Platelet Storage Pool Deficiency and Prostaglandin Synthesis in Chronic Granulocytic Leukaemia

Abstract

Platelet function was evaluated in eight patients with chronic granulocytic leukaemia (CGL), seven Ph1 positive and one Ph1 negative. Seven of the eight patients' platelets had an absence of the second wave of adrenaline induced aggregation on at least one occasion, while five had impaired collagen aggregation. The platelets of all seven patients with abnormal responses to adrenaline, aggregated with arachidonic acid, thus ruling out a cyclo-oxygenase deficiency. A marked decrease in the ADP, serotonin, and dense body content of platelets was found in all five patients evaluated. Mixtures of CGL patient platelets with platelets from normal donors who had ingested aspirin gave a normal biphasic response to adrenaline. Normal release of the storage pool contents from aspirin treated platelets was shown by stirring a mixture of CGL platelets and 14C-serotonin labelled aspirin treated platelets with adrenaline. The CGL platelets alone or in the mixture produced malondialdehyde in response to adrenaline. These experimental results suggest that CGL platelets have a storage pool deficiency but can synthesize prostaglandins and thromboxanes in response to arachidonic acid and adrenaline.

Generation of Arachidonic Acid Metabolites from Stimulated Whole Blood in Patients with Chronic Myeloproliferative Disorders

“…The normal thromboxane generation in patients with CML is in good agreement with previous data showing normal malondialdehyde synthesis in this category of patients [20]. On the contrary, CML pa tients showed a striking reduction in both i-6-keto-PGF,n and i-PGE2 production.…”

Reduced Platelet Factor X-Activating Activity

“…The marked reduction o f total platelet serotonin content we found in most patients is in agreement with previous observations (5,22,36) and consistent with the concept o f acquired 'storage pool deficiency' in myeloproliferative dis orders (14,26). Since this defect was present in all but 3 patients it can be regarded as a marker of myeloproliferative disease, entirely unrelated to the haemostatic complications.…”

Platelet Abnormalities in Thermal Injury

“…The same platelet abnormalities have already been described in various other acquired affections, namely, idiopathic thrombocytopenic purpura (ITP) (24), disseminated intravascular coagulation (12), and myeloproliferative disorders (4,17). Previous studies of platelet-dense bodies in congenital SPD have been carried out by Rendu et al (16,18) using mepacrine as a staining agent for the dense bodies (DB).…”