Hemorrhagic Diathesis

HEMORRHAGIC DIATHESIS, a term that formerly encompassed all diseases in which the main symptom or one of the main symptoms is bleeding; the principal ones among them are hemophilia, scurvy, and various forms of purpura. These diseases were called hemorrhagic diatheses based on the assumption that all of them are based on a predisposition to a specific pathological phenomenon (see Diatheses), in this case - to bleeding. At present, the grouping of all these diseases into one category of hemorrhagic diathesis has become unnecessary, since individual pathological forms that have nothing in common with each other either etiologically or pathogenetically, and are even little similar in clinical picture, are gradually being separated from this group. Thus, for example, scurvy has been completely separated from this group as an avitaminosis. Among the individual forms that were classified as hemorrhagic diatheses, scurvy (already mentioned by Hippocrates) and hemophilia, for which indirect indications are already found in the Talmud, have been known for the longest time; more definite information about it is found in the works of the Arab physician of the 11th century, Alsagarawi. In the 30s of the 17th century, Werlhof described a new form of hemorrhagic diathesis, named after the author (see Werlhof's disease). Subsequently, follow 'purpura s. peliosis rheumatica' (Schönlein, 40s of the 19th century), 'purpura abdominalis' and 'purpura fulminans' (Henoch, last quarter of the 19th century; see Purpura), 'hemorrhagic aleukemia' - a term introduced by Frank in 1915 for cases previously described as aplastic anemia (see Aleukemia), fibropenia. The first case of this form was described by Rabe and Salomon in 1920. Along with the named forms, the literature has long included names such as 'purpura simplex', 'purpura cachectica', 'purpura senilis', etc. Despite the abundance of named forms, descriptions of cases that do not fit existing classifications appear in the literature. Just as often, in connection with the growth of our knowledge about the etiology and pathogenesis of hemorrhagic diathesis, different authors propose new names for already known pathological forms: 'essential thrombocytopenia' by Frank, 'anaphylactoid purpura' by Glanzmann, his 'hemorrhagic thrombasthenia', forme fruste of Werlhof's disease (Levit and Malkova). All this sufficiently proves that the question of hemorrhagic diathesis has been studied far from fully, although in recent years one can note a significant growth of our knowledge in this direction. In the history of the development of the doctrine of hemorrhagic diathesis, it is interesting to note that as early as the first half of the 19th century, individual forms were quite correctly distinguished and correctly classified. Later, on the contrary, the view became extremely widespread that different forms of purpura are nothing but various gradations of the same disease, and some authors went so far as to consider all types of hemorrhagic diathesis as one and the same disease. As for scurvy and hemophilia, such a view did not gain wide acceptance, but as for all other forms of hemorrhagic diathesis, it was almost universally accepted until recently. Now in this respect they are returning again to the views of the old authors, and it is necessary to note that this was done earlier in other countries in France, where, thanks to the work of the school of Hayem (Naquet), Werlhof's disease received its clear formulation as early as the end of the 19th century. It is no longer possible to confuse the latter with other forms of purpura at present. The next step in this direction was the isolation of anaphylactoid purpura and forme fruste of Werlhof's disease. Thanks to the establishment of these latter forms, we can at present assign the overwhelming majority of cases of purpura either to anaphylactoid purpura or to Werlhof's disease (essential or secondary). But it must still be admitted that if scurvy (and its childhood form - Barlow's disease, see), hemophilia and Werlhof's disease at present appear to be quite strictly and fully outlined and mutually demarcated, this cannot yet be said regarding other forms of hemorrhagic diathesis.