[Adult-type agammaglobulinemia with manifestations of funicular myelosis due to vitamin B 12 deficiency].
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The activity of platelet factor 4 (FP4) was examined in 37 patients affected with blastic leukaemia, in 16 patients with blastic crisis in chronic myeloid leukaemia and in 2 patients with Willebrand's syndrome. The real activity of FP4 determined by modifying the method according to NIEWIAROWSKI after a complete lysis of granular membrances by means of triton X-100 was found to be lowered in 9 patients affected with blastic leukaemia, in 5 patients with blastic crisis and in two patients with Willebrand's syndrome. Presuming that a maximal FP4 release of the irreversible platelet aggregation must be obtained, which corresponds to the real activity, the author has examined the apparent activity of FP4 released from the aggregated platelets with the help of her own method. In this way the quality of the release reaction from the platelets can indirectly be characterized with their extremely important role for haemostasis. Whereas in exacerbated myeloid leukaemia and Willebrand's syndrome the apparent activity will correspond to the real one, there is a severe specific disturbance of the platelet release response in blastic leukaemia. The platelet aggregation is incomplete caused by the derailment of the energy metabolism and the disturbance of the adenine nucleotides, thus causing an apparent as well as a real FP4 deficiency which can be brought into the same line with pathogenesis of thrombopathy in blastic leukaemia.
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