Early diagnosis of thrombotic microangiopathy by paraffin sections of aspirated bone-marrow.
نویسندگان
چکیده
Thrombotic microangiopathy, particularly the type affecting adults and usually known as thrombotic thrombocytopenic purpura, has until recently been regarded as an almost invariably fatal disease. In infancy and early childhood it is generally described as the haemolytic-uraemic syndrome. However, the latter syndrome may result from more than one pathological process, and certainly it is unknown what proportion of children with haemolysis and uraemia have microangiopathic lesions; but about half of these patients survive with modern treatment (Gianantonio, Vitacco, Mendilaharzu, Rutty, and Mendilaharzu, 1964; Shinton, Galpine, Kendall, and Williams, 1964; McQuiggan, Oliver, Littler, and Cerny, 1965; McLean, Jones, and Sutherland, 1966). By contrast, the number of adult patients with thrombotic microangiopathy who have recovered has been small; Amorosi and Ultmann (1966) in an exhaustive review of 271 reported cases found only 13 survivors of 'classic TTP' (thrombotic thrombocytopenic purpura). Allanby, Huntsman, and Sacker (1966) have recently reported a further case of thrombotic microangiopathy in an adult, treated and apparently cured by the use of a magnesium-heparin-corticosteroid regime, and we have recently been impressed by the rapid and apparently complete recovery (Case 2 below) of a boy of 15 with this disease, who was treated by the same method in Bristol. It is probable that the very prompt institution of this treatment played a part in the successful outcome, and it seems that a definitive diagnosis of thrombotic microangiopathy at the earliest possible moment in suspected cases has become a matter of considerable importance. Thrombotic microangiopathy is not a common disease, and early diagnosis depends upon recognizing a combination of haemolytic anaemia, purpura, renal impairment, neurological disturbances, and frequently of fever; but to make the diagnosis with
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 42 222 شماره
صفحات -
تاریخ انتشار 1967