Alexander's syndrome (Benjamin Alexander)

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A congenital disorder of both sexes with onset in childhood or adult life. Like haemophilia but less severe.

Description

A congenital disorder of both sexes with onset in childhood or adult life. Like haemophilia but less severe. Deficiency of factor VII (serum prothrombin conversion accelerator) results in hemophilia-like hemorrhagic diathesis with epistaxes, deep muscular hematomas, and internal hemorrhages. Frequency 1:500.000. Probably transmitted as an autosomal recessive trait.

Bibliography

  • B. Alexander, R. Goldstein, G. Landwehr, et al:
    Congenital SPCA deficiency. A hitherto unrecognised coagulation defect with hemorrhage rectified by serum and serum fractions.
    Journal of Clinical investigation, New York, 1951, 30: 596-608.

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