RESUMO
INTRODUCTION: The sticky platelet syndrome (SPS) is a common cause of thrombosis. There are no prospective studies concerning treatment. OBJECTIVE: To analyze changes in platelet hyperaggregability of patients with SPS who were given antiplatelet drugs and to assess its association with rethrombosis. METHODS: A total of 55 patients with a history of thrombosis and SPS phenotype were prospectively studied before and after treatment with aspirin and/or clopidogrel. RESULTS: Patients were followed for 1 to 129 months, median 13. Of 55 patients, 40 received aspirin, 13 received aspirin + clopidogrel, and 2 received only clopidogrel. The platelet aggregation response to adenosine diphosphate and epinephrine significantly diminished after treatment, and only 2 patients developed rethrombosis 52 and 129 months after starting therapy, with the freedom from rethrombosis rate of the patients being 96.4% at 129 months. CONCLUSION: Using antiplatelet drugs, the platelet hyperreactivity of patients with the SPS phenotype was reverted; and this translated into a substantial decrease in the rethrombosis rate.
Assuntos
Transtornos Plaquetários/tratamento farmacológico , Trombofilia/tratamento farmacológico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aspirina/uso terapêutico , Transtornos Plaquetários/sangue , Transtornos Plaquetários/etiologia , Criança , Clopidogrel , Feminino , Humanos , Masculino , México , Pessoa de Meia-Idade , Agregação Plaquetária/efeitos dos fármacos , Inibidores da Agregação Plaquetária/uso terapêutico , Estudos Prospectivos , Síndrome , Trombofilia/sangue , Trombofilia/etiologia , Trombose/sangue , Trombose/etiologia , Trombose/prevenção & controle , Ticlopidina/análogos & derivados , Ticlopidina/uso terapêutico , Adulto JovemRESUMO
Hemostasis is the balance between bleeding and clotting and includes coagulation and fibrinolysis with platelet interactions. Despite developmental hemostasis that describes the major differences between neonates and older children and adults, neonates do not have increased bleeding or clotting unless clinical situations disturb the "balance." Perinatal asphyxia alters the balance of hemostasis, resulting in abnormalities that may result in bleeding and thrombosis. The following discussion will describe normal hemostasis, laboratory measures of hemostasis, developmental hemostasis, and the effects of asphyxia on hemostasis.
Assuntos
Asfixia Neonatal/complicações , Coagulação Sanguínea , Transtornos Plaquetários/etiologia , Hemostasia , Asfixia Neonatal/sangue , Transtornos Plaquetários/sangue , Hemorragia/sangue , Hemorragia/etiologia , Humanos , Lactente , Recém-Nascido , Trombose/sangue , Trombose/etiologiaRESUMO
We observed increased bleeding tendency and platelet function abnormalities in 3 boys with McCune-Albright syndrome (MAS). We speculate that platelet dysfunction contributed to excessive blood loss in our patients. This report of platelet dysfunction in MAS highlights the need for assessment of platelet functions in the condition.
Assuntos
Transtornos Plaquetários/etiologia , Displasia Fibrosa Poliostótica/complicações , Hemorragia/etiologia , Adolescente , Criança , Displasia Fibrosa Poliostótica/cirurgia , Humanos , Lactente , Complicações Intraoperatórias , Masculino , Testes de Função PlaquetáriaRESUMO
This study aimed to clarify whether smoking had any influence on platelet aggregability in coronary patients with different lipoprotein abnormalities. We studied 297 non-diabetic patients with coronary heart disease, 40 to 85 years of age, 223 (75%) male, 167 smokers and 130 never smokers. After 3 months on Step-One diet, without any regular medication, patients had fasting plasma total cholesterol levels > or = 6.2 mmol/L; low-density lipoprotein > or = 4.14 mmol/L; and different levels of high-density lipoprotein and triglycerides. Platelet aggregation was analyzed by turbidometric method of Born. Patients were classified in groups of smokers and non-smokers. Results showed that platelet hyperaggregability was more prevalent in smokers with lower levels of high-density lipoprotein (47% vs. 20%; P=0.004 for spontaneous platelet aggregation, 56% vs. 33%; P=0.02 for adenosine diphosphate induced platelet aggregation), and in smokers with hypertrygliceridemia (64% vs. 29%; P=0.004 for spontaneous, 81% vs. 43%; P<0.0001 for adenosine diphosphate induced, and 87% vs. 46%; P<0.0001 for adrenaline induced platelet aggregation). Platelet hypoaggregability was greater in non-smokers with normal high-density lipoprotein and triglycerides plasma levels when compared to non-smokers with the same lipid profile (39% vs. 12%; P=0.004). In conclusion, smoking increased platelet reactivity in hypercholesterolemic patients with low high-density lipoprotein levels or high triglycerides levels.
Assuntos
Transtornos Plaquetários/fisiopatologia , Doença das Coronárias/fisiopatologia , Hiperlipidemias/complicações , Agregação Plaquetária , Fumar/efeitos adversos , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Transtornos Plaquetários/etiologia , Doença das Coronárias/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes de Função PlaquetáriaRESUMO
En los últimos tres años han ocurrido avances notables en el esclarecimiento de las causas de la trombofilia. Hasta hace poco los estudios de laboratorio en pacientes con trombofilia familiar permitían esclarecer la causa sólo en el 5-10 por ciento de los casos, en los que se identifican deficiencia de proteína C de coagulación, de proteína S de coagulación, de antitrombina III, etc. La recien identificación de la resistencia de la proteína C activada (RPCa) ha cambiado este panorama: hasta el 50 por ciento de los pacientes con trombofilia familiar tienen el genotipo de la RPCa, mutación de nucleótido G por A en la posición 1691 del gen del factor V, lo que produce la mutación R-506-Q (tipo Leiden) de la molécula del factor V. Esta mutación codifica la síntesis de un factor V con actividad precoagulante normal pero "resistente" a la acción lítica de la proteína C activada; ocurre en proporciones variables entre 0 y 15 por ciento de la población general y en poblaciones nórdicas en el defecto genético relacionado con enfermedad más frecuente de todos. El fenotipo de l RPCa se estudia en el laboratorio por medio de una modificación de la medición del tiempo de tromboplastina parcial activada y puede presentarse en pacientes con el genotipo de la RPCa o ser secundario a enfermedades autoinmunes, hepatopatías, empleo de anovulatorios, embarazo, etc. El estudio de todo paciente con trombofilia debe incluir la investigación del fenotipo y en su caso, el genotipo de la RPCA. Este estudio, junto con las investigaciones de las actividades antigénica y procoagulante de las proteínas C, S y antitrombina III, permiten esclarecer la causa de la trombofilia familiar en 60-70 por ciento de los casos
Assuntos
Análise Mutacional de DNA , Fator V/genética , Proteína C/análise , Proteína C/deficiência , Proteína C/genética , Trombose/etiologia , Trombose/genética , Transtornos Plaquetários/etiologiaRESUMO
Five types of oculocutaneous albinism and two types of ocular albinism were found among 349 Puerto Rican albinos. The most prevalent type of albinism was the Hermansky-Pudlak syndrome (HPS). HPS was observed in five of every six albinos in Puerto Rico. The prevalence of HPS was highest in the northwestern quarter of the island, affecting approximately one in 1,800 persons, and approximately one in 22 are carriers of the gene. HPS is an autosomal recessively inherited triad of a tyrosinase-positive type of albinism, a hemorrhagic diathesis due to storage pool deficient platelets and accumulation of ceroid in tissues. The pigmentary phenotype of HPS albinos resembled that of any other type of oculocutaneous or ocular albinism. The most reliable method of diagnosing HPS is by a deficiency of platelet dense bodies observed by electron microscopy. The accumulation of ceroid in the tissues is associated with fibrotic restrictive lung disease and granulomatous enteropathic disease. The enteropathic disorder resembles Crohn's disease and with few exceptions, had its onset after 13 years of age. The major causes of death were fibrotic restrictive pulmonary disease, hemorrhagic episodes and sequelae of granulomatous enteropathic disease. Menometrorrhagia was common in women with HPS. No immune deficiency was found in HPS patients. The majority of patients with HPS had visual acuities of 20/200 or worse and consequently were legally blind. Albinos of all types, including HPS, lacked binocular vision due to nearly complete crossing of the optic tracts.
Assuntos
Albinismo Ocular/epidemiologia , Albinismo Oculocutâneo/epidemiologia , Albinismo Oculocutâneo/complicações , Albinismo Oculocutâneo/genética , Transtornos Plaquetários/etiologia , Causas de Morte , Estudos Transversais , Humanos , Porto Rico/epidemiologiaRESUMO
Five types of oculocutaneous albinism and two types of ocular albinism were found among 349 Puerto Rican albinos. The most prevalent type of albinism was the Hermansky-Pudlak syndrome (HPS). HPS was observed in five of every six albinos in Puerto Rico. The prevalence of HPS was highest in the northwestern quarter of the island, affecting approximately one in 1,800 persons, and approximately one in 22 are carriers of the gene. HPS is an autosomal recessively inherited triad of a tyrosinase-positive type of albinism, a hemorrhagic diathesis due to storage pool deficient platelets and accumulation of ceroid in tissues. The pigmentary phenotype of HPS albinos resembled that of any other type of oculocutaneous or ocular albinism. The most reliable method of diagnosing HPS is by a deficiency of platelet dense bodies observed by electron microscopy. The accumulation of ceroid in the tissues is associated with fibrotic restrictive lung disease and granulomatous enteropathic disease. The enteropathic disorder resembles Crohn's disease and with few exceptions, had its onset after 13 years of age. The major causes of death were fibrotic restrictive pulmonary disease, hemorrhagic episodes and sequelae of granulomatous enteropathic disease. Menometrorrhagia was common in women with HPS. No immune deficiency was found in HPS patients. The majority of patients with HPS had visal acuities of 20/200 or worse and consequently were legally blind. Albinos of all types, including HPS, lacked binocular...
Assuntos
Humanos , Albinismo Ocular/epidemiologia , Albinismo Oculocutâneo/epidemiologia , Albinismo Oculocutâneo/complicações , Albinismo Oculocutâneo/genética , Transtornos Plaquetários/etiologia , Causas de Morte , Estudos Transversais , Porto Rico/epidemiologiaRESUMO
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.
Assuntos
Transtornos Plaquetários/etiologia , Plaquetas/ultraestrutura , Adulto , Transtornos Plaquetários/sangue , Transtornos Plaquetários/diagnóstico , Membrana Celular/ultraestrutura , Diagnóstico Diferencial , Feminino , Humanos , Microscopia Eletrônica , Adesividade Plaquetária , Agregação Plaquetária/efeitos dos fármacos , Contagem de Plaquetas , Propriedades de SuperfícieRESUMO
Prospective evaluation of platelet activation and hypercoagulability was performed in 31 patients with Kawasaki syndrome. Most patients had elevated acute-phase reactants when studied during the first 3 weeks of their illness; 17 of 25 (68%) patients had factor VIII activity greater than 150%, 18 of 24 (75%) had fibrinogen greater than 400 mg/dl, and 17 of 31 (55%) had a platelet count greater than 450,000/mm3. Antithrombin III was depressed initially in 17 of 25 (68%) patients. Depleted fibrinolytic activity, as measured by a euglobulin lysis time greater than 300 minutes, was documented in nine of 20 (45%) patients. Plasma beta-thromboglobulin (BTG) measured at 0 to 3 weeks was elevated (greater than 43 ng/ml) in seven of 24 (29%) patients. All patients with coronary artery aneurysms had elevated BTG values. The mean BTG in the group with aneurysms was 72.3 ng/ml when measured during the first 3 weeks after onset of fever, and 87.7 ng/ml at 4 to 7 weeks. The group without aneurysms had mean BTG values of 29.4 and 28.3 ng/ml at 0 to 3 and 4 to 7 weeks, respectively. The difference between the two groups was significant (P less than 0.002) for both the initial and later values. An elevated BTG during the first 3 weeks after onset of fever was highly associated with aneurysm formation in our patients (P less than 0.007). No aneurysms occurred in patients with a normal BTG value.