Clinical characteristics of hemophilia A patients with hemarthrosis

Setyo Handryastuti; Djajadiman Gatot; Arwin A. P. Akib

doi:10.14238/pi42.5-6.2002.101-5

Setyo Handryastuti Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Djajadiman Gatot Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Arwin A. P. Akib Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta

DOI: https://doi.org/10.14238/pi42.5-6.2002.101-5

Keywords: hemophilia A, hemarthrosis, clinical characteristics

Abstract

Background Hemarthrosis is the most frequent bleeding manifestation of severe hemophilia. Repeated hemarthrosis will cause chronic arthropathy, which results in a physical disability.

Objectives To obtain data of clinical characteristics of hemophilia- A patients with hemarthrosis (particularly chronic hemarthrosis) and to know the effect of on-demand therapy on joints of the patients.
Methods We evaluated 102 hemophilia A patients in Pediatric Hematology and Oncology Division, Cipto Mangunkusumo Hospital for 6 months beginning from March 2001.

Results The number of cases of chronic hemarthrosis was 22%,smaller than previous study (54%). Chronic hemarthrosis mostly occurred in hemophilia A patients who aged between 13-18 years, had severe hemophilia A and frequency of hemarthrosis more than 12 times a year, and also patients who did not receive adequate therapy. The joint which most frequently suffered from hemarthrosis were knee (26%), ankle (23%) and elbow (21%). The critical period for the first hemarthrosis was at the age of 2-12 years, and repeatedÂ hemarthrosis episodes commonly occurred at the age 6-18 years
owing to the child's increasing physical activities.

Conclusion Hemarthrosis can be prevented and anticipated. It is important to notice the critical period when first hemarthrosis
and repeated hemarthrosis occur. The certain joints like knee, ankle and elbow must be given more attention due to the risk of
repeated hemarthrosis.

References

1. Robert HR, Jones MR. Hemophilia and related conditions congenital deficiencies of prothrombin (factor II) , factor V, and factors VII to XII. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA, editors. Hematology. 4th ed. New York: McGraw-Hill; 199l.p.l453-72.
2. Carvell JE, Duthie RB. Management of the hemophilic knee. In: lnsall JN, editor. Surgery of the knee. 1st ed. New York: Churchill Livingstone; 1984. p. 743-74.
3. Goldsmith J C, Phillips MD. A guide to bleeding disorders for case managers understanding treatment for hemophilia & von Willebrand disease. Available from: URL: http://www.cmrg.com/dmhemophilia.htm.
4. Setiabudi R, Gatot D. Ekspresi fenotipik gen hemofilia pada penderita hemofilia di RS Dr. Cipto Mangunkusumo. Laporan akhir penelitian riset pembinaan lptek kedokteran. Jakarta, 2000.
5. Lusher JM. Considerations for current and future management of haemophilia and its complications. Haemophilia 199 5; 1: 2-lO.
6. Bern top E. Methods ofhaemophilia care delivery: regular prophylaxis versus episodic treatment. Haemophilia 1995;1(Suppll):3-7.
7. Bemtop E. The treatment of haemophilia, including prophylaxis, constant infusion and DDAVP. Baillieres Clin Haematol 1996;9:259-71.
8. Gruppo RA. Prophylaxis for hemophilia: State of the art or state of confusion? J Pediatr 1998; 132:915-7.
9. Montgomerry RR, Scot JP. Hemostasis: disease of the fluid phase. In: Nathan DG, Oski FA, editors. Hematology of infancy and childhood. 4th ed. Philadelphia: WB Saunders; 1993. p. 1605-50.
10. Corrigan JJ. Hemorrhagic disorder. In: Behrman RE, Kliegman RM, Nelson WE, Vaughan VC, editors. Nelson textbook of pediatrics. 15th ed. Philadelphia: WB Saunders; 1996. p. 1424-7.
11. Markum AH. Tumbuh kembang. In: Buku ajar ilmu kesehatan anak jilid 1. Jakarta: Fakultas Kedokteran Universitas Indonesia; 1991. p. 25-29.
12. Pilgerstorfer Hw, Merxner MA. Hemophilic arthropathy: Evaluation of a system of classifYing radiological signs.ln: Ala F, Denson KWE, editors. Congress of the World Federation of Haemophilia. Vienna: ExcerptaMedica; 1973. p. 184-90.
13. Kreuz W Prevention of joint damage in hemophilic children with early prophylaxis. Orthopade 1999;28:341-6.
14. Moeslichan S. Mengenal masalah hemofilia di Indonesia. Seminar Hemofilia; 2001, April17th;Jakarta.
15. Roy S, Chadially FN. Sinovial membrane in experimentally produced chronic hemarthrosis. Ann Rheum Dis 1969;28:402-7.
16. Roosendal G, Mauser-Bunschoten EP, De Kleijn P, Heijnen L, Van Rinsum AC, VD Berg HM, et al. Synovium in haemophilic arthropathy. Haemophilia 1998;4:502-5.