Factor VIII Inhibitors in Patients with Hemophilia A

Ören, Hale; Yaprak, Işın; İrken, Gülersu

doi:10.1159/000040966

Acta Haematol

1999

DOI: 10.1159/000040966

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Factor VIII Inhibitors in Patients with Hemophilia A

Hale Ören¹,

Işın Yaprak²,

Gülersu İrken³

Abstract: In 58 hemophilia A patients aged 1–18 years (mean 9.5 ± 4.7 years), the prevalence of inhibitors was found to be 27% by the Bethesda method in November 1995. Inhibitor activity was not detected in any of 14 patients with mild hemophilia while it was present in 9 of 27 (33%) patients with moderate, and 7 of 17 (41%) with severe disease. During follow-up, the inhibitors were transient in 10 of 16 patients (17%) and the prevalence of inhibitors was 10% at the end of the study. Our study has demonstrated that the … Show more

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Cited by 12 publications

(16 citation statements)

References 34 publications

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“…In contrast to the findings of Oren et al [1], none of our patients got rid of the inhibitors over 1-5 years of follow-up. As their patients were not clinically symptomatic due to inhibitors but were inhibitor positive only on assay, they are likely to have less severe immunological disturbance than our patients who were primarily picked up because of clinical suspicion of inhibitors.…”

contrasting

confidence: 99%

“…In line with the findings of Oren et al [1], most of our patients with inhibitors belonged to higher age groups compared to the hemophilia patients with inhibitors seen in the West [6], probably because in both these places, the patients received few factor concentrates till they started developing joint deformity in late childhood and juvenile years.…”

supporting

confidence: 90%

“…Generally, inhibitors tend to occur in severe hemophiliacs; hence, it is surprising to see so many moderately affected hemophiliacs developing inhibitors in the series of Oren et al [1]. All our patients who developed clinically significant inhibitor had severe disease (basal factor VIII level less than 1% before they developed inhibitors).…”

mentioning

confidence: 63%

“…We read with interest the paper by Oren et al [1]. It is expected that there are approximately 110,000 hemophilia A and 50,000 hemophilia B patients in India, which has a population of more than one billion.…”

mentioning

confidence: 99%

“…It remains to be seen whether some of the patients of Oren et al [1] who got rid of their inhibitors redevelop them when rechallenged by factor concentrates.…”

mentioning

confidence: 99%

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

Shetty¹,

Ghosh²,

Pathare³

et al. 2000

Acta Haematol

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contrasting

confidence: 99%

supporting

confidence: 90%

mentioning

confidence: 63%

mentioning

confidence: 99%

“…It remains to be seen whether some of the patients of Oren et al [1] who got rid of their inhibitors redevelop them when rechallenged by factor concentrates.…”

mentioning

confidence: 99%

See 3 more Smart Citations

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

Shetty¹,

Ghosh²,

Pathare³

et al. 2000

Acta Haematol

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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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Two patients with haemophilia and acute leukaemia

Zülfikar¹

2002

Haemophilia

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Acute leukaemia is the commonest form of malignancy in childhood. The coincidental development of leukaemia in children or adults with haemophilia is extremely rare, although cases of leukaemia and other malignancies have been reported previously in HIV-positive subjects. Of a total of 440 people with haemophilia registered with our society, two were diagnosed with acute leukaemia last year. The development of leukaemia in a subject with haemophilia has previously been reported from our country in 1985, but the negative HIV status of these recent cases is very interesting. The first case involved a 14-year-old boy with moderate haemophilia A, who developed acute lymphoblastic leukaemia (ALL) [French-American-British (FAB) classification L2]. The second subject was a 16-year-old boy who had moderately severe haemophilia A with no previous family history, and developed acute nonlymphocytic (myelomonocytic) leukaemia (FAB-M4). Both patients received conventional chemotherapy and this report discusses the potential problems in management of such cases, including diagnosis and administration of chemotherapy in subjects with a pre-existing haemorrhagic disorder. Extensive cutaneous and mucosal bleeding, as well as bleeds in joints previously affected by haemarthrosis and alterations of haematological values were all initially suggestive of the development of inhibitors against factor VIII, but the appearance of blasts in the peripheral blood and bone marrow led to the definitive diagnosis. The risk of bleeding, due to the combination of both leukaemia and the consequences of the chemotherapy, was overcome by the administration of coagulation factor concentrates (daily initially followed by prophylactic doses after successful induction of remission in both patients). The young patient with ALL is now receiving the maintenance phase of the Children's Cancer Study Group 1961 protocol and is in the 15th month of follow-up, without any complications. The other case relapsed in the seventh month, developing enterobacter sepsis, and died. An important lesson to be learnt from these cases is that the possible diagnosis of leukaemia should not be overlooked in a patient with haemophilia and severe haemorrhagic problems, if the first-line differential diagnosis of inhibitor development against factor VIII (or IX) has been excluded.

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Factor VIII Inhibitors in Patients with Hemophilia A

Cited by 12 publications

References 34 publications

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

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

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

Two patients with haemophilia and acute leukaemia

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