Clinicohematological Study of Hemophilia Patients in Bhopal

Abstract

The X-linked inherited coagulation disorders, hemophilia A (Factor VIII deficiency) and hemophilia B (factor IX deficiency), together with Von Willebrand disease comprise 95 to 97 percent of all the Inherited deficiencies of coagulation factors. (1,2) Replacement of the deficient factor is the mainstay of treatment; it may be "on demand" or "prophylactic" to prevent hemarthrosis in severe deficiency. The prevalence of hemophilia A is 1 in 5000 and that of hemophilia B is 1 in 30,000 (3,4) male live births and Von Willebrand disease with over all prevalence in the general population being 1:100. (4) Clinically both the deficiencies present as a lifelong bleeding disorder with considerable morbidity due to crippling arthropathy. Most common cause of death in these patients is Intra Cerebral Hemorrhage. Hemophilia A and B can only be distinguished on the basis of specific coagulation Factor VIII or IX assays. AIM: The present study was conducted with the aim to study the clinico-hematological profile of Persons With Hemophilia(PWH), their clinical presentations, incidence of inhibitors, estimating the burden of transfusion related complications at our hemophilia care center and to compare the findings with other studies of similar nature MATERIAL & METHOD: This retrospective study was conducted in the Department of Pathology in collaboration with Hemophilia Care Centre at Gandhi Medical College, Bhopal during the period of August 2003 to July 2013. Diagnosis was made on the basis of history, physical examination and laboratory investigations such as bleeding time (BT), Prothrombin time (PT), Activated Partial Thromboplastin Time (APTT),correction studies(factor VIII and factor IX estimation whenever possible). CONCLUSION: Bleeding after injury is obvious in healthy people but difficult to decide, when it is due to bleeding disorder. Serious congenital conditions e. g. severe hemophilia becomes obvious in early childhood but may be misdiagnosed as non-accidental injury, whereas mild cases may go undetected till later years especially those who have not undergone surgery in early adult life. Presence of hemarthrosis, bruises, hematoma either spontaneous or traumatic in an otherwise normal child should warrants for investigating in the line of hemophilia. Repeated episodes early in life show severe hemophilia. Most common cause of death was found Intra Cerebral Hemorrhage (ICH).

Clinico-Haematological Profile of Haemophilia in Patients Attending Gandhi Medical College and Associated Hamidia Hospital, Bhopal

“…20,21 In present study, in 52% patients bleeding starts spontaneously while in 48% patients bleeding starts following major or minor trauma. Similar results are seen in study done by R K Nigam 14 et al (Spontaneous bleed -57%, traumatic bleed-33%). While in study done by Shamoon 22 et al percentage of spontaneous and traumatic bleeding in patients was 32% and 68% respectively.…”

“…Which is corresponding to Saurabh Mishra 15 et al (58%) and MM Uddin 11 et al (60%). Family history was positive in 70% of cases in study done by R. K. Nigam 14 et al and 40% in study done by MA Karim 12 et al In most of the studies family history was positive in 40 -70% of cases. These results are in accordance with other studies.…”

“…The difference in percentage of mild, moderate and severe cases between present study and in study done by R. K. Nigam et al 14 appears due to difference in study design. In study done by R. K. Nigam et al 14 all registered patients of haemophilia were studied which resulted in larger sample size and larger spectrum of cases. However present study was observational study where only the patients reporting during the study period were included.…”

Reassessment of Coagulation Dysfunction And Its Impact on Classification of Hemophilia in Duhok City

“…The higher mutation rate in hemophilia A than hemophilia B could justify the later finding. The results of the current study showed that the moderate cases were more than the mild and the severe ones.This result is compatible with the results of Al Tonbaryet al, (2010) who indicated that the moderate presentation represents the majority in 17.2% followed by sever presentationin 4.7%,and Nigam et al, 2014 who found that the frequent type of hemophila was moderate group in hemophilia A 87 (38.84%), while our findings differed from these of Al-Beldawi, (2010), andMannuccio (2018), who proved the severe bleeding cases were more than the moderate cases.…”

Assessment of Chromolena odorata potential ability of hemostasis in patients with and without Haemophilia A Inhibitors

“…These inhibitors can signi icantly increase morbidity status in terms of bleeding complications. Hemarthrosis is the most commonly manifested co-morbidity of hemophilia pathogenesis (Nigam et al, 2014). Severity of Haemophilia A can be diagnosed clinically with lower coagulation factor level of <0.01 IU/ml and < 1% of normal (Uijl et al, 2011).…”