Evans syndrome

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


Wikimedia Foundation. 2010.

Игры ⚽ Нужно сделать НИР?

Look at other dictionaries:

  • Evans syndrome — Ev·ans syndrome (evґənz) [Robert Sherman Evans, American physician, 1912–1974] see under syndrome …   Medical dictionary

  • Evans syndrome — autoimmune hemolytic anemia accompanied by immune thrombocytopenia …   Medical dictionary

  • Howel-Evans syndrome — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 33404 ICD10 = ICD9 = ICDO = OMIM = 148500 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = Howel Evans syndrome is an extremely rare condition in which the skin of the palms of the… …   Wikipedia

  • Howel-Evans syndrome — How·el Ev·ans syndrome (houґəl evґənz) [William Howel Evans, British physician, 1925–1997] see under syndrome …   Medical dictionary

  • Syndrome — Pour les articles homonymes, voir syndrome (homonymie). Un syndrome est un ensemble de signes cliniques et de symptômes qu un patient est susceptible de présenter lors de certaines maladies, ou bien dans des circonstances cliniques d écart à la… …   Wikipédia en Français

  • Syndrome d'Evans — Classification et ressources externes CIM 10 D69.3 CIM 9 287.32 DiseasesDB …   Wikipédia en Français

  • Syndrôme — Syndrome Pour les articles homonymes, voir syndrome (homonymie). Un syndrome est un ensemble de signes cliniques et de symptômes qu un patient est susceptible de présenter lors de certaines maladies, ou bien dans des circonstances cliniques d… …   Wikipédia en Français

  • Syndrome de Gilbert — Classification et ressources externes CIM 10 E80.4 CIM …   Wikipédia en Français

  • Syndrome post PL — Syndrome post ponction lombaire La ponction lombaire est un geste invasif qui se complique fréquemment de céphalées, parfois très sévères et invalidantes. Le syndrome post ponction lombaire, survenant après la réalisation d une ponction lombaire …   Wikipédia en Français

  • Syndrome post pl — Syndrome post ponction lombaire La ponction lombaire est un geste invasif qui se complique fréquemment de céphalées, parfois très sévères et invalidantes. Le syndrome post ponction lombaire, survenant après la réalisation d une ponction lombaire …   Wikipédia en Français

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”