Clinically Significant Inhibitors in Hemophilia A Patients from India Tend to Persist

Shetty, Shrimati; Ghosh, Kanjaksha; Pathare, Anil; Mohanty, Dipika

doi:10.1159/000041044

Cited by 6 publications

(7 citation statements)

References 3 publications

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“…This is a much older age than that of the western haemophilia cohorts, who developed inhibitors by the first decade of life. In most of our haemophilia patients with symptomatic inhibitors, the inhibitors tend to persist [19].…”

Section: Discussionmentioning

confidence: 99%

“…It has been suggested that patients who are put on one brand of factor concentrate tend to develop inhibitors less often than patients who frequently switch products [19,24]. The large majority of Indian haemophiliacs have no other option but to use products that are available from the Haemophilia Federation, which in turn buys factor concentrates from many different companies as a cost‐reduction exercise.…”

Section: Discussionmentioning

confidence: 99%

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Development of inhibitors in patients with haemophilia from India

et al. 2001

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Four hundred and seven patients (352 haemophilia A and 55 haemophilia B) were investigated for the presence of factor VIII and IX inhibitors. Twenty-four out of 292 severe and two out of 36 moderate haemophilia A patients showed the presence of inhibitors. The mean age at development of inhibitors was 17.7 years (range 6-52 years). In 12 patients the inhibitors were detected due to suboptimal response to factor replacement therapy (symptomatic) and in the remaining 14 patients the inhibitors were detected during the routine screening of the patients' samples for inhibitors. They had, however, responded well to the usual doses of factor concentrates and there was no suspicion in these patients that they had developed an inhibitor (asymptomatic). There were two families in which the inhibitors were detected in more than one family member. The level of inhibitors in symptomatic patients ranged from 2.2 Bethesda units (BU) mL(-1) to 460.6 BU mL(-1), and in asymptomatic patients it ranged from 0.8 BU mL(-1) to 3.2 BU mL(-1). The inhibitors persisted in all patients except one, who developed an inhibitor postoperatively for a brief period of 3 months. All these patients were followed up from first factor exposure and were tested for inhibitors at least twice a year. The mean number of exposure days before they developed inhibitors was 47.5 exposure days (range 17-98 exposure days). No inhibitors appeared after more than 100 exposure days in any of the patients. When 50 consecutive patients were investigated for intron 22 inversions of the factor VIII gene, 17 patients were found to be positive for inversions (10 proximal inversion; seven distal inversion) out of whom four patients developed inhibitors, three patients belonging to the same family. Out of 35 haemophilia B patients, only one patient developed an inhibitor. The overall prevalence of inhibitors was thus 8.2%, which is similar to the reports from western countries, prior to the introduction of highly purified factor concentrate therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Development of inhibitors in patients with haemophilia from India

et al. 2001

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“…After inhibitor diagnosis, they were managed with plasma-derived activated prothrombin complex concentrate (aPCC; FEIBA Ò ; Baxter Bioscience, Vienna, Austria) or highly purified factor IX concentrates. The age of developing inhibitors among those patients was higher when compared to that reported by Western studies, most likely due to late exposure to factor replacement [15]. Ghosh et al (2002) [16] described a case series of 35 patients, out of whom six developed inhibitors postoperatively.…”

Section: Burden Of Inhibitorsmentioning

confidence: 84%

“…During a 5 year follow up, 23 of 283 severe hemophilia patients were investigated for the presence of inhibitors before surgery or because of a suboptimal response to factor replacement therapy [15]. Nine patients were positive for inhibitors, and no transient cases were found in that cohort.…”

Section: Burden Of Inhibitorsmentioning

confidence: 99%

Hemophilia Care in India: A Review and Experience from a Tertiary Care Centre in Uttar Pradesh

Phadke

2011

Indian J Hematol Blood Transfus

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Approximately 14,000 people with hemophilia are registered at the Hemophilia Federation of India; however, hemophilia remains under-diagnosed and many cases are not registered. In June 2009, the Government of Uttar Pradesh made anti-hemophilic factors available at a few centers, including the Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow. Consequently, the level of hemophilia care has improved considerably in recent times. Amongst the many challenges facing people with hemophilia, the development of inhibitors, which neutralize clotting factors provided by replacement therapy, is the most feared one. Healthcare professionals who treat people with hemophilia should not only be knowledgeable about the condition and committed to bettering the management of hemophilia, but also take responsibility for the judicious allocation of resources for various aspects of managing hemophilia. This manuscript aims to raise awareness regarding the detection and management of inhibitors in hemophilia based on the experience of a tertiary care hemophilia treatment centre in Uttar Pradesh, India.

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“…Lower prevalence of inhibitors in Indian patients is related to the less than optimum factor concentrate and blood component usage in such patients. It is not known how often Indian haemophiliacs develop transient inhibitors to FVIII but clinically significant inhibitors in Indian haemophiliacs tend to persist [3]. In India, transient inhibitors to FVIII may have been missed because in most centres of India that undertake care haemophilia patients, inhibitor screening and assays are not routinely performed.…”

Section: Introductionmentioning

confidence: 99%

First‐time development of FVIII inhibitor in haemophilia patients during the postoperative period

et al. 2002

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Development of inhibitor to FVIII in haemophilia patients is well-known and is not uncommon. However, their development for the first time during the postoperative period has hardly been reported. In a developing country such as India, where resources are limited, development of such an eventuality may prove disastrous. However, as many of our patients are sparingly treated, therefore, even if they test negative for the inhibitor preoperatively, they may get the requisite FVIII antigenic stimulation during the preoperative and immediate postoperative period, leading to the development of inhibitors during this critical time of wound healing. We describe here six patients who developed such an inhibitor, from a group of 35 patients with haemophilia A who underwent various surgical procedures (19%). We stress that such an eventuality may not remain rare in developing countries as more patients of severe haemophilia undergo surgery and are therefore challenged for the first time in their life with large amounts of FVIII concentrate during their preoperative period.

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Clinically Significant Inhibitors in Hemophilia A Patients from India Tend to Persist

Cited by 6 publications

References 3 publications

Development of inhibitors in patients with haemophilia from India

Development of inhibitors in patients with haemophilia from India

Hemophilia Care in India: A Review and Experience from a Tertiary Care Centre in Uttar Pradesh

First‐time development of FVIII inhibitor in haemophilia patients during the postoperative period

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