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. 1996 Apr;74(4):319–322. doi: 10.1136/adc.74.4.319

Severe aplastic anaemia in the Nordic countries: a population based study of incidence, presentation, course, and outcome.

N Clausen 1, A Kreuger 1, T Salmi 1, I Storm-Mathisen 1, G Johannesson 1
PMCID: PMC1511473  PMID: 8669932

Abstract

PURPOSE: Incidence data for severe aplastic anaemia (SAA) in children are scanty and vary. Few population based studies have been reported. A retrospective and prospective study was conducted to determine the incidence and course of SAA. PATIENTS AND METHODS: All children with a diagnosis of SAA in the Nordic countries from 1982 through 1993 were registered and have been followed up since 1987. RESULTS: A total of 101 children were diagnosed with SAA. The mean annual child population was 4.31 million. A constant incidence of 1.95/million children/year was found: 2.4 for boys and 1.5 for girls. A non-significant increase of cases occurred from November to March. Possible aetiological agents were noted in 29%. The actuarial survival was 79% after one year and 68% after five years without significant difference between boys and girls. CONCLUSION: The incidence of SAA in the Nordic countries remains stable with a preponderance among boys. SAA has still a high initial mortality and a risk of late deaths.

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Selected References

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

  1. Baumelou E., Guiguet M., Mary J. Y. Epidemiology of aplastic anemia in France: a case-control study. I. Medical history and medication use. The French Cooperative Group for Epidemiological Study of Aplastic Anemia. Blood. 1993 Mar 15;81(6):1471–1478. [PubMed] [Google Scholar]
  2. Bayever E., Champlin R., Ho W., Lenarsky C., Storch S., Ladisch S., Gale R. P., Feig S. A. Comparison between bone marrow transplantation and antithymocyte globulin in treatment of young patients with severe aplastic anemia. J Pediatr. 1984 Dec;105(6):920–925. doi: 10.1016/s0022-3476(84)80078-5. [DOI] [PubMed] [Google Scholar]
  3. Böttiger L. E., Westerholm B. Aplastic anaemia. I. Incidence and aetiology. Acta Med Scand. 1972 Oct;192(4):315–318. [PubMed] [Google Scholar]
  4. Camitta B. M., Doney K. Immunosuppressive therapy for aplastic anemia: indications, agents, mechanisms, and results. Am J Pediatr Hematol Oncol. 1990 Winter;12(4):411–424. doi: 10.1097/00043426-199024000-00005. [DOI] [PubMed] [Google Scholar]
  5. Camitta B. M., Thomas E. D., Nathan D. G., Gale R. P., Kopecky K. J., Rappeport J. M., Santos G., Gordon-Smith E. C., Storb R. A prospective study of androgens and bone marrow transplantation for treatment of severe aplastic anemia. Blood. 1979 Mar;53(3):504–514. [PubMed] [Google Scholar]
  6. Cartwright R. A., McKinney P. A., Williams L., Miller J. G., Evans D. I., Bentley D. P., Bhavnani M. Aplastic anaemia incidence in parts of the United Kingdom in 1985. Leuk Res. 1988;12(6):459–463. doi: 10.1016/0145-2126(88)90111-7. [DOI] [PubMed] [Google Scholar]
  7. Clausen N. A population study of severe aplastic anemia in children. Incidence, etiology and course. Acta Paediatr Scand. 1986 Jan;75(1):58–63. doi: 10.1111/j.1651-2227.1986.tb10157.x. [DOI] [PubMed] [Google Scholar]
  8. Issaragrisil S., Sriratanasatavorn C., Piankijagum A., Vannasaeng S., Porapakkham Y., Leaverton P. E., Kaufman D. W., Anderson T. E., Shapiro S., Young N. S. Incidence of aplastic anemia in Bangkok. The Aplastic Anemia Study Group. Blood. 1991 May 15;77(10):2166–2168. [PubMed] [Google Scholar]
  9. Khatib Z., Wilimas J., Wang W. Outcome of moderate aplastic anemia in children. Am J Pediatr Hematol Oncol. 1994 Feb;16(1):80–85. [PubMed] [Google Scholar]
  10. Malkin D., Koren G., Saunders E. F. Drug-induced aplastic anemia: pathogenesis and clinical aspects. Am J Pediatr Hematol Oncol. 1990 Winter;12(4):402–410. doi: 10.1097/00043426-199024000-00004. [DOI] [PubMed] [Google Scholar]
  11. Mary J. Y., Baumelou E., Guiguet M. Epidemiology of aplastic anemia in France: a prospective multicentric study. The French Cooperative Group for Epidemiological Study of Aplastic Anemia. Blood. 1990 Apr 15;75(8):1646–1653. [PubMed] [Google Scholar]
  12. Matloub Y. H., Brunning R. D., Arthur D. C., Ramsay N. K. Severe aplastic anemia preceding acute lymphoblastic leukemia. Cancer. 1993 Jan 1;71(1):264–268. doi: 10.1002/1097-0142(19930101)71:1<264::aid-cncr2820710140>3.0.co;2-8. [DOI] [PubMed] [Google Scholar]
  13. Nissen C., Gratwohl A., Tichelli A., Stebler C., Würsch A., Moser Y., Dalle Carbonare V., Signer E., Buser M., Ritz R. Gender and response to antilymphocyte globulin (ALG) for severe aplastic anaemia. Br J Haematol. 1993 Feb;83(2):319–325. doi: 10.1111/j.1365-2141.1993.tb08288.x. [DOI] [PubMed] [Google Scholar]
  14. Seip M., Vidnes J. Cyclosporine A in a case of refractory severe aplastic anaemia. Scand J Haematol. 1985 Mar;34(3):228–230. doi: 10.1111/j.1600-0609.1985.tb02782.x. [DOI] [PubMed] [Google Scholar]
  15. Szklo M., Sensenbrenner L., Markowitz J., Weida S., Warm S., Linet M. Incidence of aplastic anemia in metropolitan Baltimore: a population-based study. Blood. 1985 Jul;66(1):115–119. [PubMed] [Google Scholar]

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