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a term that describes a number of congenital or acquired pathological states in which the basic manifestation is an increased disposition to hemorrhages and apoplexies. The mechanism of development of these diseases depends on the state of the blood coagulation system, the thrombocyte system, and the blood vessels. Among the diseases associated with impairment of blood coagulation are hemophilia, hemolytic disease of the newborn, and certain other congenital and acquired diseases associated with an insufficiency of these components and also with increased fibrinolytic activity of the blood (diseases accompanied by insufficient formation of prothrombin with a deficiency of vitamin K, as a result of impairment of digestion or damage to the liver, with certain infectious diseases, with an overdose of anticoagulants, and so on).
Deficiency of the thrombocyte system also causes illnesses such as thrombocytopenic purpura (Werlhofs disease, named after the German doctor P. G. Werlhof who described it); hemorrhagic hereditary thrombasthenia, or Glanzmann’s disease (named after the German hematologist E. Glanz-mann); and angiohemophilia, or Willebrand’s disease (named after the Finnish hematologist E. A. Willebrand).
Hemorrhagic vasculitis is included among the hemorrhagic diatheses that occur owing to an increase in the permeability of the vascular wall, while the blood coagulation system remains unimpaired. The development of hemmorhagic diatheses resulting from damage to blood vessels is also observed in other diseases: Waldenstrüm’s macroglobulinemic purpura (named after the Swedish doctor J. Waldenstrom), scurvy, infantile scurvy (Moeller-Barlow disease, named after the German doctor J. Moeller and the English doctor T. Barlow), and various infections and intoxications.
Hemorrhagic vasculitis (capillary toxicosis), or Schönlein-Henoch disease (named after the German doctors J. L. Schönlein and E. H. Henoch, who described the illness) is characterized by pain in the abdomen, hemorrhagic skin rashes with itching, swelling of the joints, and pathologico-anatomic changes in the urine. The development of the disease is associated with increased permeability and brittleness of the blood vessels: of importance is sensitization of the organism to various allergens of both bacterial and nonbacterial origin.
A special form of hemorrhagic diathesis is hemorrhagic metropathy in young girls, which manifests itself in copious uterine bleeding. Hormonal impairments of the pituitary gland and ovaries form the basis of the disease. Hemorrhagic metropathy is encountered primarily during the period of sexual maturation in young girls.
The therapy for hemorrhagic diathesis depends on the form, manifestations, and course of the disease.
REFERENCESKassirskii, I. A., and G. A. Alekseev. Klinicheskaia gematologiia, 4th ed. Moscow, 1970.
Abezgauz, A. M. Gemorragicheskie zabolevaniia u detei. Leningrad, 1963.
Tur, A. F. Gematologiia detskogo vozrasta, 2nd ed. Leningrad, 1963.
R. N. RYLEEVA and M. IA. STUDENIKIN