ABSTRACT
La trombocitemia esencial forma parte del grupo de neoplasias mieloproliferativas. Se caracteriza por síntomas microvasculares y vasomotores, recuento plaquetario superior a 450 x 109/l, proliferación megacariocítica con morfología grande y madura, ausencia de proliferación eritroide y granulocítica, demostración de JAK2V617F u otro marcador clonal y ausencia de evidencia de trombocitosis reactiva. Se reporta el manejo anestésico en una paciente donde las principales consideraciones están relacionadas con la prevención de eventos hemorrágicos y trombóticos. La suspensión de la aspirina, el mantenimiento del tratamiento con hidroxiurea, la preparación con ácido tranexámico, el uso pre y posoperatorio de fraxiparina, hidratación adecuada, uso de medias elásticas en miembros inferiores, deambulación precoz, buena hemostasia quirúrgica y disponibilidad de concentrados de plaquetas son los elementos fundamentales en la conducción anestésica de esta paciente(AU)
Essential thrombocythemia is part of the group of myeloproliferative neoplasms. It is characterized by microvascular and vasomotor symptoms, platelet count over 450x109/L, megakaryocytic proliferation with large and mature morphology, absence of erythroid and granulocytic proliferation, demonstration of JAK2V617F or other clonal marker, and absence of evidence of reactive thrombocytosis. Anesthetic management is reported in a patient, whose case's main considerations are related to the prevention of hemorrhagic and thrombotic events. Aspirin suspension, maintenance of hydroxyurea treatment, preparation with tranexamic acid, pre- and post-operative use of fraxiparin, adequate hydration, use of elastic stockings in lower limbs, early ambulation, good surgical hemostasis, as well as availability of platelet concentrates are the fundamental elements in the anesthetic management of this patient(AU)
Subject(s)
Humans , Female , Middle Aged , Platelet Count , Thrombocythemia, Essential/complications , Hemostasis, Surgical , Tranexamic Acid/therapeutic use , Stockings, Compression , Anesthetics/therapeutic useABSTRACT
SUMMARY The essential thrombocythemia is one of the seven described forms of myeloproliferative neoplasms. It is characterized by megakaryocytic hyperplasia with consequent thrombocytosis maintained in the peripheral blood, favoring the occurrence of thrombo-hemorrhagic phenomena. We present the case of an 81-year-old woman with a history of ischemic stroke in the context of a sustained thrombocytosis, which led to a spinal study and a search for the V617F mutation in the JAK2 gene, which was positive. The patient started cytoreductive therapy with hydroxyurea with favorable current evolution.
Subject(s)
Humans , Female , Aged , Stroke/etiology , Thrombocythemia, Essential/complications , Risk Factors , Stroke/genetics , Janus Kinase 2/genetics , Thrombocythemia, Essential/genetics , MutationABSTRACT
Summary Introduction: In patients with essential thrombocythemia (ET), the vascular complications contribute to morbidity and mortality. To better predict the occurrence of thrombotic events, an International Prognostic Score for Thrombosis in Essential Thrombocythemia (IPSET-thrombosis) has recently been proposed. We present the application of this score and compare its results with the usual classification system. Method: We retrospectively evaluated the characteristics and risk factors for thrombosis of 46 patients with a diagnosis of ET seen in the last 6 years at Faculdade de Medicina do ABC (FMABC). Results: Thrombosis in the arterial territory was more prevalent than in venous sites. We observed that cardiovascular risk factors (hypertension, hypercholesterolemia, diabetes mellitus, and smoking) were also risk factors for thrombosis (p<0.001). Age over 60 years and presence of JAK2 V617F mutation were not associated with the occurrence of thrombotic events. No patient classified by IPSET-thrombosis as low risk had a thrombotic event. Furthermore, using the IPSET-thrombosis scale, we identified two patients who had thrombotic events during follow-up and were otherwise classified in the low-risk group of the traditional classification. Leukocytosis at diagnosis was significantly associated with arterial thrombosis (p=0.02), while splenomegaly was associated with venous thrombotic events (p=0.01). Conclusion: Cardiovascular risk factors and leukocytosis were directly associated with arterial thrombosis. IPSET-thrombosis appears to be better than the traditional classification at identifying lower risk patients who do not need specific therapy.
Resumo Introdução: em pacientes com trombocitemia essencial (TE), complicações vasculares contribuem para morbidade e mortalidade. Para melhor predizer a ocorrência de eventos trombóticos, um escore prognóstico internacional de trombose para TE (IPSET-trombose) foi recentemente desenvolvido. Apresentamos aqui a aplicação desse escore e comparamos seus resultados com o sistema de classificação usual. Método: avaliamos retrospectivamente as características e os fatores de risco para trombose em 46 pacientes com diagnóstico de TE que foram atendidos nos últimos 6 anos na Faculdade de Medicina do ABC. Resultados: trombose em território arterial é mais prevalente que em sítio venoso. Observamos que fatores de risco cardiovascular (hipertensão, hipercolesterolemia, diabetes mellitus e tabagismo) foram considerados fatores de risco para trombose (p<0,001). Idade > 60 anos e presença de mutação JAK2 V617F não se associaram à ocorrência de eventos trombóticos. Nenhum paciente classificado como baixo risco pelo IPSET-trombose apresentou evento trombótico. Quando comparado à classificação de risco tradicional, IPSET-trombose foi capaz de identificar dois pacientes que evoluíram com trombose no seguimento e estavam categorizados no grupo de baixo risco. Leucocitose ao diagnóstico foi mais prevalente em pacientes que apresentaram trombose arterial (p=0,02), e esplenomegalia, entre aqueles com evento trombótico venoso (p=0,01). Conclusão: fatores de risco cardiovascular e leucocitose se associaram de forma direta com trombose arterial. IPSET-trombose parece ser melhor que a classificação tradicional na identificação de pacientes de baixo risco que não precisam de terapia específica.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Aged , Aged, 80 and over , Young Adult , Thrombosis/etiology , Risk Assessment/methods , Thrombocythemia, Essential/complications , Prognosis , Reference Values , Thrombosis/diagnosis , Brazil , Smoking/adverse effects , Retrospective Studies , Risk Factors , Diabetes Complications , Janus Kinase 2/genetics , Hospitals, Public/statistics & numerical data , Hypertension/complications , Middle AgedABSTRACT
La trombocitosis esencial (TE) es una panmielopatía clonal de escasa frecuencia, que está incluida dentro de los síndromes mieloproliferativos crónicos. Se caracteriza por la proliferación anormal de los megacariocitos, y las manifestaciones clínicas principales son: hemorragia y/o diátesis tromboembólica, en cuyo caso se presentan fenómenos necróticos y ulcerosos. Se presentan dos pacientes de sexo masculino de 54 y 80 años, cuyas primeras manifestaciones fueron lesiones úlcero-necróticas cutáneas. Con recuentos plaquetarios mayores a 1.000.000 mm3 y la punción biopsia de médula ósea que reveló hiperplasia megacariocítica, se arriba al diagnóstico de TE.
Essential thrombocytosis (ET) is a rare, clonal panmyelopathy and one of a group of disordersknown as chronic myeloproliferative diseases.ET is associated with sustained megakaryocyte proliferation, and clinically presents hemorrhage orthrombosis with ulcerative or necrotic lesions.We report two male patients aged 54 and 80-years-old, respectively, with ulcerative and necroticlesions on the skin as the first manifestation of this disease. The platelet count was higher than1.000.000 mm3 and the bone marrow aspirate biopsy showed megakaryocytic hyperplasiadiagnostic of ET.
Subject(s)
Humans , Male , Middle Aged , Thrombocythemia, Essential/complications , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/drug therapy , Skin/pathology , Thrombocytosis/complications , Thrombocytosis/diagnosis , Thrombocytosis/drug therapyABSTRACT
Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. After many years, a few patients with ET may develop bone marrow (BM) fibrosis and rarely develop osteosclerosis. A 60-yr-old female was admitted due to severe left upper quadrant abdominal discomfort. She had been diagnosed as ET 19 yrs ago. On liver computed tomography severe splenomegaly was shown. Laboratory tests revealed WBC 24.3x10(9)/L, hemoglobin 13.4 g/dL, platelets 432x10(9)/L, lactate dehydrogenase 4,065 IU/L (reference range; 240-480). Blood smear demonstrated leukoerythroblastosis, teardrop cells, and giant and hypogranular platelets. BM study revealed inadequate aspirate due to dry tap. BM biopsy showed clusters of dysplastic megakaryocytes, grade 3 fibrosis, and severe osteosclerosis. Major/minor BCR-ABL1 rearrangement and JAK2 V617F mutation were not detected. Cytogenetic studies revealed normal karyotype. According to the 2008 WHO diagnostic criteria, the patient was diagnosed as having post-essential thrombocythemia myelofibrosis with severe osteosclerosis.
Subject(s)
Female , Humans , Middle Aged , Bone Marrow/pathology , Megakaryocytes/pathology , Osteosclerosis/complications , Primary Myelofibrosis/complications , Splenomegaly/etiology , Thrombocythemia, Essential/complications , Tomography, X-Ray ComputedABSTRACT
Essential thrombocythemia (ET) is a clonal disorder of myeloid stem cells that causes thrombocytosis. As a result, ET can lead to vascular thrombosis and tissue ischemia; the association of coronary artery abnormalities such as myocardial infarction or unstable angina is rare. Here we describe a 45-year-old male patient with essential thrombocythemia who presented with unstable angina. Elective coronary angiography showed total occlusion of mid right coronary artery and mid left anterior descending coronary artery. ET was confirmed by a bone marrow biopsy; treatment was started with antiplatelet therapy including aspirin and clopidogrel along with cytostatic therapy with hydroxyurea and anagrelide. After the initiation of the treatment, the platelet count decreased to 20 s 10(4)/microliter. In addition, percutaneous coronary angioplasty was successfully performed with stent placement at the right coronary artery without hemorrhagic or thrombotic complications.
Subject(s)
Middle Aged , Male , Humans , Thrombocythemia, Essential/complications , Stents , Angioplasty, Balloon, Coronary , Angina, Unstable/etiologyABSTRACT
Essential thrombocythemia is an entity characterized by clonal proliferation of megakaryocytes and thromboccytosis. We have analyzed the presenting manifestation of 13 patients with Essential Thrombocythemia diagnosed in the last three years. The major findings include the frequent presence of symptomatology of a hypercoagulable state with pro,pt improvement of these symptoms once the thrombocytosis is controlled. Constitutional symptoms, organomegaly, modest Leukocytosis and eosinophillia were also often seen. No catastrophic events were detected with a mean follow up period of 17.5 months
Subject(s)
Adolescent , Adult , Middle Aged , Female , Humans , Male , Thrombocythemia, Essential , Aged, 80 and over , Bone Marrow/pathology , Hemorrhagic Disorders/etiology , Platelet Count , Thrombocythemia, Essential/blood , Thrombocythemia, Essential/complications , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/pathology , Thrombosis/etiologyABSTRACT
A rare case of erythroblastopenia associated with essential thrombocythemia (ET) is described. The patient had markedly elevated platelet count (5200 x 10(9)/1) and significant platelet dysfunction leading to extensive soft tissue and gastrointestinal hemorrhage. There was paucity of erythroid precursors in the bone marrow - a feature hitherto undescribed in ET. The thrombocytosis responded to well busulphan therapy but patient succumbed to fulminant infection consequent upon drug induced neutropenia.
Subject(s)
Female , Humans , Middle Aged , Red-Cell Aplasia, Pure/etiology , Thrombocythemia, Essential/complicationsABSTRACT
O proposito do presente trabalho e colaborar para o estudo da patogenia da plaquetopenia que ocorre na Leptospirose. A pesquisa foi feita de maneira prospectiva e o grupo de casos foi constituido por 30 pacientes internados com hipotese diagnostica de Leptospirose na Clinica de Doencas Infecciosas e Parasitarias do Hospital das Clinicas da Faculdade de Medicina de Sao Paulo. Investigou-se a possibilidade de haver consumo de plaquetas por coagulacao intra vascular disseminada, ou a possibilidade de destruicao periferica de plaquetas por anticorpos e, se poderia haver inibicao da producao de plaquetas em nivel medular. Para a investigacao desejada foram utilizados os seguintes exames: contagem de plaquetas, tempo de protrombina, tempo de recalcificacao, tempo de trombina, tempo de tromboplastia parcial ativada, dosagem do fibrinogenio, dosagem do fator V, dosagem do fator VIII, dosagem dos produtos de degradacao da fibrina, dosagem da antitrombina III, mielograma e pesquisa de anticorpos antiplaquetas. Foram ainda estudados o hemograma, a dosagem de ureia, a dosagem de creatinina, a dosagem das enzimas hepaticas (aspartatoaminotransferase, alaninaaminotransferase, desidrogenase lactica, gamaglutamiltranspeptidade e fosfatase alcalina), e a dosagem das bilirrubinas. A analise dos dados obtidos no presente trabalho