Pseudohemophilia

PSEUDOPHEMOPHILIA, a collective concept for various forms of hemorrhagic diatheses. Some authors (Rabe, Salomon, Opitz, Frei) include cases of fibropenia—sporadic forms of bleeding with reduced or absent blood clotting, which is based on a deficiency of fibrinogen. In these cases, bleeding is a symptom of impaired fibrin-forming function of the liver. Frank introduces the term P. (pseudo-haemophilia hepatica) for cases of bleeding observed in various liver diseases (acute yellow atrophy of the liver, poisoning by phosphorus, chloroform, Weil's disease). Other authors (Levit and Malkova, Wilbrand) classify various hereditary forms of bleeding as P. Levit describes a case of mutation in humans, where bleeding from mucous membranes is noted as a dominant trait in four generations; both men and women are affected. Blood clotting, hemorrhagic syndrome (see Werlhoff's disease) show no deviations from normal. Wilbrand, upon re-examining his case of P., which he described in 1926 as a dominant, sex-linked trait, already in 1933 calls it constitutional thrombopathy. Treatment of P. is symptomatic.