Bleeding Patterns among Severe Hemophilia A and B Patients in West Java

Muhammad Mufakkirul Islam, Susi Susanah, Amaylia Oehadian


Background: The clinical manifestations of hemophilia A (HA) and hemophilia B (HB)are quite similar; however, the bleeding characteristics of these two hemophilia types have been reported to be different. This study aimed to explore the bleeding patterns among patients with severe HA and severe HB.

Methods: A cross-sectional study was conducted among patients with severe HA and HB registered at the West Java Indonesian Hemophilia Society. The inclusion criteria were patients with severe hemophilia diagnosed for at least one year. The bleeding patterns included bleeding episodes and bleeding types. The Mann-Whitney test was used to compare bleeding episodes and a chi-square test for bleeding types.

Results: In total, 158 severe HA patients and 21 severe HB patients were included with a median bleeding frequency per patient per year for HA and HB was 24 (range 0–48) and 24 (range 5–48), respectively. The bleeding types in HA and HB were ecchymosis (69% vs. 66.7%), hematoma (62.7% vs. 61.9%), hemarthrosis (99.4% vs. 100%), epistaxis (46.8% vs. 38.1%), gum bleeding (87.3% vs. 95.2%), intracranial hemorrhage (15.2% vs. 9.5%), multiple hematomas (36.7% vs. 47.6%), hemarthrosis-hematoma (61.4% vs.61.9%), and hemarthrosis-ecchymosis (69% vs. 61.9%). However, there was no significant difference in all types of bleeding between HA and HB.

Conclusions: There is no difference in the pattern of hemorrhage between severe HA and severe HB in West Java. However, the bleeding phenotypes in hemophilia has considerable implications in the therapeutic process. Further research is needed to optimize the treatment regimens.


Bleeding episodes, bleeding types, hemophilia A, hemophilia B

Full Text:



Srivastava A, Brewer AK, Mauser-Bunschiten EP, Key NS, Kitchen A, Llinas A, et al. Guidelines for the management of hemophilia. Haemophilia. 2013;19(1):e1–47.

Rambe IK, Sungkar E, Moeliono MA, Astini S. Prediction of hemophilia joint health score based on age and disease severity of hemophilia A and B with on demand therapy in West Java. Malays J Paediatr Child Health. 2018;24(2):1–10.

Berntorp E, Shapiro AD. Modern haemophilia care. Lancet. 2012;379(9824):1447–56.

Potgieter JJ, Damgaard M, Hillarp A. One-stage vs. chromogenic assays in haemophilia A. Eur J Haematol. 2015;94(Suppl 77):38–44.

Gale AJ. Continuing education course #2: current understanding of hemostasis. Toxicol Pathol. 2011;39(1):273–80.

Versteeg HH, Heemskerk JWM, Levi M, Reitsma PH. New Fundamentals in Hemostasis. Physiol Rev. 2013;93(1):327–58.

Periayah MH, Halim AS, Saad AZM. Mechanism action of platelets and crucial blood coagulation pathways in hemostasis. Int J Hematol Oncol Stem Cell Res. 2017;11(4):319–27.

Fijnvandraat K, Cnossen MH, Leebeek FWG, Peters M. Diagnosis and management of haemophilia. BMJ. 2012;344:e2707

Santagostino E, Fasulo MR. Hemophilia A and hemophilia B: Different types of diseases? Semin Thromb Hemost. 2013;39(7):697–701.

Mannucci PM, Franchini M. Is haemophilia B less severe than haemophilia A? Haemophilia. 2013;19(4):499–502.

Nagel K, Walker I, Decker K, Chan AKC, Pai MK. Comparing bleed frequency and factor concentrate use between haemophilia A and B patients. Haemophilia. 2011;17(6):872–4.

World Federation of Hemophilia. Annual Global Survery 2017. Canada: World Federation of Hemophilia; 2018.

Biere-Rafi S, Haak BW, Gerdes VEA, Büller HR, Kamphuisen PW. The impairment in daily life of obese haemophiliacs. Haemophilia. 2011;17(2):204–8.

Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388(10040):187–97.

Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison´s principles of internal medicine. 18th Ed. New York: McGraw-Hill Professional; 2012.

Escobar M, Sallah S. Hemophilia A and hemophilia B: Focus on arthropathy and variables affecting bleeding severity and prophylaxis. J Thromb Haemost. 2013;11(8):1449–53.


Article Metrics

Abstract view : 702 times
PDF - 387 times

 This Journal indexed by




Creative Commons License
AMJ is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License


View My Stats