Evans syndrome

Infobox_Disease
Name = PAGENAME


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 cells and platelets.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=] Both of these events may occur simultaneously or one follow on from the other.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]

Its overall pathology resembles a combination of autoimmune hemolytic anemia and immune thrombocytopenic purpura. 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 platelets are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.

Epidemiology

igns and symptoms

It has been variously reported that between 10% [GPnotebook|-919273445|Evan's syndrome] and 23%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=] of patients who have autoimmune haemolytic anaemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.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=]

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.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]

Diagnosis

The diagnosis is made upon blood tests to confirm not only haemolytic anaemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying aetiology.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]

Other antibodies may occur directed against neutrophils and lymphocytes,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] and "immunopancytopenia" has been suggested as a better term for this syndrome.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]

Treatment

Initial treatment is with glucocorticoid corticosteroids or intravenous immunoglobulin,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=] 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=] and particularly in children autoimmune hemolytic anemia is often an acute illness that responds in 80% to a short steroid course.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]

Although the majority of cases initially respond well, the condition often relapses and immunosuppressive drugs (e.g. ciclosporin,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] 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=] mycophenolate mofetil, vincristinecite 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=] and danazolcite 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=] ) are then used,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] or combinations of these.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=]

A drug, Rituxan, has given some good results in acute and refractory Evans syndrome cases,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] [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] although further relapse may occur within a year.

Surgical splenectomy is also used in some cases,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=] but may give only transient benefit in some.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]

The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic stem cell transplantation (SCT).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] 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]

Prognosis

Evan's Syndrome is rare, serious, and has a reported mortality rate of 7%.fact|date=September 2007

It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia,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=] 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.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]

Resources

* [http://health.groups.yahoo.com/group/EvansSyndrome/?v=1&t=search&ch=web&pub=groups&sec=group&slk=3 Evans Syndrome discussion group]

References