Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1997 Sep;77(3):245–248. doi: 10.1136/adc.77.3.245

The spectrum of Evans' syndrome

S Savasan 1, I Warrier 1, Y Ravindranath 1
PMCID: PMC1717303  PMID: 9370906

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.



Full Text

The Full Text of this article is available as a PDF (74.6 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ashby G. H., Evans D. I. Cartilage hair hypoplasia with thrombocytopenic purpura, autoimmune haemolytic anaemia and cell-mediated immunodeficiency. J R Soc Med. 1986 Feb;79(2):113–114. doi: 10.1177/014107688607900216. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Cuesta B., Fernández J., Pardo J., Páramo J. A., Gómez C., Rocha E. Evan's syndrome, chronic active hepatitis and focal glomerulonephritis in IgA deficiency. Acta Haematol. 1986;75(1):1–5. doi: 10.1159/000206070. [DOI] [PubMed] [Google Scholar]
  3. Curtin J. J., Murray J. G., Apthorp L. A., Franz A. M., Webster A. D. Mediastinal lymph node enlargement and splenomegaly in primary hypogammaglobulinaemia. Clin Radiol. 1995 Jul;50(7):489–491. doi: 10.1016/s0009-9260(05)83167-8. [DOI] [PubMed] [Google Scholar]
  4. EVANS R. S., TAKAHASHI K., DUANE R. T., PAYNE R., LIU C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951 Jan;87(1):48–65. doi: 10.1001/archinte.1951.03810010058005. [DOI] [PubMed] [Google Scholar]
  5. Español T., Catala M., Hernandez M., Caragol I., Bertran J. M. Development of a common variable immunodeficiency in IgA-deficient patients. Clin Immunol Immunopathol. 1996 Sep;80(3 Pt 1):333–335. doi: 10.1006/clin.1996.0132. [DOI] [PubMed] [Google Scholar]
  6. Fisher G. H., Rosenberg F. J., Straus S. E., Dale J. K., Middleton L. A., Lin A. Y., Strober W., Lenardo M. J., Puck J. M. Dominant interfering Fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome. Cell. 1995 Jun 16;81(6):935–946. doi: 10.1016/0092-8674(95)90013-6. [DOI] [PubMed] [Google Scholar]
  7. Guo B. C., Saxon A. B cell lines from a subset of patients with common variable immunodeficiency undergo enhanced apoptosis associated with an increased display of CD95 (Apo-1/fas), diminished CD38 expression, and decreased IgG and IgA production. Cell Immunol. 1995 Nov;166(1):83–92. doi: 10.1006/cimm.1995.0010. [DOI] [PubMed] [Google Scholar]
  8. Habibi B., Homberg J. C., Schaison G., Salmon C. Autoimmune hemolytic anemia in children. A review of 80 cases. Am J Med. 1974 Jan;56(1):61–69. doi: 10.1016/0002-9343(74)90751-7. [DOI] [PubMed] [Google Scholar]
  9. Hansen O. P., Sørensen C. H., Astrup L. Evans' syndrome in IgA deficiency. Episodic autoimmune haemolytic anaemia and thrombocytopenia during a 10 years observation period. Scand J Haematol. 1982 Sep;29(3):265–270. doi: 10.1111/j.1600-0609.1982.tb00592.x. [DOI] [PubMed] [Google Scholar]
  10. Le Deist F., Emile J. F., Rieux-Laucat F., Benkerrou M., Roberts I., Brousse N., Fischer A. Clinical, immunological, and pathological consequences of Fas-deficient conditions. Lancet. 1996 Sep 14;348(9029):719–723. doi: 10.1016/S0140-6736(96)02293-3. [DOI] [PubMed] [Google Scholar]
  11. Miescher P. A., Tucci A., Beris P., Favre H. Autoimmune hemolytic anemia and/or thrombocytopenia associated with lupus parameters. Semin Hematol. 1992 Jan;29(1):13–17. [PubMed] [Google Scholar]
  12. Miller B. A., Schultz Beardsley D. Autoimmune pancytopenia of childhood associated with multisystem disease manifestations. J Pediatr. 1983 Dec;103(6):877–881. doi: 10.1016/s0022-3476(83)80705-7. [DOI] [PubMed] [Google Scholar]
  13. Mountz J. D., Zhou T., Su X., Wu J., Cheng J. The role of programmed cell death as an emerging new concept for the pathogenesis of autoimmune diseases. Clin Immunol Immunopathol. 1996 Sep;80(3 Pt 2):S2–14. doi: 10.1006/clin.1996.0136. [DOI] [PubMed] [Google Scholar]
  14. Nuss R., Wang W. Intravenous gamma globulin for thrombocytopenia in children with Evans syndrome. Am J Pediatr Hematol Oncol. 1987 Summer;9(2):164–167. doi: 10.1097/00043426-198722000-00012. [DOI] [PubMed] [Google Scholar]
  15. Oda H., Honda A., Sugita K., Nakamura A., Nakajima H. High-dose intravenous intact IgG infusion in refractory autoimmune hemolytic anemia (Evans syndrome). J Pediatr. 1985 Nov;107(5):744–746. doi: 10.1016/s0022-3476(85)80405-4. [DOI] [PubMed] [Google Scholar]
  16. Pegels J. G., Helmerhorst F. M., van Leeuwen E. F., van de Plas-van Dalen C., Engelfriet C. P., von dem Borne A. E. The Evans syndrome: characterization of the responsible autoantibodies. Br J Haematol. 1982 Jul;51(3):445–450. doi: 10.1111/j.1365-2141.1982.tb02801.x. [DOI] [PubMed] [Google Scholar]
  17. Petrides P. E., Hiller E. Autoimmune hemolytic anemia combined with idiopathic thrombocytopenia (Evans syndrome). Sustained remission in a patient following high-dose intravenous gamma-globulin therapy. Clin Investig. 1992 Jan;70(1):38–39. doi: 10.1007/BF00422936. [DOI] [PubMed] [Google Scholar]
  18. Pui C. H., Wilimas J., Wang W. Evans syndrome in childhood. J Pediatr. 1980 Nov;97(5):754–758. doi: 10.1016/s0022-3476(80)80258-7. [DOI] [PubMed] [Google Scholar]
  19. Rackoff W. R., Manno C. S. Treatment of refractory Evans syndrome with alternate-day cyclosporine and prednisone. Am J Pediatr Hematol Oncol. 1994 May;16(2):156–159. [PubMed] [Google Scholar]
  20. Rieux-Laucat F., Le Deist F., Hivroz C., Roberts I. A., Debatin K. M., Fischer A., de Villartay J. P. Mutations in Fas associated with human lymphoproliferative syndrome and autoimmunity. Science. 1995 Jun 2;268(5215):1347–1349. doi: 10.1126/science.7539157. [DOI] [PubMed] [Google Scholar]
  21. Sanal O., Ersoy F., Metin A., Tezcan I., Berkel A. I., Yel L. Selective IgA deficiency with unusual features: development of common variable immunodeficiency, Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura. Acta Paediatr Jpn. 1995 Aug;37(4):526–529. doi: 10.1111/j.1442-200x.1995.tb03369.x. [DOI] [PubMed] [Google Scholar]
  22. Sander C. A., Medeiros L. J., Weiss L. M., Yano T., Sneller M. C., Jaffe E. S. Lymphoproliferative lesions in patients with common variable immunodeficiency syndrome. Am J Surg Pathol. 1992 Dec;16(12):1170–1182. doi: 10.1097/00000478-199212000-00004. [DOI] [PubMed] [Google Scholar]
  23. Saxon A., Keld B., Diaz-Sanchez D., Guo B. C., Sidell N. B cells from a distinct subset of patients with common variable immunodeficiency (CVID) have increased CD95 (Apo-1/fas), diminished CD38 expression, and undergo enhanced apoptosis. Clin Exp Immunol. 1995 Oct;102(1):17–25. doi: 10.1111/j.1365-2249.1995.tb06630.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Scaradavou A., Bussel J. Evans syndrome. Results of a pilot study utilizing a multiagent treatment protocol. J Pediatr Hematol Oncol. 1995 Nov;17(4):290–295. [PubMed] [Google Scholar]
  25. Simons S. M., Main C. A., Yaish H. M., Rutzky J. Idiopathic thrombocytopenic purpura in children. J Pediatr. 1975 Jul;87(1):16–22. doi: 10.1016/s0022-3476(75)80061-8. [DOI] [PubMed] [Google Scholar]
  26. Sneller M. C., Straus S. E., Jaffe E. S., Jaffe J. S., Fleisher T. A., Stetler-Stevenson M., Strober W. A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease. J Clin Invest. 1992 Aug;90(2):334–341. doi: 10.1172/JCI115867. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Sneller M. C., Strober W., Eisenstein E., Jaffe J. S., Cunningham-Rundles C. NIH conference. New insights into common variable immunodeficiency. Ann Intern Med. 1993 May 1;118(9):720–730. doi: 10.7326/0003-4819-118-9-199305010-00011. [DOI] [PubMed] [Google Scholar]
  28. Villiger P. M., Fey M. F., Lotz M., Tobler A. IgG2 and IgG4 subclass deficiency and Evan's syndrome in an adult patient. J Allergy Clin Immunol. 1992 Oct;90(4 Pt 1):693–694. doi: 10.1016/0091-6749(92)90147-t. [DOI] [PubMed] [Google Scholar]
  29. Wang W. C. Evans syndrome in childhood: pathophysiology, clinical course, and treatment. Am J Pediatr Hematol Oncol. 1988 Winter;10(4):330–338. doi: 10.1097/00043426-198824000-00013. [DOI] [PubMed] [Google Scholar]
  30. Wang W., Herrod H., Pui C. H., Presbury G., Wilimas J. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol. 1983 Dec;15(4):381–390. doi: 10.1002/ajh.2830150409. [DOI] [PubMed] [Google Scholar]
  31. Watanabe-Fukunaga R., Brannan C. I., Copeland N. G., Jenkins N. A., Nagata S. Lymphoproliferation disorder in mice explained by defects in Fas antigen that mediates apoptosis. Nature. 1992 Mar 26;356(6367):314–317. doi: 10.1038/356314a0. [DOI] [PubMed] [Google Scholar]
  32. Wu J., Wilson J., He J., Xiang L., Schur P. H., Mountz J. D. Fas ligand mutation in a patient with systemic lupus erythematosus and lymphoproliferative disease. J Clin Invest. 1996 Sep 1;98(5):1107–1113. doi: 10.1172/JCI118892. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Zuelzer W. W., Mastrangelo R., Stulberg C. S., Poulik M. D., Page R. H., Thompson R. I. Autoimmune hemolytic anemia. Natural history and viral-immunologic interactions in childhood. Am J Med. 1970 Jul;49(1):80–93. doi: 10.1016/s0002-9343(70)80116-4. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES