scholarly journals Screening and quantitative estimation of factor VIII inhibitors by Nijmegen-Bethesda assay in hemophilia a patient of Southern Odisha, India

2020 ◽  
Vol 7 (2) ◽  
pp. 255
Author(s):  
Jayanti Nayak ◽  
Sonali Kar ◽  
Monali Kar
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Quantitative Estimation ◽  
Current Treatment ◽  
Haemophilia A ◽  
Severe Hemophilia ◽  
Blood Products ◽  
Clotting Factors ◽  
Cross Sectional ◽  
Medical College

Background: The current treatment of haemophilia is replacement of factor VIII or IX which is effective till development of inhibitor against factors. There has been no study on factor VIII inhibitors in Southern Odisha using Nijmegen–Bethesda assay. This study was planned with objectives to screen factor VIII inhibitors in hemophilia-A patients, to do quantitative estimation of it using Nijmegen-Bethesda assay and to explore factors associated with development of inhibitors.  Methods: This cross-sectional study was carried out from September 2016 to August 2018 in Department of pathology, MKCG medical college, Berhampur. Haemophilia-A patients coming to MKCG medical college and registered Haemophilia-A cases under Haemophilia society of Berhampur were included. Patients denying consent and having multiple clotting factors deficiencies were excluded. 1.8ml blood was collected. Mixing study was done to screen factor VIII inhibitors and then in positive cases inhibitors level measured by Nijmegen-Bethesda method. All data were analysed using SPSS (version 16.0).Results: 70 cases of Hemophilia-A patients were studied. Majority (50%) were with severe hemophilia-A. 7 patients developed inhibitors where 3 were high and 4 were low responders. Inhibitor level ranged from 0.8 to 64 Nijmegen-Bethesda units. Patients with severe hemophilia A, more than 10 transfusions and who switched to receive recombinant FVIII from other blood products developed inhibitors which were significant.Conclusions: Severity of hemophilia, increase frequency of transfusion and switching of blood products significantly increases chances of inhibitor development and hence intensive inhibitor screening is needed in these cases. Quantification of inhibitor is needed to monitor treatment and to manage bleeding episodes effectively.

scholarly journals Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center

2017 ◽  
Vol 26 (1) ◽  
pp. 47-53
Author(s):  
Teny T. Sari ◽  
Novie A. Chozie ◽  
Djajadiman Gatot ◽  
Angela B.M. Tulaar ◽  
Rahayuningsih Dharma ◽  
...  
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Joint Destruction ◽  
Joint Damage ◽  
Cross Sectional Study ◽  
Treatment Center ◽  
Severe Hemophilia ◽  
Older Children ◽  
Cross Sectional ◽  
Episodic Treatment

Background: Recurrent joint bleeds leading to arthropathy is the main problem in severe hemophilia children. This study aimed to investigate joint status in severe hemophilia A children receiving episodic treatment in Cipto Mangunkusumo Hospital, Jakarta.Methods: A cross-sectional study was conducted in Cipto Mangunkusumo Hospital as Indonesian National Hemophilia Treatment Center on children (4–18 years) with severe hemophilia A, who previously received episodic treatment, with no history of inhibitor factor VIII. Hemophilia Joint Health Score was evaluated according to HJHS version 2.1 2011. Joint ultrasonography was done for six index joints (bilateral elbows, knees and ankles) using Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) methods. Data of age of first joint bleed, number of target joints and inhibitor factor VIII were obtained from the Pediatric Hemophilia Registry and medical records.Results: There were 59 subjects aged 4 to 18 years. Twenty-nine out of 59 (49.2%) subjects experienced first joint bleed before of 2 years of age. The most common of joint bleeds was a right ankle. Mean total HJHS was 8.71±8.73. Subjects aged 4–10 years showed lower HJHS (4.6±3.7) as compared to subjects aged >10–18 years (12.3±10.3), p<0.001; 95% CI=4.9–13. Mean HEAD-US scores in subjects aged 4–10 years (18.7±5.6) was lower than in subjects aged >10–18 years (28±7.9), p<0.001, 95% CI= -12.9–-5.6.Conclusion: HJHS and HEAD-US scores of severe hemophilia A children receiving episodic treatment aged 4–10 years are lower compared to subjects aged >10–18 years, indicating more severe joint destruction in older children and progressivity of joint damage over time. It is important to start prophylactic treatment to prevent progressivity of joint damage.

scholarly journals Hemophilia a in a Girl with Deletion of a Part of the long ARM of one X Chromosome

1977 ◽  
Author(s):  
M. Samama ◽  
Ch. Perrotez ◽  
R. Houissa ◽  
A. Hafsia ◽  
J. Seger
Keyword(s):  
Factor Viii ◽  
X Chromosome ◽  
Bleeding Time ◽  
Hemophilia A ◽  
Haemophilia A ◽  
Severe Hemophilia ◽  
First Case ◽  
Sex Chromatin ◽  
Phenotypic Female ◽  
Blood Grouping

A severe hemorragic diathesis has been discovered in a 10 years old girl whose brother has severe hemophilia with classical haemarthrosis. The factor VIII biological activity is less than 1% in both of them with an almost normal factor VIII related antigen. The bleeding time was normal. There was no consanguinity and paternity was not disproved by extensive blood grouping tests. Cytogenetics studies showed a deletion of a part of the long arm of one X chromosome. The formula of the caryotype is 46, X, del X (q.212). The sex chromatin showed the presence of a phenotypic female. The X chromatin was smaller than normal. This is the first case to our knowledge of true female haemophilia A due to a deletion of one X chromosome.

Correlation of Antibodies to LAV/HTLV III in Hemophiliacs with the Use of Virus-Inactivated Clotting Factors

1986 ◽  
Vol 56 (01) ◽  
pp. 050-052 ◽  
Author(s):  
Brigitte Dietz ◽  
H J Klose ◽  
L Gürtler ◽  
J Eberle ◽  
F Deinhardt ◽  
...  
Keyword(s):  
Heat Treatment ◽  
Factor Viii ◽  
Acquired Immunodeficiency Syndrome ◽  
Hemophilia A ◽  
Factor Ix ◽  
Blood Products ◽  
Clotting Factors ◽  
Acquired Immunodeficiency ◽  
Immunodeficiency Syndrome

SummaryThe retrovirus LAV/HTLV III, highly likely to be responsible for the acquired immunodeficiency syndrome (AIDS) in some recipients of blood products, can be inactivated by chemical and/ or heat treatment, so the use of virus-inactivated factor VIII and factor IX preparations for treating hemophilia A and B has become important. We examined hemophilic children and found that those children treated since 1979 with virus-inactivated preparations did not develop antibodies against LAV/HTLV III. In contrast, 77% of patients treated with conventional factor VIII or factor IX preparations had antibodies against this virus.

scholarly journals The prevalence of factor VIII inhibitor in patients with severe hemophilia-A and its clinical characteristics

2016 ◽  
Vol 45 (4) ◽  
pp. 177 ◽  
Author(s):  
Harijadi Harijadi ◽  
Djajadiman Gatot ◽  
Arwin AP Akib
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Factor Viii Inhibitor ◽  
Severe Hemophilia ◽  
Cross Sectional ◽  
On Demand ◽  
Treat Ment ◽  
Inhibitor Titre ◽  
Chronic Arthropathy ◽  
Viii Inhibitor

Background Hemophilia is a hereditary blood-clotting disorderdue to factor VIII deficiency. Up to this date, the administration offactor VIII in preventing and managing bleeding has been the maintreatment. One of the complications, which may occur due to re-peated administrations of factor VIII, is the formation of factor VIIIantibody (factor VIII inhibitor).Objective To find out the prevalence of severe hemophilia-A withfactor VIII inhibitor and its clinical characteristics.Methods A cross-sectional descriptive study was performed onchildren with severe hemophilia-A at the National Hemophilia CareCentre, Cipto Mangunkusumo Hospital, Jakarta, Indonesia, in June-August 2004.Results Out of 45 children studied, 16 had factor VIII inhibitor withaverage inhibitor titre of 1.15 Bethesda units (BU) (range 0.15-15BU). Most of them (12 patients) had inhibitor titre <5 BU. Chronicarthropathy was found in 17 out of 45 (37%) children with severehemophilia-A, consisting of nine patients from positive inhibitorgroup and 8 patients from negative inhibitor group. Thirty-ninepatients (86%) used an on-demand treatment pattern, among whom15 had positive inhibitor. Among patients receiving prophylactictreatment pattern, only one had positive inhibitor. There were 39patients (86%) treated using cryoprecipitate, among whom factorVIII inhibitor was found in 12, while among those treated with fac-tor VIII concentrate, the inhibitor was positive in 4/6. The averageamount of factor VIII transfused in positive and negative factor VIIIinhibitor groups was similar.Conclusion The prevalence of factor VIII inhibitor in severe he-mophilia-A patients was 35%. Chronic arthropathy occurred moreoften in patients with positive factor VIII inhibitor. Factor VIII inhibi-tor was found more frequently in patients with an on-demand treat-ment pattern and in those using factor VIII concentrate

T Lymphocyte Proliferative Responses Induced by Recombinant Factor VIII in Hemophilia A Patients with Inhibitors

1996 ◽  
Vol 76 (01) ◽  
pp. 017-022 ◽  
Author(s):  
Sylvia T Singer ◽  
Joseph E Addiego ◽  
Donald C Reason ◽  
Alexander H Lucas
Keyword(s):  
T Lymphocytes ◽  
Factor Viii ◽  
Cellular Proliferation ◽  
Mononuclear Cells ◽  
Hemophilia A ◽  
Normal Subjects ◽  
Normal Male ◽  
Cell Fractionation ◽  
Severe Hemophilia ◽  
Human Factor Viii

SummaryIn this study we sought to determine whether factor VUI-reactive T lymphocytes were present in hemophilia A patients with inhibitor antibodies. Peripheral blood mononuclear cells (MNC) were obtained from 12 severe hemophilia A patients having high titer inhibitors, 4 severe hemophilia A patients without inhibitors and 5 normal male subjects. B cell-depleted MNC were cultured in serum-free medium in the absence or presence of 2 µg of recombinant human factor VIII (rFVIII) per ml, and cellular proliferation was assessed after 5 days of culture by measuring 3H-thymidine incorporation. rFVIII induced marked cellular proliferation in cultures of 4 of 12 inhibitor-positive hemophilia patients: fold increase over background (stimulation index, SI) of 7.8 to 23.3. The remaining 8 inhibitor-positive patients, the 4 hemophilia patients without inhibitors and the 5 normal subjects, all had lower proliferative responses to rFVIII, SI range = 1.6 to 6.0. As a group, the inhibitor-positive subjects had significantly higher proliferative responses to rFVIII than did the inhibitor-negative and normal subjects (p < 0.05 by t-test). Cell fractionation experiments showed that T lymphocytes were the rFVIII-responsive cell type, and that monocytes were required for T cell proliferation. Thus, rFVIII-reactive T lymphocytes are present in the peripheral circulation of some inhibitor-positive hemophilia A patients. These T cells may recognize FVIII in an antigen-specific manner and play a central role in the regulation of inhibitor antibody production

Incidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom

Blood ◽  
2011 ◽  
Vol 117 (23) ◽  
pp. 6367-6370 ◽  
Author(s):  
Charles R.M. Hay ◽  
Ben Palmer ◽  
Elizabeth Chalmers ◽  
Ri Liesner ◽  
Rhona Maclean ◽  
...  
Keyword(s):  
United Kingdom ◽  
Factor Viii ◽  
Hemophilia A ◽  
Interquartile Range ◽  
Severe Hemophilia ◽  
The United Kingdom ◽  
Potential Risk Factors ◽  
History Of ◽  
Previously Treated Patients ◽  
Adjusted Incidence

Abstract The age-adjusted incidence of new factor VIII inhibitors was analyzed in all United Kingdom patients with severe hemophilia A between 1990 and 2009. Three hundred fifteen new inhibitors were reported to the National Hemophilia Database in 2528 patients with severe hemophilia who were followed up for a median (interquartile range) of 12 (4-19) years. One hundred sixty (51%) of these arose in patients ≥ 5 years of age after a median (interquartile range) of 6 (4-11) years' follow-up. The incidence of new inhibitors was 64.29 per 1000 treatment-years in patients < 5 years of age and 5.31 per 1000 treatment-years at age 10-49 years, rising significantly (P = .01) to 10.49 per 1000 treatment-years in patients more than 60 years of age. Factor VIII inhibitors arise in patients with hemophilia A throughout life with a bimodal risk, being greatest in early childhood and in old age. HIV was associated with significantly fewer new inhibitors. The inhibitor incidence rate ratio in HIV-seropositive patients was 0.32 times that observed in HIV-seronegative patients (P < .001). Further study is required to explore the natural history of later-onset factor VIII inhibitors and to investigate other potential risk factors for inhibitor development in previously treated patients.

EFFICACY AND SAFETY OF MOROCTOCOG ALFA IN AN OPEN, MULTICENTER, PROSPECTIVE, CLINICAL STUDY IN CHILDREN 2 TO 6 YEARS OF AGE WITH SEVERE HEMOPHILIA A

2021 ◽  
Vol 100 (6) ◽  
pp. 154-161
Author(s):  
M.A. Timofeeva ◽  
◽  
V.V. Lebedev ◽  
O.I. Plaksina ◽  
N.I. Zozulya ◽  
...  
Keyword(s):  
Adverse Reactions ◽  
Hemophilia A ◽  
Prospective Clinical Study ◽  
Study Group ◽  
Haemophilia A ◽  
Severe Hemophilia ◽  
Efficacy And Safety ◽  
Prophylactic Dose ◽  
Traumatic Bleeding ◽  
Post Traumatic

The purpose of the study was to assess the efficacy, safety and pharmacokinetics of the moroctocog alfa (Octofactor) in children aged 2-6 with haemophilia A. Materials and methods of research : six patients between 2 and 6 years of age (average age 4.3±0.8 years) were included in the open multicenter prospective clinical trial. The efficacy of the drug was assessed against the background of the introduction of 30±10 IU/kg every 2–3 days, the safety was assessed by the frequency and causality of adverse reactions. Results: 7 post-traumatic bleeding was registered. The average prophylactic dose of the drug is 37.84±7.13 IU/kg. The dose of the drug for stopping bleeding was 1000 IU. 2 adverse events have been reported that are not related to moroсtocog alfa. Conclusion: the obtained data indicate the efficacy and safety of moroсtocog alfa in the study group of patients.

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