Thrombocytopathy and transiltory forms between thrombocytopathy and thrombocytopenia.
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Blood Platelet Disorders↗
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A new approach to the thrombocytopathies; thrombocytopathy A.
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Blood Platelet Disorders↗
[Management of patients with coagulation disorder in oral and maxillofacial surgery. I. Management of patients with hypocoagulation caused by primary thrombocytopathy].
Any oral and maxillo-facial surgical treatment, however urgent it may be, must not include pathological states in which the patient's life may be particularly at risk as, for example, with Disseminated Intravascular Coagulation (DIC) or throm-botic thrombocytopenic purpura. In this article the authors present the result of studies carried out on the nosology of thrombocytopathy from an odontostomatological point of view. Thrombocytopathy can be divided into two groups: the first including the pathologies with a predominant defective number of thrombocytes (i.e.: thrombocytopenia, thrombocythemia, thrombocyto-sis), the second including forms with predominant qualitative defects (commonly known as thrombocytopathies). The authors, after having presented in short the physiopathologic functions of thrombocytes, illustrate the clinical and therapeutic aspects of the most important thrombocytopathies. Morbus Maculosus Werhofii, Glanzmann's disease, Bernard-Soulier syndrome, thrombocytopathies from defective reaction of release, Thrombocytopathies from defective procoagulant activity of blood plaques, thrombocytopathies in linkage to other genetic anomalies, von Willebrand's pseudodisease and a lot of acquired thrombocytopathies are identified. In the last part the authors illustrate the most opportune clinical steps corresponding to the most important thrombocytopathies. The results obtained suggest the necessity of keeping to the management that was described, Actually a low percentage of accidents occurred only when the above-mentioned clinical processes were completely performed.
Blood Loss, Surgical↗
Hereditary thrombocytopathy: a familial bleeding disorder due to impaired platelet coagulant activity.
A family with a bleeding disorder due to congenital thrombocytopenic thrombocytopathy is described, with ten affected members in three generations. The disorder is inherited as an autosomal dominant trait and is characterized by thrombocytopenia, morphologically abnormal platelets, prolonged bleeding time, platelet coagulant activity deficiency and abnormal platelets, prolonged bleeding time, platelet coagulant activity deficiency and abnormal platelet aggregation. Patients' platelets adhered to collagen, but aggregation was reversible and the release of platelet constituents was minimal. Aggregation with ADP was similarly reversible, but the platelet response to thrombin was normal. These defects in platelet aggregation and release were not corrected by addition of normal plasma indicating an intrinsic abnormality of platelets. By definition thrombocytopathy consists of a deficiency in platelet coagulant activity. It was shown that the deficiency of platelet coagulant activity caused a delay and decrease in the conversion of prothrombin to thrombin, and it is proposed that the lack of thrombin accounts for the defective release reaction and the reversible aggregation. An adequate haemostatic plug due to decreased release of ADP, together with instability of the plug provide an explanation for the bleeding tendency in thrombocytopathy.
Adenosine Diphosphate↗
[Factor VIII (von Willebrand antigen) in patients with acquired thrombocytopathies].
Factor VIII/von Willebrand antigen (VA), part of the molecule of plasma factor VIII, realizes the interaction between platelets and vascular endothelium and the triggering of primary hemostasis. The modern diagnostics and treatment of the complicated acquired thrombocytopathies are impossible without the investigation on the concentration of factor VIII/von Willebrand antigen. The immune coagulation method used allows the objective, exact and fast determination of VA--referent values have been developed in healthy subjects. The patients with blastic leukosis studied--28 and with chronic myeloleukemia--18, all with severe endogenous complicated thrombocytopathy, functionally and biochemically confirmed, showed normal values of VA/von Willebrand antigen. On the contrary, a slightly elevated VA was established in patients with diabetes with no vascular-degenerative syndrome, corresponding to the activation of platelet functions and to enhanced adhesiveness in particular, contributing to thrombotic complications. The data obtained are discussed in connection with the etiopathogenesis of the separate kinds of thrombocytopathies and the necessity of substitutive therapy.
Acute Disease↗
Is lower ploidy of megakaryocytes another reason for uremic thrombocytopathy?
Though it has been generally accepted that uremic serum contains substances which impair platelet function and inhibit erythropoiesis, the mechanism of uremic thrombocytopathy and bleeding tendency is still not clearly understood. By measuring the nuclear DNA content of the megakaryocytes of 20 patients with end-stage renal failure, we demonstrated that ploidy levels correlate with parameters of uremia. High creatinine and BUN levels as well as low hemoglobin or creatinine clearance values correlate with low average ploidy of megakaryocytes. In explaining this phenomenon, uremic inhibition of DNA replication seems to be more important than dialysis or the type of renal disease. As megakaryocytes of higher ploidy are thought to produce more reactive platelets than megakaryocytes of lower DNA content, this could be another reason for uremic thrombocytopathy and bleeding tendency.
Adult↗
Thrombocytopenia with thrombocytopathy possibly related to abnormalities of intracellular Ca++ fluxes and followed by the development of leukaemia.
A patient is described who presented a thrombocytopenia with thrombocytopathy followed by the development of a leukaemia. The disorder was characterized by decreased aggregation in the presence of ADP, and a lack of aggregation in the presence of arachidonic acid, natural endoperoxide or collagen. In parallel, 14C-serotonin release was severely decreased or nil in response to these inducers. Thrombin induced a slightly decreased aggregation and a normal 14C-serotonin release. Thromboxane B2 (T X B2) synthesis was normal after stimulation by arachidonic acid, natural endoperoxide or thrombin showing a normal arachidonate metabolism. In addition, the mepacrine test showed no significant decrease of the number of dense bodies with an average of 4.6 per platelet (versus 5.4 +/- 0.8 sd in controls). Stimulation by ionophore A 23187 failed to induce aggregation, 14C-serotonin release, or T X B2 synthesis. Furthermore, in the presence of EDTA, A 23187 did not provoke activation as reflected by 14C-serotonin release or T X B2 synthesis. Thus, in this case of thrombocytopathy, the hypothesis of abnormal intracellular Ca++ fluxes responsible for the defective platelet release phenomenon, was suggested.
Adenosine Diphosphate↗
Thrombocytopathy due to a defect of the platelet membrane complex.
A new type of primary thrombocytopathy is described. Three main alterations were found: (1) a defect of the aggregation reaction with ADP, epinephrine and collagen and a normal response to ristocetin and arachidonic acid; (2) a moderate deficiency of platelet procoagulant activity, and (3) a combined hypertrophy of the two membrane systems of the platelet--the open canalicular and the dense tubular. The latter defect is shown as an abnormal membrane complex situated on one of the platelet poles. This thrombocytopathy is discussed as a new variety of primary platelet disorder.
Adult↗
Association of the hemophilia A carrier state and hemorrhagic thrombocytopathy with dilatation of the platelet membrane complex.
Associations of hereditary abnormalities of the factor VIII complex and hereditary platelet disorders have previously been reported in 12 families. Another family is reported in which 6 members had a bleeding tendency and thrombocytopathy characterized by impaired platelet aggregation and dilatation of the platelet membrane complex. Apart from the platelet function abnormalities the proband had diminished levels of factor VIII clotting activity (36 U/dl) and factor VIII clotting antigen (31%) while factor VIII-related antigen and ristocetin cofactor were normal. The other affected family members had normal levels of factor VIII:C. Consequently, the proband was defined as a hemophilia A carrier manifesting also hereditary thrombocytopathy.
Adult↗
[Thrombocytopenia and thrombocytopathy in neonates and infants].
The authors analyze seven cases of thrombocytopenia and three cases of thrombocytopathies in neonates and infants who were hospitalized within a short period of time at the First Paediatric Clinic in Brno. The results support the view that thrombocytopenia and thrombocytopathies in neonates and infants are fairly frequent. Because they are not manifested by clinical symptoms, their frequency escapes attention.
Blood Platelet Disorders↗
[Hemorrhage caused by thrombocytopathies: new aspect of treatment (author's transl)].
Hemorrhage due to thrombocytopathies can in most cases be stopped by application of the homologous coagulation-active phospholipide complex Fibraccel, as shown in a study in which 78 patients suffering from various thrombocytopathies were checked. This effect is especially important in patients with immunothrombocytopathies which do not permit any thrombocyte substitution. Recalcification time and thromboelastogram should be normalised during Fibraccel treatment. In existing extravascular coagulation Fibraccel treatment is contraindicated, especially in cases of inadequate protection by heparin.
Blood Coagulation Tests↗
Congenital release thrombocytopathy: pathophysiology and management.
Congenital release thrombocytopathy must be included in the differential diagnosis of bleeding diatheses in women. A review of the coagulation profiles of 7 patients with congenital release thrombocytopathy suggests that more sophisticated platelet aggregation studies must be performed when routine screening procedures fail to determine the cause of a clinically suspected bleeding disorder. Establishing the diagnosis helps to avoid the use of medications which cause an acquired platelet dysfunction, and contributes to adequate prophylaxis against surgical and obstetric hemorrhage.
Adenosine Diphosphate↗
Macular cherry-red spots and beta-galactosidase deficiency in an adult. An autopsy case with progressive cerebellar ataxia, myoclonus, thrombocytopathy, and accumulation of polysaccharide in liver.
An adult patient with macular cherry-red spots, a gargoyle-like physical appearance, cerebellar ataxia, myoclonus, convulsive seizures, and pyramidal tract signs showed a profound deficiency of beta-galactosidase in liver and brain. Thrombocytopathy of undetermined etiology was evident since childhood, and the patient died of intracranial bleeding at age 22. Cerebral ganglioside pattern was normal. Hepatic mucopolysaccharides were not increased. GM1-gangliosidosis and mucopolysaccharidosis were ruled out by those analytical data. However, a large amount of amylopectin-like polysaccharide was found to be accumulated in liver. Hepatocyte contained numerous inclusion bodies with granulofibrillary structure similar to Lafora bodies, corpora amylacea, and inclusion bodies in glycogenosis type IV. This case seems to represent a new inborn metabolic disease closely related to GM1-gangliosidosis and mucopolysaccharidosis. The primary metabolic defect is not known at present.
Adult↗
Type IIB von Willebrand's disease associated with a complex thrombocytopenic thrombocytopathy.
A familial bleeding disorder characterized by an association of Type IIB von Willebrand's disease (vWD) with a complex thrombocytopenic thrombocytopathy is described in two patients from the same generation. Findings typical of type IIB vWD included enhanced ristocetin-induced binding of patient von Willebrand factor (vWF) to platelets of patients and normal individuals in association with the absence of larger multimers from plasma. Abnormalities in platelet function included deficient platelet aggregation to ADP, collagen, epinephrine, and arachidonic acid; and defective release of 14C-serotonin, vWF, and platelet factor 4 (PF4) in response to thrombin, collagen, or ADP. Platelet factor 4 and platelet vWF were decreased when measured per mg of total platelet protein. In addition, the binding of normal vWF to patient platelets stimulated with thrombin was decreased. Platelet size was increased with a very heterogeneous distribution width. Electron microscopic evaluation showed giant platelets with dense and alpha bodies present. The platelet count was borderline or slightly decreased in the resting state and declined to frankly thrombocytopenic levels at the time of acute bleeding episodes; this state was associated with the presence of platelet aggregates in blood smears.
Antibodies, Monoclonal↗
Diagnosis of von Willebrand's disease. A comparative study of diagnostic tests on nine families with von Willebrand's disease and its differential diagnosis from hemophilia and thrombocytopathy.
Nine probands with von Willebrand's disease, and their family members, totalling 43 people, were examined. Twenty-seven had a history of bleeding; 29 had an increased factor VIII activity:factor VIII related antigen ratio; 24 had a decreased factor VIII related antigen; 23 had a prolonged bleeding time; 19 had a reduced platelet adhesiveness; 16 had a decreased factor VIII activity; and 14 had an abnormal ristocetin-induced platelet aggregation. Eight members with both normal beleeding time and normal factor VIII activity were found to have other abnormal tests: elevated ratio of factor VIII activity to factor VIII related antigen in seven; decreased factor VIII related antigen in four; and reduced platelet adhesiveness in one. Therefore, ratio of factor VIII activity to factor VIII related antigen and factor VIII related antigen are more sensitive and may be used for the detection of heterozygous carriers of von Willebrand's disease. Although patients with thrombocytopathy may have a prolonged bleeding time, decreased platelet adhesiveness and reduced platelet aggregation by ristocetin, their factor VIII activity, factor VIII related antigen and ratio of factor VIII activity to factor VIII related antigen are normal and their abnormal ristocetin test cannot be corrected by the addition of factor VIII concentrate. Hemophilic subjects and hemophilic carriers, who are deficient in factor VIII activity, usually have a normal bleeding time, normal platelet adhesiveness, and normal ristocetin test. In contrast to patients with von Willebrand's disease, their factor VIII related antigen is normal or slightly increased and their ratio of factor VIII activity to factor VIII related antigen is significantly reduced. We conclude that ratio of factor VIII activity to factor VIII related antigen and factor VIII related antigen are not only more sensitive but also more specific for the diagnosis of von Willebrand's disease.
Antigens↗
Circulation and hemostatic function of autologous fresh, liquid-preserved, and cryopreserved baboon platelets transfused to correct an aspirin-induced thrombocytopathy.
BACKGROUND: The survival of fresh and preserved platelets has been used primarily to determine their therapeutic effectiveness. The function of the fresh and preserved platelets has been difficult to assess. In stable thrombocytopenic patients, platelet function of fresh and preserved allogeneic platelets is evaluated by the reduction in bleeding time. In this study of healthy male baboons, both the survival and function of autologous fresh, liquid-preserved, and cryopreserved platelets in the correction of an aspirin-induced thrombocytopathy was evaluated. STUDY DESIGN AND METHODS: Five healthy male baboons were studied on eight occasions over a 4-year period. To produce a prolonged bleeding time, the baboon was administered 325 mg of aspirin 18 hours before receiving autologous transfusion. The fresh, liquid-preserved, and previously frozen washed platelets were labeled with (111)In-oxine before autologous transfusion. The autologous, nonaspirinated platelets' ability to reduce the aspirin-induced prolonged bleeding time and increase the shed blood thromboxane B2 level at the template bleeding time site was studied. RESULTS: Platelets stored at 22 degrees C for 48 hours had in vivo recovery values similar to those platelets stored for 18 hours, and they significantly reduced the bleeding time and increased the shed blood thromboxane level after transfusion. Platelets stored at 22 degrees C for 72 hours had in vivo recovery values similar to those platelets stored for 18 hours, but the bleeding time was not corrected after transfusion, although there was a significant increase in the shed blood thromboxane B2 level. The cryopreserved platelets significantly reduced the bleeding time and significantly increased the shed blood thromboxane level after transfusion. Cryopreserved platelets had better in vivo survival and function than the 5-day liquid-stored platelets. CONCLUSIONS: The survival of autologous fresh, liquid-preserved, or cryopreserved platelets did not correlate with their function to reduce an increased bleeding time in baboons treated with aspirin.
Animals↗
A study on the efficacy of a phospholipid solution in dogs with aspirin-induced thrombocytopathy.
The aim of the present study was to test the haemostyptic properties of a phospholipid complex with platelet factor-3 (PF-3) activity in platelet-dependent haemostatic disorders. The substance was investigated in an animal model comprising six investigational groups (five dogs each). A thrombocytopathy was created in the dogs belonging to groups 1-5 by intravenous administration of 20 mg/kg body weight (BW) acetylsalicylic acid (ASA). Two hours later a human albumin solution (5%) (control group) or a phospholipid complex with PF-3 activity was injected or infused intravenously in different dosages. In dogs pre-treated with ASA, injection of 2 ml/kg BW of the phospholipid complex shortened capillary bleeding time, which was prolonged as a consequence of ASA-treatment. This effect lasted for 4 h at least. The capability of the platelets to aggregate increased 5 min after intravenous injection of the phospholipid without differences in the respective groups. At the same time platelet counts dropped to approximately 50%, but increased again distinctly after 30 min. When the phospholipid complex was administered to clinically healthy dogs who had not been treated with ASA, this resulted in prolongation of the capillary bleeding time as well as a significant platelet drop in the peripheral blood. Although these were only short-term effects after administration of the phospholipid complex, a disadvantageous effect cannot be excluded in patients suffering from haemorrhagic diathesis. For this reason, a phospholipid complex with PF-3 activity cannot be recommended as a therapeutic agent for platelet-dependent coagulation disorders in the dog.
Animals↗
A new type of mucolipidosis associated with hereditary thrombocytopathy and color blindness.
Autopsy findings of a 22-year-old Japanese male who showed the symptoms of both mucopolysaccharidosis and sphingolipidosis are reported. The patient had a gargoyle-like face, bone change with cherry-red spot and absence of mucopolysacchariduria, and moreover accompanied by hereditary thrombocytopathy and color blindness. Autopsy findings were almost the same as those of mucopolysaccharidosis, histochemically and electron microscopically. Unique findings were, however, present in the hepatocytes, another inclusion containing dense fine granuloreticular structures was found electron microscopically. Some foamy cells in the lymph nodes, liver including sinusiodal cells, bone marrow and spleen contained intracytoplasmic sudanophilic substance in the form of moderate electron dense globules by electron microscopy. The outstanding finding of the enzymatic activity was the decrease of beta-galactosidase in the liver and brain.
Adult↗