Immunologie Studies in von Willebrand’s Disease

Y, Sultan; Simeon, J.; Maisonneuve, P.; Jp, Caen

doi:10.1055/s-0038-1647917

Cited by 11 publications

(2 citation statements)

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“…These observations raise the intriguing possibility that there may be a continuous conversion of the higher molecular weight forms (possessing von Willebrand factor activity) into lower molecular forms (lacking von Willebrand activity) (28). Similar observations after transfusion in patients with von Willebrand disease suggest in vivo conversion of high molecular weight VIII:Ag into lower molecular weight VIII:Ag (9).…”

Section: Methodssupporting

confidence: 63%

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Structural analysis of factor VIII antigen in von Willebrand disease.

Nachman¹,

Jaffe²,

Miller³

et al. 1980

Proc. Natl. Acad. Sci. U.S.A.

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The Factor VIII antigen molecules in the plasma of patients with classical type 1 and variant type 2A von Willebrand disease were compared to the Factor VIII antigen molecules in normal plasma. Factor VIII antigen was isolated from plasma by solid-phase immunoprecipitation and analyzed by NaDodSO4/polyacrylamide gel electrophoresis; the stained Factor VIII antigen bands were removed, radioiodinated, and subjected to tryptic digestion. Computerized analysis of autoradiographs revealed that the two-imensional peptide maps of the different Factor VIII antigens were remarkably similar. The results suggest that the Factor VIII antigen molecules in these two forms of von Willebrand disease are probably identical to the Factor VIII antigen molecules present in normal plasma. It is thus likely that the differences observed in plasma Factor VIII antigen in classical and variant von Willebrand disease are not due to qualitatively abnormal molecules but rather represent quantitative shifts in the metabolism of normal Factor VIII antigen molecules.

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Section: Methodssupporting

confidence: 63%

“…Trace amounts of VIII:Ag in these patients also lack the larger, less anodal molecular forms (7). (4,5,9). Recent studies suggest that the abnormality in von Willebrand disease is due to an abnormal distribution of polymeric forms of VIII:Ag (10).…”

mentioning

confidence: 99%

Structural analysis of factor VIII antigen in von Willebrand disease.

Nachman¹,

Jaffe²,

Miller³

et al. 1980

Proc. Natl. Acad. Sci. U.S.A.

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Neonatal von Willebrand's Disease: Diagnostic Difficulty at Birth

Weinger¹

1980

Arch Pediatr Adolesc Med

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disease (vWD) is an inherited disorder of bleeding, which is characterized by easy bruisability, mucosal hemorrhages, excessive posttraumatic bleeding, prolonged skin bleeding times, and quantitative or qualitative defects of the factor VIII molecular "complex." von Willebrand's disease and the structure and physiology of the factor VIII molecule have recently been reviewed.1-3We studied a 7-month-old affected male infant whose father and paternal grandfather were known to have vWD. Because of his family history, the child's plasma was studied at birth, at 6 months of age, and after the intravenous (IV) administration of cryoprecipitate. Our findings emphasize the difficulty in establishing the diagnosis of vWD in the immediate postnatal period.Report of a Case.\p=m-\Thepatient is a 7\ x =r eq-\ month-old boy, product of an uncomplicated 40-week gestation, born to a 19\ x=r eq-\ year-old, blood group 0, Rh-positive, gravida 1, para 1, abortus 0, hemostatically normal woman. Labor was induced with IV

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