After a critical study, a splenectomy was performed in a 6‐year‐old boy with chronic thrombocytopenic purpura.
Failure of surgery and immunosuppressive therapy prompted new investigations which led to the discovery of a cyclic thrombocytopenic purpura related to a periodic variation in maturity of megakaryocytes.
The patient's platelets were morphologically and functionally normal and it was not possible to demonstrate neither immunological mechanism, nutritional deficiency, influence of the environment nor consumption or excessive destruction of platelets. Cyclic thrombocytopenia was detected in the father and also cyclic variations in platelet counts from normal values to over 1,000 times 109/1 in 4 of 9 siblings. In view of these findings. The abnormal condition in this family was named Garcia's disease.
Platelets from a group of 24 malnourished children with purpura, and a group of 28 clinically healthy adults and 5 normal children, were studied with determination of platelet mean diameter, soluble protein, lactate concentration and platelet viscous metamorphosis.
The data obtained from platelet rich plasma in malnourished children showed an increase in lactate concentration and an inability to increase its lactate when thrombin was added; an abnormal viscous metamorphosis was found also in some patients, due to either a plasma or a platelet abnormality and also in a third group of patients to the presence of both defects.
The abnormal PC in BSS is a defect that has not been extensively studied to date. For this reason 4 patients with BSS who had normal platelet aggregation tests (except with bovine fibrinogen and with Ristocetin), but abnormal bleeding times and PC on different occasions, during which they showed variable thrombocytopenia, were studied. The PC was normal in only one case and in only one occasion, which was coincidental with a circulating platelet count that also approximated normal.The abnormal PC in the 4 cases was corrected when platelet substitute, or washed platelets from normal subjects or from patients with BSS were added. These same platelets also corrected the abnormal PC in cases of ITP or aplastic anemia, and shortened the recalcification time of platelet-poor plasmas from normal subjects and patients with specific plasma deficiencies (Factors V, VIII and XI).The above suggests that the abnormal PC in some cases of BSS may be due to the thrombocytopenia rather than to a defect in the procoagulant activity of the platelets in this syndrome.
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