I |title=Management of Evans syndrome |journal=Br. J. Haematol. |volume=132 |issue=2 |pages=125–37 |year=2006 |pmid=16398647 |doi=10.1111/j.1365-2141.2005.05809.x}}</ref> Its overall pathology approximates to a combination of the two [[autoimmune]] induced conditions: [[autoimmune hemolytic anemia]] and [[immune thrombocytopenic purpura]].<ref name="pmid14782741" /> '''Autoimmune hemolytic anemia''' is a condition in which the [[red blood cells]] that normally carry [[oxygen]] and [[carbon dioxide]] are destroyed by an [[autoimmune]] process. '''Immune thrombocytopenic purpura''' is a condition in which the [[platelets]] in the [[blood]] are destroyed by an [[autoimmune]] process. Platelets are a component of blood that contribute to the formation of [[blood clot]]s in the body to prevent [[bleeding]]. ==Epidemiology== <!-- see eMedicine article - need extract out primary sources for information --> ==Signs and symptoms== It has been variously reported that between 10%<ref>{{GPnotebook|-919273445|Evan's syndrome}}</ref> and 23%<ref name="pmid2632179">{{cite journal |author=Cai JR, Yu QZ, Zhang FQ |title=[Autoimmune hemolytic anemia: clinical analysis of 100 cases] |language=Chinese |journal=Zhonghua Nei Ke Za Zhi |volume=28 |issue=11 |pages=670–3, 701–2 |year=1989 |pmid=2632179 |doi=}}</ref> of patients who have autoimmune haemolytic anaemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.<ref name="pmid1451398">{{cite journal |author=Ng SC |title=Evans syndrome: a report on 12 patients |journal=Clinical and laboratory haematology |volume=14 |issue=3 |pages=189–93 |year=1992 |pmid=1451398 |doi=}}</ref> ==Causes== The precise cause is not yet known, but the immunology does differ from patients with just ITP with decreased T4 (T-helper), increased T8 (T-suppressor) and a decreased T4:T8 ratio. Together with decreased serum levels of [[IgG]], [[IgM]], and [[IgA]] these findings suggest an unsuccessful autoimmune response to an unknown trigger.<ref name="pmid6606357">{{cite journal |author=Wang W, Herrod H, Pui CH, Presbury G, Wilimas J |title=Immunoregulatory abnormalities in Evans syndrome |journal=Am. J. Hematol. |volume=15 |issue=4 |pages=381–90 |year=1983 |pmid=6606357 |doi=10.1002/ajh.2830150409}}</ref> ==Diagnosis== The diagnosis is made upon blood tests to confirm not only haemolytic anaemia and immune thrombocytopenic purpura, but also a positive [[Coombs test|direct antiglobulin test]] (DAT) and an absence of any known underlying aetiology.<ref name="pmid16398647">{{cite journal |author=Norton A, Roberts I |title=Management of Evans syndrome |journal=Br. J. Haematol. |volume=132 |issue=2 |pages=125–37 |year=2006 |pmid=16398647 |doi=10.1111/j.1365-2141.2005.05809.x}}</ref> Other antibodies may occur directed against neutrophils and lymphocytes,<ref name="pmid7104228">{{cite journal |author=Pegels JG, Helmerhorst FM, van Leeuwen EF, van de Plas-van Dalen C, Engelfriet CP, von dem Borne AE |title=The Evans syndrome: characterization of the responsible autoantibodies |journal=Br. J. Haematol. |volume=51 |issue=3 |pages=445–50 |year=1982 |pmid=7104228 |doi=10.1111/j.1365-2141.1982.tb02801.x}}</ref> and "immunopancytopenia" has been suggested as a better term for this syndrome.<ref name="pmid7191890">{{cite journal |author=Pui CH, Wilimas J, Wang W |title=Evans syndrome in childhood |journal=J. Pediatr. |volume=97 |issue=5 |pages=754–8 |year=1980 |pmid=7191890 |doi=10.1016/S0022-3476(80)80258-7}}</ref> ==Treatment== Initial treatment is with [[glucocorticoid]] [[corticosteroid]]s or [[intravenous immunoglobulin]],<ref name="pmid2438958">{{cite journal |author=Nuss R, Wang W |title=Intravenous gamma globulin for thrombocytopenia in children with Evans syndrome |journal=The American journal of pediatric hematology/oncology |volume=9 |issue=2 |pages=164–7 |year=1987 |pmid=2438958 |doi=}}</ref><ref name="pmid1483999">{{cite journal |author=Mehta JB, Singhal SB, Mehta BC |title=Intravenous immunoglobulin therapy of immune thrombocytopenia |journal=The Journal of the Association of Physicians of India |volume=40 |issue=5 |pages=340–2 |year=1992 |pmid=1483999 |doi=}}</ref> and particularly in children autoimmune hemolytic anemia is often an acute illness that responds in 80% to a short steroid course.<ref name="pmid12531800">{{cite journal |author=Zecca M, Nobili B, Ramenghi U, ''et al'' |title=Rituximab for the treatment of refractory autoimmune hemolytic anemia in children |journal=Blood |volume=101 |issue=10 |pages=3857–61 |year=2003 |month=15 May |pmid=12531800 |doi=10.1182/blood-2002-11-3547 |url=http://bloodjournal.hematologylibrary.org/cgi/content/full/101/10/3857}}</ref> Although the majority of cases initially respond well, the condition often relapses and [[immunosuppressive]] drugs (e.g. [[ciclosporin]],<ref name="pmid8639426">{{cite journal |author=Emilia G, Messora C, Longo G, Bertesi M |title=Long-term salvage treatment by cyclosporin in refractory autoimmune haematological disorders |journal=Br. J. Haematol. |volume=93 |issue=2 |pages=341–4 |year=1996 |pmid=8639426 |doi=10.1046/j.1365-2141.1996.4871026.x}}</ref><ref name="pmid11855146">{{cite journal |author=Liu H, Shao Z, Jing L |title=[The effectiveness of cyclosporin A in the treatment of autoimmune hemolytic anemia and Evans syndrome] |language=Chinese |journal=Zhonghua Xue Ye Xue Za Zhi |volume=22 |issue=11 |pages=581–3 |year=2001 |pmid=11855146 |doi=}}</ref> [[mycophenolate mofetil]], [[vincristine]]<ref name="pmid1404865">{{cite journal |author=Yokoyama K, Kojima M, Komatsumoto S, ''et al'' |title=[Thrombotic thrombocytopenic purpura achieving complete remission by slow infusion of vincristine] |language=Japanese |journal=Rinsho Ketsueki |volume=33 |issue=8 |pages=1084–9 |year=1992 |pmid=1404865 |doi=}}</ref> and [[danazol]]<ref name="pmid8492411">{{cite journal |author=Koike M, Ishiyama T, Saito K, ''et al'' |title=[Effective danazol therapy for a patient with Evans syndrome] |language=Japanese |journal=Rinsho Ketsueki |volume=34 |issue=2 |pages=143–6 |year=1993 |pmid=8492411 |doi=}}</ref>) are then used,<ref name="pmid16398647">{{cite journal |author=Norton A, Roberts I |title=Management of Evans syndrome |journal=Br. J. Haematol. |volume=132 |issue=2 |pages=125–37 |year=2006 |pmid=16398647 |doi=10.1111/j.1365-2141.2005.05809.x}}</ref> or combinations of these.<ref name="pmid7583383">{{cite journal |author=Scaradavou A, Bussel J |title=Evans syndrome. Results of a pilot study utilizing a multiagent treatment protocol |journal=J. Pediatr. Hematol. Oncol. |volume=17 |issue=4 |pages=290–5 |year=1995 |pmid=7583383 |doi=}}</ref> A drug, [[Rituxan]], has given some good results in acute and refractory Evans syndrome cases,<ref name="pmid12531800">{{cite journal |author=Zecca M, Nobili B, Ramenghi U, ''et al'' |title=Rituximab for the treatment of refractory autoimmune hemolytic anemia in children |journal=Blood |volume=101 |issue=10 |pages=3857–61 |year=2003 |month=15 May |pmid=12531800 |doi=10.1182/blood-2002-11-3547 |url=http://bloodjournal.hematologylibrary.org/cgi/content/full/101/10/3857}}</ref><ref>{{cite journal |author=Shanafelt TD, Madueme HL, Wolf RC, Tefferi A |title=Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome |journal=Mayo Clin. Proc. |volume=78 |issue=11 |pages=1340–6 |year=2003 |month=Nov |pmid=14601692 |doi= |url=http://www.mayoclinicproceedings.com/pdf/7811/7811a3.pdf |format=PDF}}</ref> although further relapse may occur within a year.<ref name="pmid16398647"/> Surgical [[splenectomy]] is also used in some cases,<ref name="pmid16124452">{{cite journal |author=Hamidah A, Thambidorai CR, Jamal R |title=Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review |journal=Southeast Asian J. Trop. Med. Public Health |volume=36 |issue=3 |pages=762–4 |year=2005 |pmid=16124452 |doi=}}</ref> but may give only transient benefit in some.<ref name="pmid9329465">{{cite journal |author=Mathew P, Chen G, Wang W |title=Evans syndrome: results of a national survey |journal=J. Pediatr. Hematol. Oncol. |volume=19 |issue=5 |pages=433–7 |year=1997 |pmid=9329465 |doi=10.1097/00043426-199709000-00005}}</ref> The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic [[stem cell transplantation]] (SCT).<ref name="pmid9313889">{{cite journal |author=Martino R, Sureda A, Brunet S |title=Peripheral blood stem cell mobilization in refractory autoimmune Evans syndrome: a cautionary case report |journal=Bone Marrow Transplant. |volume=20 |issue=6 |pages=521 |year=1997 |pmid=9313889 |doi=10.1038/sj.bmt.1700924 |url=http://www.nature.com/bmt/journal/v20/n6/pdf/1700924a.pdf |format=PDF}}</ref><ref name="pmid11781654">{{cite journal |author=Oyama Y, Papadopoulos EB, Miranda M, Traynor AE, Burt RK |title=Allogeneic stem cell transplantation for Evans syndrome |journal=Bone Marrow Transplant. |volume=28 |issue=9 |pages=903–5 |year=2001 |pmid=11781654 |doi=10.1038/sj.bmt.1703237 |url=http://www.nature.com/bmt/journal/v28/n9/full/1703237a.html}}</ref> ==Prognosis== Evan's Syndrome is rare, serious, and has a reported mortality rate of 7%.{{fact|date=September 2007}}<!-- thi svakue from eMedicine article, but we should cite primary source for this --> It has been observed that there is a risk of developing other autoimmune problems and [[hypogammaglobulinemia]],<ref name="pmid3071168">{{cite journal |author=Wang WC |title=Evans syndrome in childhood: pathophysiology, clinical course, and treatment |journal=The American journal of pediatric hematology/oncology |volume=10 |issue=4 |pages=330–8 |year=1988 |pmid=3071168 |doi=}}</ref> with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- [[T cells]] which is a strong predictor for having [[autoimmune lymphoproliferative syndrome]].<ref name="pmid15542578">{{cite journal |author=Teachey DT, Manno CS, Axsom KM, ''et al'' |title=Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS) |journal=Blood |volume=105 |issue=6 |pages=2443–8 |year=2005 |pmid=15542578 |doi=10.1182/blood-2004-09-3542 |url=http://bloodjournal.hematologylibrary.org/cgi/content/full/105/6/2443}}</ref> ==References== {{reflist|2}} {{Hematology}} [[Category:Blood disorders]] [[pl:Zespół Evansa]]