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PubMed1985

Haemostasis in children: anything new?

Our understanding of haemostatic mechanisms has increased in the last decades. This knowledge at the cellular and molecular levels has helped us to appreciate the complexity of haemostatic mechanisms and their disturbance in various disorders. The advance in techniques to measure early activation of coagulation and platelets opens hope of treating acquired bleeding disorders e.g., disseminated intravascular coagulation before the full blown picture. More study needs to be done to define what constitutes normal haemostasis in newborns. The better understanding of control mechanisms of haemostasis will help us in diagnosis and treatment.

Antithrombin III↗
PubMed1994

[Simultaneous occurrence of lupus anticoagulant and acquired "storage pool" disease of thrombocytes].

The authors describe the case of a female patient with a history of simultaneous abortion and deep vein thrombosis as well as moderate bleeding disturbances. Investigations revealed the presence of lupus anticoagulant while a thrombocyte "storage pool" disease was confirmed. This case demonstrates the association of these two haemostatic disturbances, and points to a possible relationship between them. Since detailed analyses failed to demonstrate the presence of antiplatelet antibodies, the authors suggest a possible damaging effect of lupus anticoagulant to the endothelium leading to thrombocyte activation.

Abortion, Spontaneous↗
PubMedDate not supplied

[Utility of desmopressin in 4 cases of thrombocytopathies associated with giant platelets].

Shear-induced aggregation requires the platelet glycoprotein complexes (Gp), the von Willebrand factor (vWf) and ADP. The Bernard Soulier syndrome (BS) and the gray platelet syndrome (GPS) are platelet function defects characterized by absence of GP Ib/IX and alpha granules, respectively, with mucocutaneous hemorrhages, prolonged bleeding time (BT) and moderate thrombocytopenia in both syndromes. There are reports that desmopressin (DDAVP) shortens the BT in some patients with platelet dysfunction. The purpose of this study was to evaluate the response t(DDAVP) in four female patients (2 with GPS plus Marfan's disease and 2 BS). All had bleeding episodes, BTs > 10 minutes, platelet counts (PC) between 40-88 x 10(9)/L and defects in platelet aggregation. The DDAVP was administered at a dose of 0.3 microgram/kg in 15 to 30 mL of isotonic saline given by slow intravenous drip in 30 to 45 min. All patients were studied before and after DDAVP administration (BT, PC, platelet factor, mean platelet volume, factors F.VIII:C, FvW:Ag, FvW:RiC of, and platelet aggregation). After DDAVP infusion the patients had a BT < 6 min, and increased levels of F. VIII:C, FvW:Ag and FvW:RiC of (> 100 Ul/dL), and the bleeding disappeared. We conclude that there was a good response to DDAVP probably associated with improved platelet adhesion, and increases in the multimers of the von Willebrand factor.

Adolescent↗
PubMed1998

Long-term effects of erythropoietin on platelet serotonin storage and platelet aggregation in hemodialysis patients with reference to ketanserin treatment.

Correction of uremic platelet serotonin (5-HT) storage pool deficiency is one of the very early hemostatic effects of erythropoietin (Epo) therapy. In this work, platelet 5-HT with relation to primary hemostasis was studied in 15 hemodialysis patients treated with Epo for 8 months. Moreover, effects of ketanserin, a blocker of platelet and vascular smooth muscle cell 5-HT2A receptors, in these patients were followed. The parameters studied were compared with relevant values in healthy controls and in hemodialysis patients not treated with Epo, and remeasured in the long-term Epo patients after a 14-day oral ketanserin trial. Platelet 5-HT content in the eighth month of Epo therapy was not different from the one in untreated patients. Ristocetin- and collagen-induced platelet aggregation were enhanced in comparison with both control groups, as opposed to unaltered response to ADP and arachidonic acid. Fibrinogen concentration was lower than in the untreated group. An inverse correlation between ADP-induced platelet aggregation and the skin bleeding time (r=-0.536, p<0.05) and a positive one between the former and platelet 5-HT (r=0.644, p<0.01) were found. Platelet count correlated positively with both platelet 5-HT (r=0.823, p<0.0002) and ADP-induced platelet aggregation (r=0.596, p<0.02). Ketanserin produced a decrease in ristocetin-induced platelet aggregation, fibrinogen, and prolongation of the bleeding time. The first two of the changes correlated positively with their pre-ketanserin values (r=0.923, p<0.00001 and r=0.839, p< 0.0001, respectively). Post-ketanserin, positive correlations between depressed ristocetin- and arachidonic acid-induced platelet aggregation (r=0.760, p<0.005), and between collagen- and corresponding values of arachidonic acid- (r=0.622, p<0.02), ADP-induced platelet aggregation (r=0.396, p<0.01), and platelet 5-HT (r=0.654, p<0.05) were found. Efficient hemostasis in hemodialysis patients on protracted Epo therapy is, in part, dependent on enhanced platelet aggregability. Correction of platelet 5-HT storage pool deficiency is not evident in this stage but 5-HT still influences complex mechanisms of primary hemostasis. Ketanserin is of anticoagulant value in these patients but its effects must be weighted against possible exacerbation of the anemia.

Adolescent↗
PubMed1988

Storage pool deficiency in cattle with the Chédiak-Higashi syndrome results from an absence of dense granule precursors in their megakaryocytes.

Platelets from cattle with the Chédiak-Higashi syndrome (CHS) have a storage pool deficiency and virtual absence of platelet dense granules. Megakaryocytes (MKs) from five control (n = 135) and five CHS (n = 133) cattle were evaluated using standard transmission electron microscopy. Osmiophilic dense granules were not observed in control or CHS MKs. In MKs from normal cattle, clear vesicles of 200- to 650-nm diameter bounded by a sharp membrane were observed. They were easily differentiated from the demarcation membrane system, endoplasmic reticulum, and alpha granules. The clear vesicles were virtually absent in MKs from CHS cattle at all stages of maturation. MKs in bone marrow samples from two control (n = 91) and two CHS (n = 61) cattle that had been processed for the uranaffin reaction were also evaluated. The clear vesicles were replaced by uranaffin-positive granules in MKs from control cattle, but positive uranaffin granules were not observed in CHS MKs. These findings indicate that the platelet dense granule storage pool deficiency in CHS cattle results from an anatomic absence of dense granule precursors in maturing and mature CHS MKs.

Animals↗
PubMed1998

Mutation in AP-3 delta in the mocha mouse links endosomal transport to storage deficiency in platelets, melanosomes, and synaptic vesicles.

The mouse mutant mocha, a model for the Hermansky-Pudlak storage pool deficiency syndrome, is characterized by defective platelets, coat and eye color dilution, lysosomal abnormalities, inner ear degeneration, and neurological deficits. Here, we show that mocha is a null allele of the delta subunit of the adaptor-like protein complex AP-3, which is associated with coated vesicles budding from the trans-Golgi network, and that AP-3 is missing in mocha tissues. In mocha brain, the ZnT-3 transporter is reduced, resulting in a lack of zinc-associated Timm historeactivity in hippocampal mossy fibers. Our results demonstrate that the AP-3 complex is responsible for cargo selection to lysosome-related organelles such as melanosomes and platelet dense granules as well as to neurotransmitter vesicles.

Adaptor Protein Complex 3↗
PubMed2001

Acquired platelet dysfunction with eosinophilia in children in the south of Thailand.

One hundred and sixty-eight children aged 13 months to 12.6 years with acquired platelet dysfunction with eosinophilia (APDE) were studied. The male to female ratio was 1.15:1. All of the children were in good health and no history of any drug ingestion was detected. All of the children had widespread spontaneous bruising on the extremities, body and face off and on. Severe bleeding symptoms were detected in 8% of these patients. The number of platelets in these children was within the normal range but the platelet morphology was abnormal in all of them. Eosinophilia was detected in 86% of these children. Prolonged bleeding time was detected in 53% of these patients. Abnormal platelet adhesiveness was found in 33% of cases. Abnormal platelet aggregation induced by collagen was the most sensitive test in these patients. Abnormal ADP release from the platelets was detected in these patients by the absence of a second wave of aggregation during stimulation of PRP by ADP or epinephrine. Abnormal or no ATP secretion from the platelets during stimulation by ADP, epinephrine or collagen was detected in these patients. Ristocetin-induced platelet aggregation was normal in these children. Decreased or absence of platelet dense granules by TEM study was detected in some patients. These changes in platelet functions and morphology may be due to acquired storage pool deficiency of the platelet. Parasitic infection was detected in 56% of these children. About 83% of these children with APDE had serum total IgE higher than 100 IU/ml. There was no correlation between the number of eosinophils and serum total IgE and the severity of bleeding symptoms. The majority of children with APDE did not receive any treatment except those who had severe bleeding symptoms which required platelet concentrate to stop bleeding. In more than 90% of the patients, the bruising or ecchymosis disappeared within 6 months and the abnormal platelet functions returned to normal within 4 months. Recurrence of these bleeding syndromes was detected in 7% of the children.

Blood Coagulation Tests↗
PubMed1986

Platelet prothrombin converting activity in hereditary disorders of platelet function.

Prothrombinase activities of platelets have been measured in diluted platelet-rich plasma using a chromogenic substrate assay and purified coagulation factors. No abnormalities in prothrombinase activities were found for platelets from patients with storage pool disease (dense-body deficiency), grey platelet syndrome, and Glanzmann's thrombasthenia. It is concluded that neither release of dense bodies and alpha-granules nor aggregation of platelets are essential prerequisites for exposure of a procoagulant surface. Platelets from patients with Bernard-Soulier syndrome, however, have approximately 10-fold higher prothrombinase activities in the non-stimulated form than normal non-stimulated platelets. The increased procoagulant activity cannot be completely ascribed to an increase in platelet size. It is suggested that the increased prothrombinase activity reflects an increased exposure of phosphatidylserine at the outer surface of non-stimulated Bernard-Soulier platelets, earlier described by Perret et al (1983).

Bernard-Soulier Syndrome↗