Rituximab as an adjuvant therapy to immune tolerance in a haemophilia A boy with high inhibitor titre

Chuansumrit, Ampaiwan; Husapadol, S.; Wongwerawattanakoon, Pakawan; Hongeng, Suradej; Sirachainan, Nongnuch; Pakakasama, Samart

doi:10.1111/j.1365-2516.2006.01407.x

Cited by 11 publications

(18 citation statements)

References 5 publications

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“…BU, Bethesda units. The frequency of bleeding episodes during the ITT period in this reported patient was not as low as those of the previously reported patients with hemophilia A at 2 years of age [7,8]. The reason for this finding is that our reported patient was 10 years of age with existing targeted hemophilic arthropathies at both the elbow and knee joints.…”

Section: Discussioncontrasting

confidence: 74%

“…Rituximab, a monoclonal antibody against CD20-positive B cells, has shown an effectiveness in hemophiliacs with inhibitor [6][7][8]. In the present study, we report the effectiveness of rituximab as an adjuvant for ITT in a boy with hemophilia B inhibitor and anaphylaxis to factor IX concentrate.…”

Section: Introductionmentioning

confidence: 56%

“…Adrenalin and hydrocortisone were administered with responsiveness. From the ages of 2 to 10 years, even though the nurturing parents provided the appropriate care for avoiding trauma, the patient experienced a median of eight bleeding episodes per year (interquartile range, [3][4][5][6][7][8][9][10][11][12]. Due to the high cost of recombinant activated factor VII, the boy received inadequate treatment resulting in progressive hemophilic arthropathy.…”

Section: Case Reportmentioning

confidence: 99%

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The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate

Chuansumrit¹,

Moonsup²,

Sirachainan³

et al. 2008

Blood Coagulation &Amp; Fibrinolysis

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We describe a 10-year-old severe hemophilia B boy with a stop codon mutation of exon 2 in the factor IX gene who developed high inhibitor of 70 Bethesda units (BU) from 12 months of age after exposure to prothrombin complex concentrate for 14 days. The inhibitor spontaneously disappeared within 3 months. The patient, however, exhibited anaphylactic reaction to the administration of prothrombin complex concentrate and factor IX concentrate at ages 15 and 23 months, respectively. Although recombinant activated factor VII was alternatively given, he suffered from progressive hemophilic arthropathy. At the age of 10 years, the boy underwent desensitization to factor IX concentrate and could tolerate factor IX concentrate of 40 U/kg administered on day 9 of desensitization. Unfortunately, the inhibitor of 16 BU was detected on day 6 and rapidly increased to 180 BU on day 9 of desensitization. Rituximab 375 mg/m2 per week was therefore immediately initiated on day 10 and a total of four doses were given. The inhibitor gradually decreased to 21.5 BU after the fourth dose of rituximab. The daily factor IX concentrate administration of 40 U/kg was continued for 1 month and decreased to three times per week for another month, and then to once to twice per week for the remaining 14 months of desensitization. The patient was able to attend regular school and the most recent inhibitor ranged from 4.4 to 10 BU. No proteinuria or alteration of renal function was found. In conclusion, rituximab is a helpful adjuvant to immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate.

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Section: Discussioncontrasting

confidence: 74%

Section: Introductionmentioning

confidence: 56%

Section: Case Reportmentioning

confidence: 99%

See 1 more Smart Citation

The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate

Chuansumrit¹,

Moonsup²,

Sirachainan³

et al. 2008

Blood Coagulation &Amp; Fibrinolysis

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“…Similarly, rituximab cannot be advocated. There is some history of the use of rituximab in inhibitors to factor VIII and factor IX [28,29,30,31]. Rituximab has the ability to lower antibody levels to undetectable levels rapidly and needs to be studied further.…”

Section: Discussionmentioning

confidence: 99%

Factor X Inhibitor: A Fulminant Presentation and Fatal Course of a Rare Syndrome in a 59-Year-Old Male

Gollard¹,

Rahman²,

Ratnasabapathy³

2012

Acta Haematol

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Factor X inhibitors are rare. The few cases documented in the literature have occurred after viral prodromes, in association with cancer, or after exposure to antibiotics. Acquired factor X deficiencies are also rare and their etiology is largely unknown. We report a new case of a factor X inhibitor and review prior cases of both factor X inhibitors and non-amyloidosis-related acquired factor X deficiencies.

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“…It might be due to the presence of activated clotting factors in the aPCC prior to rFVIIa administration. Sequential therapy of rFVIIa and aPCC was reported among ten children who had failed their home treatment with rFVIIa and aPCC alone (n = 9),65,66 and refractory bleeding to the treatment of rFVIIa (n = 1) 67. They received alternating doses of rFVIIa (80 to 225 μg/kg/dose) and aPCC (35 to 80 IU/kg/dose) every 6 hours; and 1 to 3 doses of rFVIIa were given every 2 hours between aPCC doses.…”

Section: Independent Published Reportsmentioning

confidence: 99%

Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors

Chuansumrit¹

2010

JBM

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Hemophilia patients with inhibitors faced the constraint of inadequate treatment for several years before the era of recombinant factor VIIa (rFVII). Initially, rFVIIa was used in the compassionate-use programs. After a worldwide license was issued, more than 1.5 million doses were administered. Bleeding of joints and muscles was controlled effectively by means of an early home treatment program, with either a standard dose of 90 μg/kg every 2 to 3 hours for a few doses or a single dose of 270 μg/kg. For more serious bleeding episodes or minor surgery, an initial dose of 90 μg/kg was given every 2 hours for 24 to 48 hours followed by increased intervals of 3 to 6 hours according to the severity of bleeding and efficacy of bleeding control. In cases of major surgery such as orthopedic procedures, the same regimen can be applied except for a higher initial dose of 120 to 180 μg/kg. However, increasing the dose should be considered if there are unexpected bleeding complications since the half-life and clearance of rFVIIa differ between individuals. In addition, prophylaxis is administered to a small number of patients. Finally, the reported thromboembolic events found in hemophilia patients with inhibitors receiving rFVIIa are extremely low, much less than 1%.

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Rituximab as an adjuvant therapy to immune tolerance in a haemophilia A boy with high inhibitor titre

Cited by 11 publications

References 5 publications

The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate

The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate

Factor X Inhibitor: A Fulminant Presentation and Fatal Course of a Rare Syndrome in a 59-Year-Old Male

Critical appraisal of the role of recombinant activated factor VII in the treatment of hemophilia patients with inhibitors

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