Homeopatia em trombocitopenia canina: relato de caso / Homeopathy in canine thrombocytopenia: case report

Hemoparasitoses constituem-se de enfermidades cosmopolitas que são causadas por parasitos intracelulares obrigatórios de células sanguínea. Os mais frequentemente encontrados nos cães são a babesiose, erliquiose e anaplasmose. O tratamento consiste no uso de antibiótico do grupo das tetraciclinas, sendo a doxiciclina o medicamento mais indicado. O objetivo deste relato de caso é descrever o tratamento homeopático em um cão da raça shih-tzu, senil (8 anos de idade), cardiopata com trombocitopenia discreta secundário a hemoparasitose ­ ehrlichiose. O medicamento homeopático repertorizado foi Phosphorus 30 cH 3 glóbulos 2x ao dia por 3 dias. Foi administrado também a Calcarea Carbonica 6 cH 3 glóbulos 2x ao dia por 7 dias. Tal sucesso terapêutico foi atingido em 3 dias de tratamento, com melhora laboratorial da trombocitopenia e do quadro de dispnéia. Este estudo contribui com pesquisas existentes a caráter de novos tratamentos para a ehrlichiose canina.

Hemoparasitoses are cosmopolitan diseases that are caused by obligate intracellular parasites of blood cells. The most frequently found in dogs are babesiosis, ehrlichiosis and anaplasmosis. Treatment consists of the use of antibiotics from the tetracycline group, with doxycycline being the most indicated medication. The objective of this case report is to describe the homeopathic treatment in a dog of the shih-tzu breed, senile (8 years old), heart disease with mild thrombocytopenia secondary to hemoparasitosis - ehrlichiosis. The repertorized homeopathic medicine was Phosphorus 30 cH 3 globules 2x a day for 3 days. Calcarea Carbonica 6 cH 3 globules was also administered twice a day for 7 days. Such therapeutic success was achieved in 3 days of treatment, with laboratory improvement of thrombocytopenia and dyspnea. This study contributes to existing research on new treatments for canine ehrlichiosis.

Síndrome de Evans y embarazo: reporte de un caso / Evans syndrome and pregnancy: a case report

Resumen El síndrome de Evans es una enfermedad conformada por la presencia simultánea o secuencial de trombocitopenia inmunitaria y anemia hemolítica autoinmunitaria, que puede ser primaria o secundaria a otra patología. Es una afección poco frecuente, por lo que es necesario tener una alta sospecha, y descartar otras patologías que cursan con dichas alteraciones hematológicas, para hacer el diagnóstico. Su manejo representa un desafío terapéutico dado su curso crónico y recidivante. La presentación durante el embarazo se asocia a morbilidad materna y fetal. A continuación presentamos el caso de una gestante en quien se pesquisó trombocitopenia severa aislada al ingreso al control prenatal, y que en el curso del embarazo desarrolló AHAI conformando un síndrome de Evans, que se consideró secundario a LES incompleto al realizar el estudio reumatológico. Debido a la pobre respuesta al tratamiento médico con corticoides e inmunosupresores, la mayor parte del embarazo se mantuvo hospitalizada para observación, ajuste y cambio de terapia, siendo necesario recurrir a manejo quirúrgico con esplenectomía.

Abstract Evans syndrome is a rare entity formed by the simultaneous or sequential presence of immune thrombocytopenia and autoimmune hemolytic anemia, which can be primary or secondary to another pathology. The presentation of this disease during pregnancy is associated with maternal and fetal morbidity. The syndrome's diagnosis requires a high suspicion and the ruling out of other pathologies that can happen with the same hematological alterations. The management represents a therapeutic challenge because of its chronic and recurrent course. Below we present the case of a pregnant woman in whom isolated severe thrombocytopenia was detected at admission for prenatal control, and who developed AIHA during the pregnancy, forming Evans syndrome, which was considered secondary to incomplete SLE when performing the rheumatological study. Due to the poor response to medical treatment with corticosteroids and immunosuppressants, the patient was hospitalized for most of her pregnancy for observation, adjustment and change of therapy, and even it was necessary resort to surgical management with splenectomy.

Manifestaciones orales en pacientes con Trombocitopenia inmune primaria. Reporte de caso / Oral manifestations in primary immune thrombocytopenia patients. Case report

Resumen Introducción: La trombocitopenia inmune primaria (PTI) es una enfermedad caracterizada por la destrucción acelerada de plaquetas o la producción inadecuada de estas. Se puede clasificar según su etiología en primaria y secundaria. Métodos: Se presenta el caso de un paciente sexo masculino de 30 años de edad, que acude al servicio de urgencia dental del Hospital Barros Luco, por lesiones hemorrágicas mucosa oral y pequeñas manchas rojas en manos, brazos y espalda, de veinticuatro horas de evolución, asintomáticas y sin causa atribuible. Al examen físico se corrobora la presencia de múltiples vesículas hemorrágicas en región bucal y petequias en las zonas descritas. Se realiza diagnóstico presuntivo de PTI que se confirma al obtener un recuento plaquetario (RP) de 2000 uL. Se deriva a medicina interna para evaluación y manejo. La literatura describe que para confirmar esta enfermedad se requiere una historia clínica exhaustiva, descartar una patología sistémica o infecciosa previa y pruebas de laboratorio que evidencien recuento normal de células sanguíneas con una trombocitopenia marcada. Conclusión: El odontólogo tiene un rol significativo en el manejo multidisciplinario en este tipo de cuadros donde el conocimiento de manifestaciones orales de enfermedades sistémicas es de vital importancia para el diagnóstico, derivación y posterior tratamiento de estas.

Abstract Introduction: Primary immune thrombocytopenia (ITP) is a disease characterized by accelerated destruction of platelets or inadequate production of platelets. It can be classified according to its etiology in primary and secondary. Methods: We present the case of a 30-year-old male patient, who comes to the dental emergency service of the Barros Luco Hospital, for oral mucosa hemorrhagic lesions and small red spots on the hands, arms and back, 24 hours of evolution, asymptomatic and without attributable cause. Physical examination corroborates the presence of multiple hemorrhagic vesicles in the oral region and petechiae in the areas described. A presumptive diagnosis of ITP is made and confirmed by obtaining a platelet count (RP) of 2000 uL. He is referred to internal medicine for evaluation and management. The literature describes that confirming this disease requires an exhaustive medical history, ruling out a previous infectious or systemic pathology, and laboratory tests that show normal blood cell counts with marked thrombocytopenia. Conclusions: The dentist has a significant role in multidisciplinary management in this type of condition, where knowledge of oral manifestations of systemic diseases is of vital importance for the diagnosis, referral and subsequent treatment of these. Oral manifestations in primary immune thrombocytopenia patients. Case report

Macrothrombocytopenia, renal dysfunction and nephrotic syndrome in a young male patient: a case report of MYH9-related disease / Macrotrombocitopenia, disfunção renal e síndrome nefrótica em paciente jovem do sexo masculino: relato de caso de doença relacionada ao MYH9

ABSTRACT MYH9-related disease is an autosomal dominant disorder caused by mutations of the MYH9 gene, which encodes the non-muscle myosin heavy chain IIA on chromosome 22q12. It is characterized by congenital macrothrombocytopenia, bleeding tendency, hearing loss, and cataracts. Nephropathy occurs in approximately 30% of MYH9-related disease in a male patient carrier of a de novo missense mutation in exon 1 of the MYH9 gene [c.287C > T; p.Ser(TCG)96(TTG)Leu]. He presented all phenotypic manifestations of the disease, but cataracts. Renal alterations were microhematuria, nephrotic-range proteinuria (up to 7.5 g/24h), and rapid loss of renal function. The decline per year of the glomerular filtration rate was 20 mL/min/1.73m2 for five years. Blockade of the renin-angiotensin system, the only recommended therapy for slowing the progression of this nephropathy, was prescribed. Although MYH9-related disease is a rare cause of glomerulopathy and end-stage renal disease, awareness of rare genetic kidney disorders is essential to ensure accurate diagnosis and proper management of orphan disease patients.

RESUMO A doença relacionada ao MYH9 é um distúrbio autossômico dominante causado por mutações no gene MYH9 que codifica a cadeia pesada da miosina não muscular IIA no cromossomo 22q12. Ela é caracterizada por macrotrombocitopenia congênita, tendência a sangramento, perda auditiva e catarata. A nefropatia ocorre em aproximadamente 30% dos pacientes. O presente artigo relata o caso de um paciente com doença relacionada ao MYH9 portador de mutação missense de novo no exon 1 do gene MYH9 [c.287C > T; p.Ser(TCG)96(TTG)Leu]. Com a exceção de catarata, o paciente apresentou todas as manifestações fenotípicas da doença. As alterações renais incluíram micro-hematúria, proteinúria nefrótica (até 7,5 g/24h) e perda rápida da função renal. O declínio anual da taxa de filtração glomerular foi de 20 mL/min/1,73 m2 durante cinco anos. Foi receitado bloqueio do sistema renina-angiotensina, a única terapia recomendada para retardar a progressão dessa nefropatia. Embora a doença relacionada ao MYH9 seja uma causa rara de glomerulopatia e doença renal terminal, a conscientização sobre distúrbios genéticos renais raros é essencial para garantir o diagnóstico preciso e o manejo adequado dos pacientes com tal doença órfã.

Fatores prognósticos em pacientes graves com meningite bacteriana adquirida na comunidade e lesão renal aguda / Prognostic factors among critically ill patients with community-acquired acute bacterial meningitis and acute kidney injury

RESUMO Objetivo: Investigar os fatores prognósticos em pacientes graves com meningite bacteriana adquirida na comunidade e lesão renal aguda. Métodos: Estudo retrospectivo com inclusão de pacientes em um hospital terciário dedicado a doenças infecciosas localizado em Fortaleza (CE), com diagnóstico de meningite bacteriana adquirida na comunidade complicada por lesão renal aguda. Investigaram-se os fatores associados a óbito, ventilação mecânica e uso de vasopressores. Resultados: Incluíram-se 41 pacientes, com média de idade de 41,6 ± 15,5 anos, 56% dos quais do sexo masculino. O tempo médio entre a admissão à unidade de terapia intensiva e o diagnóstico de lesão renal aguda foi de 5,8 ± 10,6 dias. A mortalidade global foi de 53,7%. Segundo os critérios KDIGO, 10 pacientes foram classificados como estágio 1 (24,4%), 18 como estágio 2 (43,9%) e 13 como estágio 3 (31,7%). A classificação em estágio KDIGO 3 aumentou de forma significante a mortalidade (OR = 6,67; IC95% = 1,23 - 36,23; p = 0,028). A presença de trombocitopenia não se associou com aumento da mortalidade, porém foi um fator de risco para a ocorrência da classificação KDIGO 3 (OR = 5,67; IC95% = 1,25 - 25,61; p = 0,024) e para necessidade de utilizar ventilação mecânica (OR = 6,25; IC95% = 1,33 - 29,37; p = 0,02). Os pacientes que necessitaram de ventilação mecânica 48 horas após o diagnóstico de lesão renal aguda tiveram níveis mais elevados de ureia (44,6 versus 74mg/dL; p = 0,039) e sódio (138,6 versus 144,1mEq/L; p = 0,036). Conclusão: A mortalidade de pacientes graves com meningite bacteriana adquirida na comunidade e lesão renal aguda é alta. A severidade da lesão renal aguda se associou com mortalidade ainda mais elevada. A presença de trombocitopenia se associou com lesão renal aguda mais grave. Níveis mais elevados de ureia podem prever mais precocemente a ocorrência de lesão renal aguda de maior gravidade.

ABSTRACT Objective: To investigate prognostic factors among critically ill patients with community-acquired bacterial meningitis and acute kidney injury. Methods: A retrospective study including patients admitted to a tertiary infectious disease hospital in Fortaleza, Brazil diagnosed with community-acquired bacterial meningitis complicated with acute kidney injury. Factors associated with death, mechanical ventilation and use of vasopressors were investigated. Results: Forty-one patients were included, with a mean age of 41.6 ± 15.5 years; 56% were males. Mean time between intensive care unit admission and acute kidney injury diagnosis was 5.8 ± 10.6 days. Overall mortality was 53.7%. According to KDIGO criteria, 10 patients were classified as stage 1 (24.4%), 18 as stage 2 (43.9%) and 13 as stage 3 (31.7%). KDIGO 3 significantly increased mortality (OR = 6.67; 95%CI = 1.23 - 36.23; p = 0.028). Thrombocytopenia was not associated with higher mortality, but it was a risk factor for KDIGO 3 (OR = 5.67; 95%CI = 1.25 - 25.61; p = 0.024) and for mechanical ventilation (OR = 6.25; 95%CI = 1.33 - 29.37; p = 0.02). Patients who needed mechanical ventilation by 48 hours from acute kidney injury diagnosis had higher urea (44.6 versus 74mg/dL, p = 0.039) and sodium (138.6 versus 144.1mEq/L; p = 0.036). Conclusion: Mortality among critically ill patients with community-acquired bacterial meningitis and acute kidney injury is high. Acute kidney injury severity was associated with even higher mortality. Thrombocytopenia was associated with severer acute kidney injury. Higher urea was an earlier predictor of severer acute kidney injury than was creatinine.

Síndrome de Wiskott-Aldrich. Dificultades diagnósticas y terapéutucas de un paciente con inmunodeficiencia primario / Wiskott - Aldrich syndrome. Therapeutic and Diagnosis Difficulties in a patient with primary immunodeficiency

Introducción. Las inmunodeficiencias primarias son un grupo de enfermedades de origen genético que implican altera - ciones asociadas a la respuesta inmunológica. El infra diagnóstico de estas conlleva al retraso de tratamiento, evitables en gran parte; Entre estas existe el síndrome de Wiskott-Aldrich; es un trastorno raro, ligado al cromosoma X, recesivo, que se caracteriza por trom - bocitopenia, eczema e inmunodeficiencia donde su tratamiento curativo es el trasplante de medula ósea. CASO CLÍNICO : Paciente de 10 años, con antecedentes de múltiples hospitalizaciones por procesos infecciosos importantes: neumonías recurrentes, menin - gitis, diarreas, erupción cutánea generalizada y trombocitopenia de hasta 9,000 mm³. Después de múltiples estudios realizados, se confirma el diagnóstico de síndrome de Wiskott -Aldrich por inmunogenetica (mutación del gen WAS) y mediante colaboración médica internacional, se realiza trasplante de médula ósea con posterior resolución de su enfermedad. DISCUSION: Las inmunodeficiencias primarias son patologías más comunes de lo que se creía (prevalencia de hasta 1/1200), la evidencia de aparición y su importancia clínica deben ser tomadas en consideración. En este caso de Síndrome de Wiskot-Aldrich en donde el diagnóstico definitivo es in - munogenetico, (actualmente el país no cuenta), además de tratamiento inmuno-oncológico adecuado, el paciente pudo sobrevivir y mejorar su calidad de vida gracias a soporte investigativo y terapéutico multinacional. Existen colaboraciones multicentricas como el consorcio de tratamiento inmunodeficiencias primarias, que tienen como objetivo colaborar activamente en el diagnóstico y tratamien - to estos casos, salvaguardando la vida de estos pacientes y ayudando a comprender estas enfermedades raras...(AU)

Hemangioblastoma cerebeloso y trombocitopenia: reporte de caso / Cerebellar hemangioblastoma and thrombocytopenia: report of one case

The association between vascular tumors and thrombocytopenia is rare. Kasabach-Merritt Syndrome is seen in childhood and is characterized by hemangiomas and thrombocytopenia. A 42 years-old man with a cerebellar hemangioblastoma and thrombocytopenia, admitted with a subarachnoid hemorrhage is reported. The patient was operated and required a splenectomy to manage the thrombocytopenia. After the splenectomy the patient developed a subdural hematoma that was operated. Despite the surgical treatment, the patient died.

Síndrome TAR en paciente adulto sin diagnóstico previo: presentación de un caso / TAR syndrome in an adult patient without previous diagnosis, a case report

El síndrome TAR (Thrombocytopenia with Absent Radius) es una patología congénita autosómica recesiva infrecuente, caracterizada por trombocitopenia con aplasia de radio bilateral. Incluye malformaciones esqueléticas, renales, hematológicas y cardíacas. Su base genética todavía no está clara. Presentamos el caso de una paciente sin diagnóstico previo de síndrome TAR que llega a la consulta, tras haber sido evaluada por varios profesionales médicos, para el diagnóstico y el tratamiento de trastornos hematológicos, que finalmente estuvieron asociados a su síndrome congénito. (AU)

Thrombocytopenia with Absent Radius (TAR) is a rare autosomic recessive disease characterized by thrombocytopenia and bilateral radial aplasia, which includes skeletal, hematologic, renal and cardiac abnormalities. The genetics bases of this syndrome remain unclear. We report here a patient without a previous diagnosis of TAR syndrome who was seen in the clinic, after being evaluated by several medical professionals for diagnosis and treatment of blood disorders, which eventually were associated with the congenital syndrome. (AU)

Prevalencia y caracterización de trombocitopenia en el embarazo: experiencia en el Hospital "José R. Vidal" de Corrientes / Prevalence and characterization of thrombocytopenia in pregnancy: experience at the "José R. Vidal" Hospital in Corrientes

La trombocitopenia se encuentra presente entre un 6 al 10% de los todos los embarazos. La trombocitopenia gestacional (TG) es definida como una trombocitopenia moderada que ocurre en el tercer trimestre del embarazo y presenta remisión espontanea después del parto no estando relacionada a la trombocitopenia neonatal. Asimismo, no requiere tratamiento y existen pocos datos para distinguirla de la Púrpura Trombocitopénica Inmune en mujeres embarazadas de las no embarazadas debido a que el manejo clínico es diferente En este trabajo se pretende establecer un método confiable para el recuento de plaquetas (RP) y repasar las diferentes causas de trombocitopenia en nuestra Institución.Se incluyeron 9.890 mujeres embarazadas que ingresaron al Servicio de Obstetricia del Hospital "José R. Vidal" de la Ciudad de Corrientes durante octubre 2009 a octubre 2011. La incidencia de trombocitopenia durante el embarazo encontrada fue del 6.7% de las cuales, 667 embarazadas con trombocitopenia, 459 (68.8%) presentaron trombocitopenia leve, 193 (28.9 %) trombocitopenia moderada y 15 (2.2 %) trombocitopenia severa. En la población normal, el RP promedio fue de 287.61 ± 9.78 109, en las pacientes con trombocitopenia fue de 90.59 ± 6.27 109con el método de Fonio mostrando una diferencia estadísticamente significativa entre ambos grupos (p < 0.05). Lasconclusiones de este estudio indican que la incidencia de trombocitopenia durante el embarazo es similar a la encontrada por otros autores y la TG es la causa más importante de trombocitopenia. Finalmente, existen diferencias significativas en el RP cuando se emplea el método indirecto de Fonio comparado con métodos automático

Thrombocytopenia during pregnancy is present between 6 to 10% of all pregnancies. Gestational thrombocytopenia (GT) is defined as a moderate thrombocytopenia, which occurs in the third trimester of pregnancy and the spontaneous remission is presents after birth and is not related to neonatal thrombocytopenia. The GT requires no treatment and there are few data to distinguish Púrpura thrombocytopenic autoimmune in the pregnant women and in the non-pregnant because the clinical management differs. The aim of this work was to establish a reliable method for counting platelets and analyzed different causes of thrombocytopenia in our institution. 9,890 pregnancy women were admitted to the Obstetrics Service of the Hospital "Jose R.Vidal" of Corrientes during October 2009-October 2011. The incidence of thrombocytopenia during pregnancy was found 6.7 % and of the 667 pregnant women with thrombocytopenia, 459 (68.8 %) had mild thrombocytopenia, 193 (28.9 %) moderate thrombocytopenia and 15 (2.2 %) severe thrombocytopenia. In the normal population average was 287.61 109± 9.78 in patients with thrombocytopenia was 90.59 ± 6.27 109using the method of Fonio which showing a statistically significant difference between groups (p < 0.05). The findings of this study were that the incidence of thrombocytopenia during pregnancy is similar to that found by other authors being the GT is the most important cause of thrombocytopenia. Finally, there are significant differences in the RP when the indirect method of Fonio compared to automatized methods

Bone Marrow Suppression and Hemophagocytic Histiocytes Are Common Findings in Korean Severe Fever with Thrombocytopenia Syndrome Patients

The causes of cytopenia in patients with severe fever with thrombocytopenia syndrome (SFTS) are not fully understood until now. We reviewed the bone marrow (BM) findings of patients with SFTS to unravel the cause of the cytopenia. Three Korean SFTS were enrolled in this study. Thrombocytopenia, neutropenia, and anemia were detected in all three patients. Severe hypocellular marrow (overall cellularity <5%) and a decreased number of megakaryocytes were noted in one patient, and hypo-/normocellular marrow and an increased number of hemophagocytic histiocytes were observed in two patients. Megakaryocytes were relatively preserved in two patients. Although a limited number of cases are available, our observations suggest that both BM suppression and peripheral destruction or sequestration are causes of cytopenia of patients with SFTS. To the best of our knowledge, this is the first well documented pathologic evaluation of Korean SFTS.

Síndrome de aglutininas frías y púrpura trombocitopénica autoinmune: Un caso inusual de síndrome de Evans / Cold agglutinins syndrome and autoimmune thrombocytopenic purpura: An unusual case of Evans syndrome

El síndrome de Evans es un trastorno poco frecuente en el que se observan trombocitopenia y anemia, ambas de etiología autoinmune; las que pueden ocurrir de manera simultánea o sucesiva. Se presenta un caso poco usual de anemia hemolítica autoinmune por anticuerpos fríos asociada a púrpura trombocitopénica autoinmune. Paciente femenina de 22 años de edad con diagnóstico de púrpura trombocitopénica autoinmune, después de 7 años de evolución y un año en remisión, presentó una anemia hemolítica autoinmune por anticuerpos fríos, refractaria al tratamiento con esteroides y alcaloides de la Vinca, que requirió transfusiones de concentrado de eritrocitos y logró la remisión con la administración de anticuerpo monoclonal anti CD 20. Los restantes estudios de autoinmunidad fueron negativos. Actualmente se mantiene asintomática y sin tratamiento inmunosupresor(AU)

Evans syndrome is a rare disorder in which thrombocytopenia and anemia are observed, both of autoimmune aetiology, which may occur simultaneously or successively. A rare case of cold autoimmune hemolytic anemia associated to autoimmune thrombocytopenic purpura is presented. A 22-year-old female patient with diagnosis of autoimmune thrombocytopenic purpura, after 7 years of evolution and one year in remission, has a cold autoimmune hemolytic anemia, refractory to steroid treatment and vinca alkaloids, which requires transfusions of packed erythrocytes and achieves remission with anti CD 20 monoclonal antibody. The remaining studies of autoimmunity are negative. Currently the patient is asymptomatic and without immunosuppressive therapy(AU)

Síndrome HELLP: morbilidad-mortalidad materna y perinatal / HELLP syndrome: maternal morbidity and mortality perinatal

Objetivo: Evaluar la morbimortalidad materno fetal y neonatal en pacientes con síndrome HELLP. Métodos: Estudio retrospectivo, descriptivo y longitudinal que fue hecho en 128 pacientes con síndrome HELLP en el período comprendido entre enero 2004 y abril 2009 en el Servicio de Medicina Materno Fetal de la Maternidad “Concepción Palacios”. Resultados: De las 128 pacientes estudiadas, el síndrome HELLP se presentó antes del parto en 85 casos (66,4%) y después del parto en 43 casos (33,6%), el 43,8% de las pacientes fueron primigestas y el 73,4% presentaron el síndrome antes de las 37 semanas. No se presentaron casos de muerte materna. El síndrome se asoció a una alta tasa de morbilidad materna (62,5%) siendo la principal complicación la insuficiencia renal aguda (46%). No hubo ningún caso de hematoma subcapsular hepático. El 100% de las pacientes presentaron algún grado de hipertensión arterial. Hubo una alta tasa de mortalidad perinatal (18%) asociada principalmente a prematuridad. El peso promedio al nacer fue de 1 654 ± 728 g. Conclusión: El síndrome HELLP se asocia a una alta tasa de morbilidad materna extrema y alta tasa de morbimortalidad perinatal, esta última asociada principalmente a complicaciones de prematuridad.

Objective: To evaluate the maternal fetal and neonatal morbidity and mortality in patients with HELLP syndrome. Methods: Retrospective, descriptive and longitudinal study which was developed in 128 patients with HELLP syndrome in the period between January 2004 and April 2009 in the Maternal Fetal Medicine service the Maternity Concepción Palacios. Results: Of the 128 patients studied, the HELLP syndrome was presented before delivery in 85 % cases (66.4 %) and postpartum in 43 cases (33.6 %), 43.8 % of the patients were primiparous and 73.4 % had the syndrome before 37 weeks. No cases of maternal death. Syndrome is associated to a high rate of maternal morbidity (62.5 %) being the main complication of acute renal failure (46 %). There were no cases of hepatic subcapsular hematoma. 100 % of the patients had some degree of arterial hypertension. There was a high perinatal mortality rate (18 %) mainly associated with prematurity. The average birth weight was 1 654 ± 728g. Conclusion: HELLP syndrome is associated with a high rate of near miss maternal morbidity and high rates in perinatal morbidity and mortality, the latter mainly associated with complications of prematurity.

Trombocitopenia en el embarazo: gestacional, inmune y congénita / Thrombocytopenia in pregnancy: gestational, immune and congenital

La trombocitopenia afecta hasta el 10 por ciento de todos los embarazos y es un diagnóstico común y un problema en el manejo de las pacientes, ya que puede estar relacionado con condiciones prexistentes presentes en las mujeres en edad fértil, como la trombocitopenia inmune primaria y las trombocitopenias congénitas; o con trastornos intrínsecos del embarazo, como la trombocitopenia gestacional. Se recomienda que todas las mujeres embarazadas con recuento de plaquetas por debajo de 100 x 10(9)/L sean sometidas a una evaluación por el hematólogo y el obstetra. El análisis cuidadoso del momento del inicio de la trombocitopenia asociado a las manifestaciones clínicas y las pruebas de laboratorio específicas, es indispensable para proporcionar un diagnostico apropiado y una asistencia médica materna-fetal en el momento oportuno, en preparación para el desafío homeostático...

Thrombocytopenia affects up to 10 percent of all pregnant women and is a common diagnosis and a problem in the management of patients as it may be related to preexisting conditions in women of childbearing age, such as primary immune thrombocytopenia and congenital thrombocytopenia or intrinsic disorders of pregnancy as gestational thrombocytopenia. It is recommended that all pregnant women with a platelet count below 100 x 10(9) / L should undergo an evaluation by the hematologist and the obstetrician. Careful analysis of the time of onset of thrombocytopenia associated to clinical manifestations and specific laboratory tests are essential to provide appropriate diagnosis and maternal - fetal medical care at the right time, when preparing for the homeostatic challenge...

Síndrome de Evans Fisher asociado con esclerodermia / Fisher Evans syndrome associated with scleroderma

El síndrome de Evans Fisher, descrito por primera vez en 1951, es un desorden autoinmune caracterizado por la presencia simultánea o secuencial de anemia hemolítica, trombocitopenia inmune y, en ocasiones, neutropenia inmune; con una prueba de antiglobulina directa positiva. Puede ser de causa primaria o secundaria a otras condiciones, como el lupus eritematoso sistémico, los síndromes linfoproliferativos o inmunodeficiencias primarias. Es muy rara su asociación con la esclerodermia. Con el término esclerodermia, que en sentido literal significa piel dura, se designa un grupo de enfermedades y síndromes que tienen como característica común la induración y el engrosamiento cutáneos. Se caracteriza por la presencia de un depósito excesivo de los componentes del tejido conjuntivo, expresado en forma de fibrosis hística, y por alteraciones estructurales del lecho vascular. Con un cuadro clínico muy amplio, afecta fundamentalmente la piel y ciertos órganos internos, como tubo digestivo, pulmón, corazón y riñón. Se presenta una paciente femenina de 75 años de edad, piel negra, con antecedentes de hipertensión arterial, diabetes mellitus tipo 2, cardiopatía isquémica y esclerodermia, esta última diagnosticada seis meses antes de su ingreso. Acudió por decaimiento marcado, palidez cutáneo-mucosa intensa y petequias generalizadas. En los estudios realizados se detectó anemia y trombocitopenia severas, reticulocitosis, prueba de antiglobulina directa positiva e hipercelularidad medular con hiperplasia severa de los sistemas megacariopoyético y eritropoyético. Se diagnosticó un síndrome de Evans Fisher y se trató con esteroides e inmunomoduladores; se logró la mejoría clínica y la remisión hematológica

The Evans syndrome, first described in 1951, is an autoimmune disorder characterized by the simultaneous or sequential development of hemolytic anemia and immune thrombocytopenia or immune neutropenia. It may be of primary origin or secondary to other diseases or conditions such as systemic lupus erythematosus, lymphoproliferative disorders or primary immunodeficiencies. Its association with scleroderma is considered very rare. The word scleroderma, which literally means hard skin, designates a group of diseases and syndromes of common feature in induration and thickening the skin. It is characterized by the presence of excessive deposition of connective tissue components, expressed as histic fibrosis, and structural alterations of the vascular bed. With a broad clinical view, it primarily affects the skin and certain internal organs such as gastrointestinal tract, lung, heart and kidney. We present a 75 year-old female, black skin, with a history of hypertension, type 2 diabetes, ischemic heart disease and scleroderma, the latter diagnosed six months before admission. The patient referred marked weakness, pale skin and generalized petechiae. The complete blood count detected severe anemia, thrombocytopenia and reticulocytosis. Other studies showed positive direct Coombs test and severe hypercellularity. Evans Fisher syndrome was diagnosed and treated with steroids and immunomodulators; clinical improvement and hematologic remission was achieved

Manejo clínico e obstétrico em gestantes portadoras de hepatite autoimune complicada pela plaquetopenia moderada ou grave / Clinical and obstetrical management of pregnant women with autoimmune hepatitis complicated by moderate or severe thrombocytopenia

OBJETIVO: O presente trabalho tem como objetivo descrever o manejo do pré-natal e do parto em pacientes portadoras de hepatite autoimune associada à plaquetopenia moderada ou grave. MÉTODOS: Este trabalho foi realizado em hospital universitário, de nível terciário. Foram analisadas, retrospectivamente, 13 gestações em dez pacientes com diagnóstico de hepatite autoimune complicadas pela plaquetopenia. Os critérios de inclusão foram: diagnóstico clínico de hepatite autoimune, plaquetopenia moderada ou grave (contagem de plaquetas < 100 x 103/mm3), idade gestacional ao nascimento acima de 22 semanas e pacientes acompanhadas por equipe especializada da instituição. As variáveis estudadas incluíram idade materna, paridade, os regimes de tratamento, contagem de plaquetas, exames para investigação da função hepática, tipo de parto, peso ao nascer e idade gestacional no momento do parto. RESULTADOS: A média da idade materna foi de 24,5 anos (DP = 5,3) e seis (50%) ocorreram em nulíparas. Durante a gravidez, a monoterapia com prednisona foi adotada em 11 (92%) casos. De acordo com o perfil de autoanticorpos, sete (58%) gestações possuíam diagnóstico de hepatite autoimune tipo I, duas (17%) do tipo II e três (25%) eram portadoras de hepatite crônica criptogênica (títulos de autoanticorpos indetectáveis). A hipertensão portal foi caracterizada em 11 (92%) gestações. A idade gestacional média no parto foi de 36,9 semanas (DP = 1,5 semana), com média de peso ao nascer de 2446g (DP = 655g), sendo oito (67%) pequenos para a idade gestacional. No momento do parto, a plaquetopenia grave foi caracterizada em quatro (33%) casos e a cesárea foi realizada em sete (58%). As complicações no parto ocorreram em três casos (25%), uma paciente apresentou atonia uterina e duas, hematoma perineal. Não houve morte materna ou perinatal. CONCLUSÃO: As complicações em pacientes plaquetopênicas com hepatite autoimune são elevadas, no entanto, com os cuidados e atenção necessários, podem ser contornáveis. A associação de duas patologias graves parece aumentar o risco de prematuridade e restrição do crescimento fetal, demandando atenção pré-natal especializada, bem como vigilância do bem-estar do concepto.

OBJECTIVE: To describe the management of prenatal care and delivery in patients bearing autoimmune hepatitis associated with moderate or severe thrombocytopenia. METHODS: This study was performed in a tertiary level university hospital. Thirteen pregnancies in ten patients diagnosed with autoimmune hepatitis, complicated by thrombocytopenia, were retrospectively analyzed. The inclusion criteria were as follows: clinical diagnosis of autoimmune hepatitis, moderate or severe thrombocytopenia (platelet count < 100 x 103/mm3), gestational age at birth over 22 weeks, and patient followed-up by a specialized team at the institution. The variables studied were: maternal age, parity, treatment regimen, platelet count, examinations for investigation of hepatic function, type of delivery, weight at birth, and gestational age at the time of delivery. RESULTS: The average maternal age was 24.5 years (SD = 5.3) and six (50%) occurred in nulliparous women. During pregnancy, monotherapy with prednisone was adopted in 11 cases (92%). According to the autoantibody profiles, seven pregnancies (58%) had the autoimmune hepatitis type I diagnosis, two pregnancies had type II (17%), and three pregnancies (25%) had cryptogenic chronic hepatitis (undetectable titers of autoantibodies). Portal hypertension was featured in 11 pregnancies (92%). The average gestational age at delivery was 36.9 weeks (SD = 1.5 weeks), with an average weight at birth of 2,446 g (SD = 655 g). Eight infants (67%) were small for gestational age. At the time of delivery, severe thrombocytopenia was featured in four cases (33%) and cesarean surgery was performed in seven cases (58%). Complications at delivery occurred in three cases (25%), one patient presented uterine atony, and two patients presented perineal bruising. There was no perinatal or maternal death. CONCLUSION: The complications of thrombocytopenic patients with autoimmune hepatitis are elevated; nevertheless, with appropriate attention and care, they can be resolved. The association between two severe pathologies appears to increase the risk of prematurity and fetal growth restriction, demanding specialized prenatal care, as well as surveillance of newborn well-being.

Myelodysplastic syndrome following essential thrombocytopenia in hypertension a case report

Myelodysplastic Syndrome [MDS] is a disorder of haemopoietic stem cell. Since, it has not been commonly observed in Pakistan, this case study is to understand the scientific and therapeutical comprehension of MDS. An 81 year old male hypertensive patient was presented in a private hospital of Islamabad, Pakistan, with anemia. On medical investigation the physician prescribed him, multi-vitamins OD for a month; injection G-CSF 300 mcg once a week; Molgramostim 300 microg on every alternate day for 3 weeks: Thalidomide 100 mg OD with Alprazolam 0.5 mg at night for 4 weeks and 5'-azacytidine for a month. Clinical and pharmaceutical inaccuracies were observed. Moreover; the high cost and long term therapy are major obstacles to cure this disease. Therefore, affordable method and short-term effective therapy and reduced cost of drugs will help to cure the disease in more efficient way and in less time with more promising results

Síndrome antifosfolípide e trombocitopenia na infância / Antiphospholipid syndrome and thrombocytopenia in childhood / Síndrome antifosfolípido y trombocitopenia en la infancia: relato de caso

OBJETIVO: Relatar o caso de uma criança com diagnóstico de síndrome do anticorpo antifosfolípide associada à trombocitopenia grave e realizar uma revisão de literatura sobre o assunto. DESCRIÇÃO DO CASO: Criança de nove anos e oito meses de idade com trombocitopenia grave associada a anticorpo anticardiolipina positivo. Os dados foram coletados por meio de anamnese, exame físico e exames complementares da paciente. O diagnóstico foi determinado de acordo com os critérios estabelecidos para a síndrome antifosfolípide, associados às manifestações mais comuns na faixa etária pediátrica: livedo reticular e trombocitopenia. COMENTÁRIOS: A síndrome do anticorpo antifosfolípide é uma doença incomum na população pediátrica e suas manifestações clínicas, com a redução do número de plaquetas, devem ser consideradas.

OBJECTIVE: To report the case of a child diagnosed with antiphospholipid syndrome associated with severe thrombocytopenia, and to review the literature on the subject. CASE DESCRIPTION: Child aged nine years and eight months old with severe thrombocytopenia associated with a positive anticardiolipin antibody. Data were collected by clinical history, physical examination, and laboratorial exams. Diagnosis was confirmed according to criteria established for the antiophospholipid syndrome, associated with the presence of the most common manifestations of the syndrome in children: livedo reticularis and thrombocytopenia. COMMENTS: The antiphospholipid syndrome is an uncommon pediatric disease, and clinical manifestations such as decreased platelet number should be considered.

OBJETIVO: Relatar el caso de un niño con diagnóstico de síndrome del anticuerpo antifosfolípido asociado a trombocitopenia grave y realizar una revisión de literatura sobre el tema. DESCRIPCIÓN DEL CASO: Niño de nueve años y ocho meses de edad, con trombocitopenia grave asociada a anticuerpo anticardiolipina positivo. Los datos fueron recogidos por medio de historia, examen físico y exámenes complementarios de la paciente internada en un hospital de Curitiba, en Paraná (Brasil). El diagnóstico fue determinado conforme a los criterios establecidos para el síndrome antifosfolípido, asociados a las manifestaciones más comunes en la franja de edad pediátrica: livedo recticular y trombocitopenia. COMENTARIOS: El síndrome del anticuerpo antifosfolípido es una enfermedad poco común en la población pediátrica, y su manifestación con reducción del número de plaquetas debe ser considerada.

Thrombocytopenia represents a risk for deterioration of liver function after radiofrequency ablation in patients with hepatocellular carcinoma

BACKGROUND/AIMS: We evaluated changes in liver function parameters and risk factors for the deterioration of liver function 12 months after percutaneous radiofrequency ablation (RFA) therapy in patients with hepatocellular carcinoma (HCC). METHODS: The subjects in this retrospective study comprised 102 patients with HCC who had undergone RFA therapy and exhibited no recurrence of HCC 12 months thereafter. Serial changes in serum total bilirubin and albumin, prothrombin time, and Child-Pugh score were evaluated before RFA and 3, 6, 9, and 12 months thereafter. Deterioration of liver function was defined when the Child-Pugh score increased by at least 2 at 12 months after RFA therapy. We determined the factors related to aggravation of liver function after RFA therapy. RESULTS: Liver function had deteriorated 12 months after RFA in 29 patients (28.4%). Serum albumin levels decreased significantly from before (3.7+/-0.1 g/dL, mean+/-SD) to 12 months after RFA therapy (3.3+/-0.1 g/dL, P=0.002). The Child-Pugh score increased significantly during the same time period (from 6.1+/-0.2 to 7.2+/-0.3, P<0.001). Pre-RFA thrombocytopenia (< or =100,000/mm3) was revealed as a significant risk factor for the deterioration of liver function after RFA. However, no patients had episodes of bleeding as a complication of RFA. CONCLUSIONS: Among the liver-function parameters, serum albumin level was markedly decreased in HCC patients over the course of 24 months after RFA therapy. A pre-RFA thrombocytopenia represents a major risk factor for the deterioration of liver function.