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Evans Syndrome Articles

The following is the result of a search of the literature written on Evans Syndrome since 1966.  This Medline search was performed through HealthGate, (http://www.healthgate.com/medline/adv-medline.shtml) and all articles can be obtained from Medline, or ordered through medical libraries.  Not all of the following have abstracts, but as you scroll through, you'll find that many do, and are from all areas of the world. 

 

Title

Danazol for systemic lupus erythematosus with refractory autoimmune thrombocytopenia or Evans' syndrome.

Author

Cervera H; Jara LJ; Pizarro S; Enkerlin HL; Fernandez M; Medina F; Fraga A; Miranda JM

Address

Rheumatic Diseases Unit, Hospital de Especialidades, Mexico City, Mexico.

Source

J Rheumatol, 1995 Oct, 22:10, 1867-71

Abstract

OBJECTIVE: To determine the efficacy of danazol for refractory autoimmune thrombocytopenia or Evans' syndrome complicating systemic lupus erythematosus (SLE). METHODS: We studied 16 consecutive patients with SLE and corticosteroid refractory autoimmune thrombocytopenia; 3 patients had coexisting autoimmune hemolysis (Evans' syndrome). Five patients had undergone splenectomy. Danazol was commenced at 200 mg/day, and increased stepwise (maximum 1200 mg/day) until benefit or toxicity was observed. After remission the danazol dose was gradually reduced to 200-400 mg/day. RESULTS: All 16 patients achieved a complete remission (platelet count >100 x 10(9)/l, hematocrit >39%) 2 months after starting danazol (range 6 weeks-8 months). Remission persisted during continued danazol therapy (mean followup 18.2 months, range 2-49 months). One patient with Evans' syndrome required discontinuation of danazol because of jaundice and biopsy proven minimal hepatic necrosis: hemolysis recurred after discontinuation of danazol. CONCLUSION: Danazol is effective for the treatment of autoimmune thrombocytopenia or Evans' syndrome complicating SLE irrespective of splenectomy status. Longer followup will be needed to determine whether the remission persists after withdrawal of danazol.

Language of Publication

English

Unique Identifier

96116398

 

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Title

Immunoregulatory abnormalities in Evans syndrome.

Author

Wang W; Herrod H; Pui CH; Presbury G; Wilimas J

Address

Source

Am J Hematol, 1983 Dec, 15:4, 381-90

Abstract

Immune function in six patients with Evans syndrome (Coombs-positive hemolytic anemia and immune thrombocytopenia) was compared to that in seven with chronic ITP. The two groups differed in measurements of T-cell subsets and immunoglobulin production. Evans syndrome patients had decreased T4 (T-helper) (P = 0.025), increased T8 (T-suppressor) (P = 0.008), and a decreased ratio of T4:T8 cells (P = 0.0009) when compared to controls. Results in chronic ITP patients were similar to those in controls. Serum IgG, IgM, and IgA levels and in vitro synthesis of IgG and/or IgM were decreased in most Evans syndrome patients. Diminished in vivo and in vitro immunoglobulin synthesis in Evans syndrome is consistent with the decreased T4:T8 ratio in these patients. The altered T4:T8 ratio may represent an unsuccessful response to an autoimmune process in which the trigger is unknown.

Language of Publication

English

 

 

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Familial Evans syndrome: a report of an affected sibship.

Author

McLeod AG; Pai M; Carter RF; Squire J; Barr RD

Address

Department of Hematology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Source

J Pediatr Hematol Oncol, 1999 May, 21:3, 244-7

Abstract

PURPOSE: This report describes the clinical course of three siblings, all of whom had Evans syndrome in childhood. PATIENTS: The coexistence of autoimmune hemolytic anemia and thrombocytopenia, in the absence of a known underlying cause, led to the diagnosis of Evans syndrome in a 4-month-old girl and subsequently in her two brothers when they were 4 and 13 years old. RESULTS: The 4-month-old girl had a life-threatening relapsing course unresponsive to corticosteroids, intravenous gamma-globulin, thymectomy, and cyclophosphamide. She eventually responded to splenectomy. Her two brothers had milder disease that responded to corticosteroids. Cytogenetic analyses revealed the presence of a familial Y;15 translocation in all three children and their father. CONCLUSION: There are few reported cases of familial Evans syndrome, and they are usually associated with an inherited congenital abnormality. We report the unusual finding of three siblings with the disease and no known congenital abnormality.

Language of Publication

English

Unique Identifier

99290528

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Evans syndrome. Results of a pilot study utilizing a multiagent treatment protocol.

Author

Scaradavou A; Bussel J

Address

Department of Pediatrics, Cornell Medical Center-New York Hospital, New York, New York 10021, USA.

Source

J Pediatr Hematol Oncol, 1995 Nov, 17:4, 290-5

Abstract

PURPOSE: We evaluate the efficacy of combination medical therapy in the treatment of ITP and AIHA episodes for patients with Evans syndrome. PATIENTS AND METHODS: Five patients with Evans syndrome were followed for a median of 3.8 years and were treated according to our multiagent (IVIG, steroids, vinca alkaloids, androgens, and cyclosporin) protocol. RESULTS: All patients initially received IVIG and IV steroids for either acute hemolysis or thrombocytopenia. IV vinca alkaloids and oral Danazol were added in three patients for ITP. All responded, but two required frequently repeated vinca alkaloid treatments. Both patients treated with vinca alkaloids for severe hemolysis responded. Two patients received oral cyclosporin for refractory ITP; one had a transient response. Splenectomy was not included in our regimen. Two patients underwent splenectomy prior to their referral; both required further therapy. In addition, review of the clinical courses of our patients indicate that the DAT may be a prognostic factor for the chronicity of ITP. CONCLUSION: In view of the high relapse rates after splenectomy and the encouraging results of our pilot study, we suggest that medical treatment with combination agents (IVIG, steroids, vinca alkaloids, androgens and possibly cyclosporin) may provide a useful therapeutic approach to patients with Evans syndrome.

Language of Publication

English

Unique Identifier

96042199

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Title

Evans' syndrome in a child with diabetes mellitus.

Author

Plevová P; Cermáková R; Lochmanová A; Pták J; Blazek B; Starý J

Address

Department of Pediatrics, University Hospital of Ostrava, Czech Republic.

Source

Pediatr Hematol Oncol, 1998 Jul, 15:4, 353-7

Abstract

A patient suffering from infantile-onset insulin-dependent diabetes mellitus is reported in whom immune pancytopenia (Evans' syndrome) developed at the age of 2 1/2 years. Hepatosplenomegaly, chronic lymphadenopathy, and elevated levels of immunoglobulins G and M were also present. The course of Evans' syndrome was fatal in this patient. The association of Evans' syndrome with other immune disorders is discussed.

Language of Publication

English

Unique Identifier

98322573

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Title

Evans' syndrome associated with Graves' disease.

Author

Yashiro M; Nagoshi H; Kasuga Y; Isobe H; Kitajima S; Nakagawa T; Ohshima J; Ide K; Someya K; Saito N

Address

Third Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki.

Source

Intern Med, 1996 Dec, 35:12, 987-90

Abstract

A 36-year-old woman who had had Graves' disease for 6 years was admitted with severe thrombocytopenia. Evans' syndrome was diagnosed. The patient's family history showed multiple cases of Graves' disease but no cases of Evans' syndrome. Both conditions in this patient improved with corticosteroid and thiamazole therapy. Several autoimmune antibodies were found, but a common autoimmune mechanism was not clearly shown. Although the combination of Graves' disease and Evans' syndrome had not occurred previously in her family, genetic factors may play an important role in the pathogenesis of both conditions.

Language of Publication

English

Unique Identifier

97182862

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Title

Evans syndrome in childhood: pathophysiology, clinical course, and treatment.

Author

Wang WC

Address

Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101.

Source

Am J Pediatr Hematol Oncol, 1988 Win, 10:4, 330-8

Abstract

Evans syndrome is defined as the simultaneous or sequential occurrence of Coombs' positive hemolytic anemia and immune thrombocytopenia without known underlying etiology. Ten cases of Evans syndrome were seen at our hospital over the past decade; three patients died. Two cases are described in detail and demonstrate the chronic, refractory nature of this condition. Most patients have required corticosteroid therapy and splenectomy, but further therapy (e.g., intravenous gammaglobulin, danazol, cyclophosphamide) is usually necessary because of recurrent autoimmune hemolytic anemia, thrombocytopenia, or both. There is a substantial risk for development of other autoimmune problems and hypogammaglobulinemia. A number of defects in humoral immunity have been described in Evans syndrome; different antibodies are directed against platelets and red blood cells. Cellular immunity is probably abnormal, but a distinct pattern of immunoregulatory disturbance has not been identified.

Language of Publication

English

Unique Identifier

89191331

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Title

[Effective cyclosporine therapy to resistant-Evans syndrome]

Author

Tsudo M; Moriguchi T

Address

Internal Medicine, Kyoto-Katsura Hospital.

Source

Rinsho Ketsueki, 1996 Nov, 37:11, 1289-92

Abstract

Evans syndrome is a rare disease defined as autoimmune hemolytic anemia plus immune thrombocytopenia. We describe a 57-year-old man with Evans syndrome whose thrombocytopenia was refractory to conventional therapy, including prednisolone, danazol, azathiopurine, cyclophosphamide, vincristine, gamma-globulin and splenectomy. The patient was then treated with three cycles of pulsed high-dose dexamethasone (40 mg/day for 4 sequential days every 4 weeks) followed by cyclosporine A therapy (300 mg/day). The platelet counts dramatically increased from 2,000/microliter to 200,000/microliter. Although thrombocytopenia appeared again with the tapering of cyclosporine A, the platelet counts recovered by re-increase of cyclosporine A, and have remained above 100,000/microliter for more than 14 months. Treatment with cyclosporine A can provide an effective therapeutic choice for refractory Evans syndrome.

Language of Publication

Japanese

Unique Identifier

97119992

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Title

Dissemination of Epstein-Barr virus associated B-cell lymphoma of the brain after development of immunological incompetence with Evans syndrome.

Author

Hirose Y; Takeshita S; Shimizu S; Ogawa Y; Masaki Y; Fukutoku M; Sugai S; Takiguchi T

Address

Department of Internal Medicine, Kanazawa Medical University, Ishikawa, Japan.

Source

Int J Hematol, 1998 Jan, 67:1, 81-6

Abstract

A 54-year-old female with Epstein-Barr virus (EBV)-associated B-cell lymphoma of the brain and Evans syndrome is presented. After treatment of the lymphoma with irradiation to the brain and chemotherapy she developed Evans syndrome with autoimmune hemolytic anemia and thrombocytopenia. Further immunosuppressive treatment for Evans syndrome caused the dissemination of EBV-associated B-cell lymphoma. The dissemination of EBV-associated B-cell lymphoma was confirmed by in situ hybridization with EBV encoded small RNAs (EBER), polymerase chain reaction with Bam HI-W fragment of EBV and lymphocyte determined membrane antigen (LYDMA) and immunohistochemistry with latent membrane protein (LMP). Since only a few cases of lymphomas associated with EBV occurring during autoimmune diseases have been reported, this is an illustrative case.

Language of Publication

English

Unique Identifier

98256805

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Title

The spectrum of Evans' syndrome [see comments]

Author

Savasan S; Warrier I; Ravindranath Y

Address

Division of Hematology/Oncology, Children's Hospital of Michigan, Wayne State University, School of Medicine, Detroit 48201, USA.

Source

Arch Dis Child, 1997 Sep, 77:3, 245-8

Abstract

Eleven patients (10 boys, one girl) with Evans' syndrome with a median follow up time of 8.0 years were evaluated retrospectively. Six patients had either persistent hepatosplenomegaly or generalised lymphadenopathy, or both. In five patients, an increase in lymph node and/or spleen size was observed during the exacerbations of cytopenias. Seven patients had quantitative serum immunoglobulin abnormalities at the time of presentation. There were associated systemic manifestations in nine patients. Various forms of treatment were used with mixed results. Four patients died from sepsis and haemorrhage; four had complete recovery--two after splenectomy. These findings show that Evans' syndrome is a heterogeneous disorder with significant morbidity and mortality. High incidence of quantitative serum immunoglobulin abnormalities, lymphoid hyperplasia, and associated systemic manifestations suggest that Evans' syndrome may represent a stage of a more broad spectrum, generalised immune dysregulation.

Language of Publication

English

Unique Identifier

98038240

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Title

[Effective danazol therapy for a patient with Evans syndrome]

Author

Koike M; Ishiyama T; Saito K; Osawa H; Hashimoto M; Wakabayashi Y; Tsuruoka N

Address

Department of Internal Medicine, Showa University.

Source

Rinsho Ketsueki, 1993 Feb, 34:2, 143-6

Abstract

Danazol administered to a 66-year-old man with Evans syndrome, which was refractory to prednisolone therapy and pulse therapy with methylprednisolone. Danazol therapy produced an excellent and sustained improvement, increasing platelet counts and Hb concentrations. Recently, danazol therapy has been reported to be of benefit to severe idiopathic thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA). And this case marked a favorable effect also on Evans syndrome, which has both characters as ITP and as AIHA. The aim of this paper is to suggest that the administration of danazol has a positive effect on Evans syndrome and probably on other various thrombocytopenic diseases and syndromes, especially on refractory cases.

Language of Publication

Japanese

Unique Identifier

93260825

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Title

[Pulmonary nocardiosis with Evans' syndrome]

Author

Yokoo A; Enomoto H; Matsui Y; Naoe S

Address

Source

Nihon Kyobu Shikkan Gakkai Zasshi, 1973 May, 11:5, 292-5

Abstract

Abstract unavailable online.

Language of Publication

Japanese

Unique Identifier

73252533

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Title

[Evan's syndrome with antiphospholipid-protein antibodies]

Author

Fro…ow M; Jankowski M; Swadzba J; Musia… J

Address

II Katedry ChorĆob WewnŔetrznych Collegium Medicum Uniwersytetu JagielloĆnskiego.

Source

Pol Merkuriusz Lek, 1996 Nov, 1:5, 344-5

Abstract

This is a case report of a 34 years old man with Evans syndrome associated with antiphospholipid-protein antibodies. They include lupus anticoagulant and antibodies against cardiolipin, prothrombin and beta 2-glycoprotein I, detected by ELISA. No thrombotic events were observed. The presence of several antibodies directed against surface cell membrane structures in Evans syndrome suggests a common pathogenetic mechanism.

Language of Publication

Polish

Unique Identifier

97395314

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Title

[Problems in Evan's syndrome. Clinical and nuclear medicine studies]

Author

Heck J; Gehrmann G

Address

Source

Dtsch Med Wochenschr, 1973 Jun, 98:23, 1163-8

Abstract

Abstract unavailable online.

Language of Publication

German

Unique Identifier

73200274

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Title

[Increase of megakaryocytopoiesis in Werlhof's disease, Evan's syndrome and lupus erythematodes visceralis]

Author

Heck J; Gehrmann G

Address

Source

Verh Dtsch Ges Inn Med, 1975, 81:, 1106-8

Abstract

Abstract unavailable online.

Language of Publication

German

Unique Identifier

76227035

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Title

[Evans' syndrome]

Author

Chinen Y; Tsunematsu T

Address

Source

Nippon Rinsho, 1977 Spr, 35 Suppl 1:, 1298-9

Abstract

Abstract unavailable online.

Language of Publication

Japanese

Unique Identifier

78174491

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Title

[Evan's syndrome as a basic expression of a reticulosarcoma]

Author

Rodrígues Cuartero A; Pérez Gálvez MN; Peláez Redondo J

Address

Source

Rev Clin Esp, 1975 Apr, 137:2, 165-9

Abstract

Abstract unavailable online.

Language of Publication

Spanish

Unique Identifier

75197527

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Title

[Production and splenic storage of platelets in autoimmune thrombocytopenia (author's transl)]

Author

Gehrmann G; Heck J

Address

Source

Dtsch Med Wochenschr, 1975 Dec, 100:52, 2641-6

Abstract

Platelet kinectics (using 51Cr labelling) was measured in 66 tests on 59 patients with autoimmune thrombocytopenia (the majority with chronic Werlhof's disease plus cases of acute Werlhof's disease, Evans' syndrome and visceral lupus), measuring platelet survival time, platelet production, and splenic storage rate of platelets. Dependent on the severity of the disease, the elimination curve showed an abnormal course during the initial phase which - in comparison with the normal - indicated approximately a difference of the spleen-dependent part of the disappearance rate from the antibody-dependent destruction. Accordingly, platelet storage in the spleen was slightly supernormal in the studied diseases. On average, platelet destruction was increased to more than twice normal. In 36% of cases platelet production remained within normal range. On average, the least increase in platelet production occurred in the acute form of Werlhof's disease. Maximal storage capacity, which can be six times normal and above, was reached in only a few cases.

Language of Publication

German

Unique Identifier

76091595

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Title

Intravenous gamma globulin for thrombocytopenia in children with Evans syndrome.

Author

Nuss R; Wang W

Address

Source

Am J Pediatr Hematol Oncol, 1987 Sum, 9:2, 164-7

Abstract

We describe three patients with Evans syndrome (immune hemolytic anemia and immune thrombocytopenia) who were refractory to conventional therapy, including steroids and splenectomy in all of the patients, vincristine in two, and cyclophosphamide in one. The patients were then treated with modified intravenous gamma globulin 0.4 g/kg/day for 5 consecutive days. Two patients failed to respond, but the third had a clinical remission after gamma globulin therapy. Given the usual chronic and relapsing course of Evans syndrome and the poor response to conventional therapy, we recommend that high dose i.v. gamma globulin be considered an alternative therapy in the management of these patients.

Language of Publication

English

Unique Identifier

87239240

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Title

Hereditary spastic paraplegia and Evans's syndrome.

Author

Ahmed FE; Qureshi IM; Wooldridge MA; Pejaver RK

Address

Department of Paediatrics, North West Armed Forces Hospital, Tabuk, Saudi Arabia.

Source

Acta Paediatr, 1996 Jul, 85:7, 879-81

Abstract

Two brothers with hereditary spastic paraplegia and Evans's syndrome are recorded. Rapid deterioration of functional motor ability followed the development of Evans's syndrome.

Language of Publication

English

Unique Identifier

96416765

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Title

[Evans' syndrome caused by D-penicillamine in rheumatoid arthritis. Value of the corticoids-danazol combination]

Author

Masson C; Brégeon C; Ifrah N; Berton V; Housseau F; Rénier JC

Address

Service de Rhumatologie, CHRU Angers.

Source

Rev Rhum Mal Osteoartic, 1991 Jul, 58:7, 519-22

Abstract

The authors report the development of thrombocytopenia purpura in one patient with seropositive and erosive rheumatoid arthritis treated successfully for 11 months with D-penicillamine. Anti-platelet-bound antibodies were present, but also: anti-erythrocyte antibodies with hemolytic anemia (then defining Evans's syndrome): higher level of antinuclear antibodies; intermittent neutropenia. The responsibility of D-penicillamine is discussed, but thrombocytopenia purpura evolved for itself. Glucocorticoids alone, intravenous immunoglobulin, vincristine did not induced remission, which at least occurred under the association danazol-glucocorticoids, without toxicity, especially on the liver function.

Language of Publication

French

Unique Identifier

92022272

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Title

Treatment of refractory Evans syndrome with alternate-day cyclosporine and prednisone.

Author

Rackoff WR; Manno CS

Address

Section of Pediatric Hematology-Oncology, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis 46202-5225.

Source

Am J Pediatr Hematol Oncol, 1994 May, 16:2, 156-9

Abstract

PURPOSE: We report that the use of alternate-day cyclosporine and prednisone improved the clinical course of a 6-year-old child with severe Evans syndrome. Before the use of cyclosporine the child had experienced life-threatening episodes of hemolytic anemia despite the use of multiple therapeutic modalities. METHODS: Cyclosporine was given at a dose of 10 mg/kg/day divided into two doses on alternate days. RESULTS: The use of cyclosporine resulted in increased hemoglobin levels, increased platelet counts, and the reduction of the patient's prednisone dose from 2 mg/kg/day to as low as 1 mg/kg every other day. With this regimen, the patient had less severe hemolytic anemia, was less thrombocytopenic, and had fewer hospitalizations. No major toxic effects were associated with cyclosporine therapy. CONCLUSION: The regimen of alternate-day cyclosporine and prednisone may prove to be useful in the treatment of other patients with refractory Evans syndrome.

Language of Publication

English

Unique Identifier

94219724

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Title

Evans syndrome in childhood.

Author

Pui CH; Wilimas J; Wang W

Address

Source

J Pediatr, 1980 Nov, 97:5, 754-8

Abstract

The combination of idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia is rare in childhood. Among 164 instances of ITP and 15 instances of AHA, 11 patients were found to have this combination. Three were found to have systemic lupus erythematosus, one had aplastic anemia, and seven had Evans syndrome. Neutropenia, at times associated with bacterial infections, occurred in four of the latter patients. Unlike most cases of ITP or AHA in childhood, the clinical course of Evans syndrome is usually chronic and relapsing. Treatment including corticosteroids, splenectomy, and immunosuppressive agents has been generally unsatisfactory. In view of the frequent presence of antibodies directed at red blood cells, platelets, neutrophils, and lymphocytes, immunopancytopenia may be a better term for this condition.

Language of Publication

English

Unique Identifier

81048976

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Title

The Evans syndrome: characterization of the responsible autoantibodies.

Author

Pegels JG; Helmerhorst FM; van Leeuwen EF; van de Plas van Dalen C; Engelfriet CP; von dem Borne AE

Address

Source

Br J Haematol, 1982 Jul, 51:3, 445-50

Abstract

The immunofluorescence test on paraformaldehyde-fixed cells was used for the detection of antibodies bound to the platelets and granulocytes and present in the sera of 24 patients with Evans syndrome and a further 29 patients with both idiopathic thrombocytopenia (ITP) and idiopathic neutropenia (INP), but without autoimmune haemolytic anaemia (AIHA). The direct immunofluorescence test on platelets and/or on granulocytes was positive in all patients with a cytopenia, but the sera of only 17 patients with the Evans syndrome and 15 of the other patients contained platelet- or granulocyte-specific autoantibodies. From absorption and elution experiments, it appeared that the autoantibodies were directed against antigens specific for the various peripheral blood cells, i.e. erythrocytes, platelets and granulocytes and that they were not cross-reacting.

Language of Publication

English

Unique Identifier

82257179

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Title

Evans syndrome: results of a national survey.

Author

Mathew P; Chen G; Wang W

Address

Department of Pediatrics, Medical College of Ohio at Toledo, USA.

Source

J Pediatr Hematol Oncol, 1997 Sep, 19:5, 433-7

Abstract

PURPOSE: Our goal was to improve the management of Evans Syndrome, an uncommon and frequently refractory condition. We conducted a retrospective survey to assess the demography, presentation, clinical course, and treatment response of affected children. PATIENTS AND METHODS: Information was analyzed from a detailed questionnaire completed by pediatric hematologists mainly in the U.S. and Canada. We sought information regarding demographics, findings at presentation, approach to diagnosis, treatments used (with specific reference to splenectomy, corticosteroids, and intravenous immunoglobulin (IVIG)), course of the disease with emphasis on recurrences, and status at last follow-up. RESULTS: Forty-two patients (22 male, 20 female) were included in the study. The median age was 7.7 years (range 0.2-26.6 years). At presentation, thrombocytopenia (32 patients) and anemia (28) were common; neutropenia occurred in 10 and pancytopenia in 6. Patients received a median of 5 (range 0-12) modalities of treatment. Courses of IVIG and corticosteroids were given to almost all patients; responses were varied but the effects lasted as long as 2 years. Splenectomy was performed for 15 patients but the median duration of response was only 1 month. Other treatments included cyclosporine, vincristine, danazol, azathioprine, cyclophosphamide, and plasmapheresis. The course of the disease was characterized by recurrent thrombocytopenia, hemolytic anemia, and neutropenia. After a median follow-up of 3 years, 3 patients had died, 20 had active disease on treatment, 5 had persistent disease (not on treatment), and 14 had no evidence of disease. CONCLUSION: Evans syndrome is a chronic and recurrent condition which is often refractory to IVIG, corticosteroids, and splenectomy. Responses to other agents have been anecdotal and inconclusive. A prospective study involving these agents is needed to determine optimal therapeutic combinations.

Language of Publication

English

Unique Identifier

97470048

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Title

Nature of platelet antibody in Evans syndrome: a case report.

Author

Kakaiya RM; Sherman LA; Miller WV; Katz AJ

Address

Source

Ann Clin Lab Sci, 1981 Nov, 11:6, 511-5

Abstract

The association of autoimmune thrombocytopenia and autoimmune hemolytic anemia (Evans Syndrome) has been recognized previously. Recently the present authors investigated a patient with this disorder. Both IgG and C3d were found on the patient's red cells and the serum contained a polyspecific IgG red cell antibody demonstrable only by the antiglobulin test. IgG antiplatelet antibody was demonstrated on circulating platelets as well as in the serum from the patient by using 125I-staphylococcal Protein A (ISPA) assay. Absorption of serum with normal platelets resulted in removal of platelet antibody, but not the red cell antibody. Furthermore, eluate prepared from platelets sensitized with patient serum contained an IgG antibody directed specifically against platelets, since it did not cross react with red cells. Our data suggest the presence of two distinct antibodies, one directed against platelets and the other against red cells in our patient with Evans Syndrome. Both these antibodies are IgG in nature and belong to subclass other than IgG3 since staphylococcal Protein A (SPA) binds to all subclasses of human IgG except IgG3.

Language of Publication

English

Unique Identifier

82112019

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Title

[A case of Evans' syndrome in which CD4+CD45RA+ cells markedly decreased in its active phase]

Author

Iwase O; Ohosumi A; Fujieda H; Kuriyama Y; Kawanishi Y; Nagasu M; Yaguchi M; Aizawa S; Nakano M; Toyama K

Address

First Department of Internal Medicine, Tokyo Medical College.

Source

Rinsho Ketsueki, 1993 Jul, 34:7, 859-64

Abstract

A 38-year-old man was admitted to our hospital because of exertional dyspnea in February, 1990. The patient had already been diagnosed as having autoimmune hemolytic anemia (AIHA) in February, 1982 and treated with prednisolone (PSL) until January, 1988. The laboratory examination confirmed the relapse of AIHA (IgG-warm type) and additionally disclosed the marked decrease of CD4+CD45RA+/CD4+ ratio in peripheral blood lymphocytes. Thereupon, the patient was treated again with PSL and entered the remission in one month. Simultaneously, CD4+CD45RA+/CD4+ ratio also increased to the normal level. Afterwards, the remission had been maintained for about six months with a small dose of PSL. However, in September, 1990, the hemolysis relapsed with marked thrombocytopenia and decreased CD4+CD45RA+ ratio. Then the diagnosis was corrected to Evans' syndrome because PAIgG was highly elevated together with positive Coombs' test. Although danazol and azathioprine were administered in addition to PSL, the disease remained in active phase. Thus, splenectomy was carried out in March, 1992. Consequently, the patient entered the remission that has been maintained over six months. CD4+CD45RA+/CD4+ ratio was also normalized. These results suggest that CD4+CD45RA+ cells may play an important role in the pathogenesis of AIHA or Evans' syndrome.

Language of Publication

Japanese

Unique Identifier

93367914

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Title

[Kaposi's angiosarcoma associated with Evans syndrome (letter)]

Author

Thomas P; Rotteleur G; Gosset D; Desmons F; Jouet JP; Huart JJ; Bauters F; Goudemand J

Address

Source

Nouv Presse Med, 1980 May, 9:24, 1710

Abstract

Abstract unavailable online.

Language of Publication

French

Unique Identifier

81124241

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Title

[A case of Evans's syndrome in which thrombocytopenia and hemolysis was improved by Sairei-to]

Author

Horikoshi A; Shida M; Abe M; Hosokawa Y; Sawada S; Horie T

Address

Department of Internal Medicine, Nihon University Nerima Hikarigaoka Hospital.

Source

Rinsho Ketsueki, 1995 Oct, 36:10, 1237-9

Abstract

A 51-year-old male diagnosed as having Evans's syndrome in 1991 was treated with 25 mg of prednisolone, but his anemia and thrombocytopenia progressed. Thus, in November 1993, treatment was begun with Sairei-to, a Chinese herbal medicine consisting of several water-soluble plant extracts. Following administration of 9.0 g/day of Sairei-to granules along with prednisolone, the platelet count increased from 6.1 x 10(4)/microliters to 12.3 x 10(4)/microliters after one week, while hemoglobin levels rose from 9.5 g/dl to 12.0 g/dl after three weeks. The patient maintained a good physical condition after the prednisolone dose was reduced, although Coomb's test and PAIgG levels remained positive. Sairei-to-seems to be a promising therapeutic agent for steroid-resistant ITP and AIHA, and seems to have no side effects.

Language of Publication

Japanese

Unique Identifier

96075096

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Title

Evans syndrome: a report on 12 patients.

Author

Ng SC

Address

Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur.

Source

Clin Lab Haematol, 1992, 14:3, 189-93

Abstract

From 1981 to 1989, 12 patients of the University Hospital, Kuala Lumpur, were diagnosed to have Evans syndrome based on direct antiglobulin test (DAT) positive haemolytic anaemia and immune thrombocytopenia occurring either simultaneously (7 patients) or consecutively (5 patients). Their mean age at presentation was 24.8 years with a marked female preponderance. All 12 patients were given high dose steroid after diagnosis. Subsequently, other modalities including intravenous immunoglobulin (1 patient) and high dose methylprednisolone (1 patient) were given. Three patients died of intracranial haemorrhage during the first admission while 1 patient died of pulmonary embolism six months after diagnosis. Three patients had splenectomy because of thrombocytopenia. Six patients tested positive for antinuclear factor and antibodies to double stranded DNA and four of them died. Positive serology appeared to be associated with a poorer prognosis. Follow up observations indicate that patients who survive the acute attacks fare reasonably well.

Language of Publication

English

Unique Identifier

93083024

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Title

[Chronic lymphocytic leukemia diagnosed after corticosteroid therapy for Evans syndrome]

Author

Tomioka M; Shimizu H; Matsuzaki T; Yuasa K; Iriuchijima T; Mori M; Murakami H; Naruse T

Address

First Department of Internal Medicine, Gunma University School of Medicine.

Source

Rinsho Ketsueki, 1992 Jun, 33:6, 834-7

Abstract

A 59-year-old woman was admitted in February 1991, because of abdominal distension. On admission, she had splenomegaly, autoimmune hemolytic anemia, and idiopathic thrombocytopenic purpura. Evans syndrome had been diagnosed and daily prednisolone therapy had been performed. After this therapy, a rapid increase of lymphocytes was observed accompanied with contraction of spleen. The monoclonal proliferation of B-lymphocytes and rearrangement of JH and J kappa gene were detected and chronic lymphocytic leukemia was diagnosed. With reduction of prednisolone, the lymphocyte counts decreased and the size of the spleen returned to the previous state. It is suspected that the cause of rapid lymphocytosis in this case was due to the redistribution of lymphocytes from the spleen to the peripheral blood.

Language of Publication

Japanese

Unique Identifier

93060178

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Title

[Evans syndrome, pseudotumoral lymphoid hyperplasia and deficit of IgA-IgG2-IgG4 (letter)]

Author

Zenone T; Bastion Y; Leduc D; Salles G; Rieux C; Espinouse D; Coiffier B

Address

Source

Presse Med, 1995 Feb, 24:5, 283

Abstract

Abstract unavailable online.

Language of Publication

French

Unique Identifier

95207269

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Title

[Favorable response to vinblastine in an idiopathic case of Evans Syndrome]

Author

Guerrero Becerra MP; Kessler Saiz P; Ortiz Conde MC; Fernández Miranda C

Address

Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid.

Source

An Med Interna, 1994 Jul, 11:7, 341-4

Abstract

Evan's Syndrome (ES) may develop in isolation or associated to other autoimmune diseases, solid tumors and lymphoproliferative syndromes. This type of processes can be refractory to the usual medication, that is, to corticoids and splenectomy. High doses of Immunoglobulins (polyvalent IgG) and immunosuppressive drugs are therapeutical alternatives that have been used with different results. We present a case of idiopathic ES refractory to high doses of corticoids and IgG, showing complete remission after the administration of two infusions of vinblastin. We believe that vinblastin is a very useful therapeutical alternative in the cases of ES refractory to the usual treatment.

Language of Publication

Spanish

Unique Identifier

95072193

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Title

Evans syndrome after autologous bone marrow transplant for recurrent Hodgkin's disease.

Author

Keung YK; Cobos E; Bolanos Meade J; Issarachai S; Brideau A; Morgan D

Address

Department of Internal Medicine, Texas Tech University Health Sciences Center and Southwest Cancer Center, Lubbock 79430, USA.

Source

Bone Marrow Transplant, 1997 Dec, 20:12, 1099-101

Abstract

The association of immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) with Hodgkin's disease has been known for many years. Autoimmune cytopenia has also been described in patients that have undergone allogeneic or autologous bone marrow transplantation. We report a rare case of Evans syndrome in a patient 3 years after autologous bone marrow transplantation for recurrent Hodgkin's disease.

Language of Publication

English

Unique Identifier

98125761

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Title

Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria.

Author

Lippman SM; Winn L; Grumet FC; Levitt LJ

Address

Source

Am J Med, 1987 May, 82:5, 1065-72

Abstract

Paroxysmal cold hemoglobinuria is a rare and potentially life-threatening acquired hemolytic anemia occurring either as an acute transient anemia following several different viral syndromes, or in a chronic idiopathic form. Episodic hemolysis in paroxysmal cold hemoglobinuria is usually associated with a biphasic (Donath-Landsteiner) IgG cold-reactive complement-fixing autohemolysin with anti-P specificity. Paroxysmal cold hemoglobinuria has not previously been associated with malignancy nor has it been clearly shown to be steroid-responsive. This report describes a patient with steroid-responsive autoimmune hemolytic anemia and immune thrombocytopenia (Evans' syndrome) associated with oat cell carcinoma of the lung and a unique biphasic anti-IgM autohemolysin. This case extends the spectrum of biphasic antibody-mediated immune cytopenias and widens both the clinical and the serologic definition of paroxysmal cold hemoglobinuria.

Language of Publication

English

Unique Identifier

87210269

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Title

Immunological studies in a case of Evans' syndrome.

Author

Mönch H; Breithaupt H; Mueller Eckhardt C

Address

Source

Blut, 1981 Jan, 42:1, 27-32

Abstract

Immunological studies in a case of Evans' syndrome (autoimmune hemolytic anemia of the "warm type" associated with idiopathic thrombocytopenic purpura) are presented in whom the autoimmune nature of both conditions was verified by the demonstration of cell-bound and free serum autoantibodies against red blood cells as well as platelets by a radioimmune technique.

Language of Publication

English

Unique Identifier

81161576

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Title

Evans' syndrome associated with microcystic adenoma of the pancreas.

Author

Doll DC; List AF; Yarbro JW

Address

Source

Cancer, 1987 Apr, 59:7, 1366-8

Abstract

A case of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura (Evans' syndrome), associated with a microcystic adenoma of the pancreas is reported. Early recognition of this rare entity is important, as corticosteroids and splenectomy are usually ineffective, whereas removal of the tumor may be a cure for this condition. Possible mechanisms of the hemolytic anemia and thrombocytopenia are also discussed.

Language of Publication

English

Unique Identifier

87130604

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Title

Evans' syndrome in IgA deficiency. Episodic autoimmune haemolytic anaemia and thrombocytopenia during a 10 years observation period.

Author

Hansen OP; S‡rensen CH; Astrup L

Address

Source

Scand J Haematol, 1982 Sep, 29:3, 265-70

Abstract

A 26-year-old male with a 10-year history of complete selective IgA deficiency and recurrent autoimmune anaemia and thrombocytopenia (Evans syndrome) is presented. Both serum IgA and saliva secretory IgA were below the detection limit (less than 0.05 mg/l). No other features of autoimmunity were seen. The patient had a normal % of peripheral blood lymphocytes with surface IgM and IgG cells and normal in vitro lymphocyte transformation after stimulation with mitogens and antigens. The pleomorphic and randomly appearing immunologic features of selective IgA deficiency are emphasized by the present case.

Language of Publication

English

Unique Identifier

83067182

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Title

Unusual varicella zoster virus infection in a patient with colon carcinoma and Evans syndrome--delayed virus shedding generalized recurrent necrotic herpes zoster.

Author

Hata S; Tamaki T

Evans' syndrome in IgA deficiency. Episodic autoimmune haemolytic anaemia and thrombocytopenia during a 10 years observation period.

Author

Hansen OP; S‡rensen CH; Astrup L

Address

Source

Scand J Haematol, 1982 Sep, 29:3, 265-70

Abstract

A 26-year-old male with a 10-year history of complete selective IgA deficiency and recurrent autoimmune anaemia and thrombocytopenia (Evans syndrome) is presented. Both serum IgA and saliva secretory IgA were below the detection limit (less than 0.05 mg/l). No other features of autoimmunity were seen. The patient had a normal % of peripheral blood lymphocytes with surface IgM and IgG cells and normal in vitro lymphocyte transformation after stimulation with mitogens and antigens. The pleomorphic and randomly appearing immunologic features of selective IgA deficiency are emphasized by the present case.

Language of Publication

English

Unique Identifier

83067182

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Title

Unusual varicella zoster virus infection in a patient with colon carcinoma and Evans syndrome--delayed virus shedding generalized recurrent necrotic herpes zoster.

Author

Hata S; Tamaki T

Address

Department of Dermatology, Osaka Rosai Hospital, Japan.

Source

J Dermatol, 1990 May, 17:5, 326-8

Abstract

A 53-year-old Japanese woman with Evans syndrome and colon cancer had two episodes