Spontaneously Acquired Factor Viii Inhibitor in a Non‐haemophiliac Child

Labbé, Alain; Dubray, Claude; Bezou, M J; Travade, P.; Coulet, M

doi:10.1111/j.1651-2227.1983.tb09783.x

Cited by 7 publications

(3 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Of these, nine are presumptive inhibitors, based on the presence of a decreased level of a single factor. Two are definite acquired inhibitors [19, 20] because there were documented anti‐FVIII inhibitor titres present. Both of the latter experienced bruising and one also suffered a traumatic subdural haematoma.…”

Section: Literature Reviewmentioning

confidence: 99%

See 1 more Smart Citation

Acquired anti‐FVIII inhibitors in children

Moraca

Ragni

2002

Haemophilia

View full text Add to dashboard Cite

Acquired inhibitors to FVIII (anti-FVIII) are uncommon in children. An acquired anti-FVIII developed in a previously healthy 4-year-old boy treated with penicillin for streptococcal pharyngitis. Aspirin prophylaxis begun for suspected rheumatic fever led to compartment syndromes of all four extremities, which resolved with high-dose FVIII and surgical decompression. Anti-FVIII in this patient, and the five additional cases identified in a survey of 160 haemophilia treatment centres, occurred at a median age of 8 years, with median initial and peak titres of 4.6 and 6.9 Bethesda Units (BU), respectively. All six presented with bleeding, including haematomas (three intramuscular, one intracranial), and ecchymoses in three. The median baseline FVIII was 0.05 U mL(-1), and the median baseline activated partial thromboplastin time (APTT) was 79.8 s. The inhibitor resolved completely in five patients (83%) within a median 5 months, after treatment with FVIII concentrate, steroids, cytoxan, methotrexate, and no treatment. The inhibitor persisted in the patient with Goodpasture's disease, despite steroids, cytoxan, cyclosporin, and intravenous gamma globulin. Aspirin therapy, in two, worsened ongoing bleeding. The association of penicillin-like drugs in this and three other cases in the literature suggest that to avoid potential catastrophic bleeding, it is prudent to obtain an APTT prior to initiating aspirin for suspected rheumatic fever. In conclusion, acquired anti-FVIII inhibitors in children may cause severe bleeding, and remit in the majority after FVIII and/or immunosuppressive therapy.

show abstract

Section: Literature Reviewmentioning

confidence: 99%

“…A literature review of acquired inhibitors to coagulation FVIII over the past 20 years yielded 11 patients [15–20] with prolonged APTT and APTT mix (Table 2). Of these, nine are presumptive inhibitors, based on the presence of a decreased level of a single factor.…”

Section: Literature Reviewmentioning

confidence: 99%

Acquired anti‐FVIII inhibitors in children

Moraca

Ragni

2002

Haemophilia

View full text Add to dashboard Cite

show abstract

“…21 It is unusual to find inhibitors of specific coagulation factors in young children. 22,23 When present, these inhibitors are directed predominantly against factor VIII and appear to be related to an antecedent viral infection in some cases. Fortunately, acquired factor VIII inhibitors appear to be short-lived in this age group.…”

Section: Extrinsic Pathwaymentioning

confidence: 99%

Case 6-1998

Ewenstein¹,

Cott²

1998

N Engl J Med

View full text Add to dashboard Cite

Transient hemorrhagic diathesis associated with an inhibitor of prothrombin with lupus anticoagulant in a 1½-year-old girl: Report of a case and review of the literature

Lee

Nardi

et al. 1996

Am. J. Hematol.

View full text Add to dashboard Cite

Acquired inhibitors of coagulation causing bleeding manifestations are rare in children, particularly without an associated underlying disorder such as autoimmune disease. We describe an otherwise healthy 1 1/2-year-old girl who had extensive spontaneous bruising and prolonged bleeding from venipuncture sites. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were prolonged, with evidence of an immediate-acting inhibitor. Thrombin clotting time, fibrinogen, and platelets were normal. Biologic assay of factors II, V, VII, and X were all low, with increasing values at higher dilutions. However, by immunoassay and/or chromogenic assays, only factor II was reduced. An antibody which failed to neutralize prothrombin activity in vitro was detected against radiolabeled prothrombin. Coagulation studies normalized in parallel with clinical recovery and disappearance of the antibody. This case demonstrates acute hypoprothrombinemia-lupus anticoagulant syndrome as a rare presentation of bleeding diathesis in a healthy young child.

show abstract

Spontaneously Acquired Factor Viii Inhibitor in a Non‐haemophiliac Child

Cited by 7 publications

References 9 publications

Acquired anti‐FVIII inhibitors in children

Acquired anti‐FVIII inhibitors in children

Case 6-1998

Transient hemorrhagic diathesis associated with an inhibitor of prothrombin with lupus anticoagulant in a 1½-year-old girl: Report of a case and review of the literature

Contact Info

Product

Resources

About