Trombocitopenia neonatal: Revisión. II. Trombocitopenias no inmunes; transfusión de plaquetas / Neonatal thrombocytopenia: A review.II. Non-immune thrombocytopenia; platelet transfusion

Las trombocitopenias de causa no inmunológica son ocasionadas por múltiples patologías; las más frecuentes son las debidas a infecciones extra- o intrauterinas y las secundarias a otras patologías involucradas en la interrelación niño-placenta-madre. En este segundo artículo, se enumeran sus causas y se describen en detalle las distintas patologías. La transfusión de plaquetas es ampliamente utilizada en neonatología, tanto para tratamiento como para profilaxis de hemorragias. Sin embargo, no hay aún consenso generalizado sobre el umbral de recuento plaquetario conveniente para indicar la transfusión ni sobre sus reales indicaciones. Se comentan artículos recientes sobre las distintas estrategias propuestas. Se enfatiza la discusión sobre los múltiples efectos adversos de las transfusiones de plaquetas, cuyo conocimiento está cambiando el paradigma relativo a sus indicaciones, lo que sugiere que se debe aplicar una política mucho más restrictiva al respect

Non-immune thrombocytopenia is caused by multiple pathologies; the most common causes are extra- or intrauterine infections, whereas secondary cases result from other pathologies involved in the fetal-placental-maternal interface. This second article lists its causes and provides details of the different pathologies. Platelet transfusion is widely used in neonatology, both as treatment and as bleeding prophylaxis. However, there is no general consensus about the platelet count threshold that is convenient to indicate a transfusion or actual indications. Recent articles are commented regarding the different proposed strategies. The emphasis is on discussing the multiple adverse effects of platelet transfusions because knowledge about them is changing the paradigm for indications, suggesting that a much more restrictive policy is required

Increased platelet distribution width and reduced IL-2 and IL-12 are associated with thrombocytopenia in Plasmodium vivax malaria

BACKGROUND Thrombocytopenia in malaria involves platelet destruction and consumption; however, the cellular response underlying this phenomenon has still not been elucidated. OBJECTIVE To find associations between platelet indices and unbalanced Th1/Th2/Th17 cytokines as a response to thrombocytopenia in Plasmodium vivax infected (Pv-MAL) patients. METHODS Platelet counts and quantification of Th1/Th2/Th17 cytokine levels were compared in 77 patients with uncomplicated P. vivax malaria and 37 healthy donors from the same area (endemic control group - ENCG). FINDINGS Thrombocytopenia was the main manifestation in 55 patients, but was not associated with parasitaemia. The Pv-MAL patients showed increases in the mean platelet volume (MPV), which may be consistent with larger or megaplatelets. Contrary to the findings regarding the endemic control group, MPV and platelet distribution width (PDW) did not show an inverse correlation, due the increase in the heterogeneity of platelet width. In addition, the Pv-MAL patients presented increased IL-1β and reduced IL-12p70 and IL-2 serum concentrations. Furthermore, the reduction of these cytokines was associated with PDW values. MAIN CONCLUSIONS Our data demonstrate that an increase in MPV and the association between reductions of IL-2 and IL-12 and PDW values may be an immune response to thrombocytopenia in uncomplicated P. vivax malaria.

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.

Small clonal B-cell population in the bone marrow as a possible tool in the diagnosis of occult primary parotid lymphoma / Pequeña población de células B monoclonales en medula ósea como posible herramienta diagnóstica en el linfoma oculto primario de parótida

Case Description: An 82-years old Hispanic woman with a past medical history significant for pulmonary thromboembolism on oral anticoagulation, rheumatoid arthritis, and hypertension developed a new onset thrombocytopenia. Clinical Findings: Small clonal B-cells populations (SCBP) also known as monoclonal B-cell lymphocytosis was found as part of the workup for an idiopathic thrombocytopenia and lead ultimately to the diagnosis of parotid primary follicular lymphoma coexisting with Warthin tumor involving the bone marrow in a small extent and oncocytic papilloma located in the maxillary sinus. Treatment and Outcome: Patient was treated with Rituximab monotherapy with improvement on her platelet count. Clinical relevance: Although it is unclear the role of this clonal cells, they may work as a possible diagnostic tool for occult lymphomas. Further prospective studies are needed to confirm this possible association.

Descripción de caso: Mujer hispana de 82 años con una historia médica significativa de tromboembolismo pulmonar en anticoagulación, artritis reumatoide e hipertensión, la cual desarrolló recientemente una trombocitopenia. Hallazgos clínicos: Una pequeña población de células B monoclonales también conocida como linfocitosis monoclonal de células B fue encontrado dentro del estudio de una trombocitopenia idiopática que conllevó al diagnóstico de un linfoma folicular primario de parótida coexistiendo con un tumor de Warthin y un papiloma oncocítico localizado en el seno maxilar. Tratamiento y resultado: La paciente fue tratada con monoterapia de Rituximab con una mejoría en su conteo de plaquetas. Relevancia clínica: Aunque el rol de las pequeñas poblaciones B monoclonales no está completamente dilucidado, podrían tener una aplicación como herramienta diagnóstica. Futuros estudios prospectivos son necesarios para confirmar esta posible asociación.

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)

Unusual yet isolated oral manifestations of persistent thrombocytopenia: a rare case report

Patients with platelet-mediated disorders often present clinical manifestations of bruising and bleeding. Although these changes are detected most frequently on the skin, the oral cavity also may exhibit signs of bleeding. In this report, we describe a patient who presented isolated oral features of hemorrhagic bullae with bleeding, indicative of a bleeding disorder. Results of laboratory tests revealed severe thrombocytopenia and a careful history disclosed dengue fever as the cause. The importance of recognizing the oral manifestations of thrombocytopenia is highlighted here, since the oral cavity is a frequent site of hemorrhage and could be the only manifestation of the disease.

Variaciones hematológicas en pacientes con malaria causada por Plasmodium vivax antes, durante y después del tratamiento / Hematologic variations in patient with malaria caused by Plasmodium vivax before, during and after treatment

Para determinar la relación entre parámetros hematológicos, parasitemia y episodios maláricos, se evaluaron 59 individuos de ambos sexos, infectados con Plasmodium vivax y 30 controles del estado Sucre. Se extrajeron muestras de sangre por punción venosa y del lóbulo de la oreja el día del diagnóstico, a los 8 y 30 días postdiagnosis. Se realizó hematología completa, diagnóstico microscópico y molecular (PCR) y grado de parasitemia. Se encontraron diferencias significativas por sexo para la hemoglobina, eritrocitos y hematocrito (p < 0,01 en los tres análisis). Estos valores en los hombres disminuyeron para el día 8 del tratamiento con respecto al día 0 (p < 0,001; p = 0,006 y p = 0,025, respectivamente). En las mujeres, se observaron diferencias sólo entre los controles y los pacientes a las fechas de muestreo para hemoglobina y hematocrito (p < 0,001). El número promedio de los leucocitos para el día diagnóstico se ubicó dentro de los parámetros de referencia, pero significativamente por debajo (p < 0,001) de los del grupo control y de los obtenidos para durante y después del tratamiento. Los valores de eosinófilos se observaron por encima de los parámetros normales en los controles y los pacientes durante y después del tratamiento. Se observó trombocitopenia el día diagnóstico, pero a la semana del tratamiento los valores aumentaron significativamente (p < 0,001). La correlación de los parámetros evaluados con la parasitemia demostró una relación negativa con las plaquetas, y una positiva con la diferencia de plaquetas entre el día diagnóstico y día 8 de tratamiento (p = 0,014). Esta diferencia fue el único parámetro que se correlacionó con los episodios maláricos (p = 0,040). La trombocitopenia demostró ser un indicador de malaria aguda.

In order to determine the relationship between hematological parameters, parasitaemia and malaria episodes, we evaluated 59 individuals of both sexes, infected with Plasmodium vivax and 30 controls from Sucre state. Blood samples were obtained by venous puncture and from the earlobe at day of diagnosis, 8 and 30 days post-diagnosis. We carried out hematological analysis, microscopic and molecular (PCR) diagnosis and the parasitaemia was calculated. There were significant differences by sex for hemoglobin, erythrocytes and hematocrit (p < 0,01 in all three analysis). These values in males, decreased by day 8 with respect to day 0 (p < 0,001; p = 0,006 y p = 0,025, respectively). In females, significant differences were only seen in hemoglobin and hematocrit between controls and patients (p < 0,001). The average number of leucocytes at the day of diagnosis was within the reference values, but slightly lower compared to the controls and the samples during and after the treatment. The average proportion of eosinophils was higher than normal for both, controls and patients, during and after the treatment. Thrombocytopenia was observed at diagnosis, but a week after, the values increased significantly (p < 0,001). There was an inverse relationship between parasitaemia and platelet count and a direct relationship between the first and hemoglobin at day 0, as well as with the difference in platelet counts between day 0 and day 8 (p = 0,044 y p = 0,014, respectively). This difference was the only parameter related to the number of malaria episodes (p = 0,040). Thrombocytopenia showed to be an indicator of acute malaria.

Evaluación del paciente con trombocitopenia / Evaluation of patients with thrombocytopenia

La trombocitopenia, como la fiebre y la anemia, en un signo que en todos los casosdebe aclararse su etiología. La trombocitopenia se presenta por falta de producción delas plaquetas o por aumento en la destrucción de las mismas. En todos los casos en dondese detecte trombocitopenia, el primer paso es descartar una posible seudotrombocitopenia.Una vez confirmada la trombocitopenia, con la ayuda de la clínica y los estudios complementarios,el médico deberá estar en condiciones de esclarecer la etiología antes de iniciarcualquier tratamiento. En este módulo se presentan las herramientas para el diagnóstico dela seudotrombocitopenia y de la trombocitopenia.

Central nervous system hemorrhage in thrombocytopenic patients: computed tomographic findings in 21 cases

OBJETIVE: To describe the CT scan findings of 21 thrombocytopenic patients with central nervous system (CNS) hemorrhage. METHOD: Retrospective study of the computed tomography (CT) of 21 platelet-depleted patients with CNS hemorrhage. One patient presented two episodes of hemorrhagic episode with different intervals. The clinical data were obtained by the review of the medical records. Two radiologists analyzed the films and reached the decisions by consensus. The following findings were studied: type of bleeding, number of lesions, topography, laterality, size and associated findings. RESULTS: Intraparenchymal hemorrhage (IPH) was the most common findings, found in 20 cases, being six of them associated with subarachnoid and intraventricular hemorrhages. The size of the lesions varied between 1.8 and 10.5 cm. The parietal lobes were more commonly affected (n=11, 50 percent), followed by the temporal (n=7, 31.8 percent), frontal (n=7, 31.8 percent) and occipital (n=2, 9.09 percent) lobes. In 15 cases (68.2 percent) there was a single area of hemorrhage and in the remaining cases there were multiple hemorrhages. Associated findings were found in 20 cases. The most prevalent were edema (n=17, 77.3 percent), hydrocephalus (10, 45.4 percent) and midline shift (n=9, 41 percent). CONCLUSION: The most frequent CT scan findings in thrombocytopenic patients with CNS hemorrhage are single IPH, located mostly in the parietal, temporal and frontal lobes, with varied sizes and associated with edema, hydrocephalus and midline shift.

OBJETIVO: Descrever os achados tomográficos de 21 pacientes trombocitopênicos com hemorragia no sistema nervoso central (SNC). MÉTODO: Estudo retrospectivo das tomografias computadorizadas (TC) de 21 pacientes trombocitopênicos que apresentaram hemorragia no SNC. Um dos pacientes apresentou 2 episódios hemorrágicos em épocas diferentes. Os dados clínicos foram obtidos por revisão de prontuários médicos. Dois radiologistas analisaram os exames e estabeleceram os achados por consenso. Os seguintes achados foram estudados: tipo de sangramento, número de lesões, topografia, lateralidade, tamanho e achados associados. RESULTADOS: A hemorragia intraparenquimatosa foi o achado mais comum, observada em 20 casos, sendo que em seis deles apresentavam hemorragia subaracnóidea e intraventricular associadas. O tamanho das lesões variou entre 1,8 e 10,5 cm (mediana= 4,5 cm). Os lobos parietais foram mais acometidos (n=11, 50 por cento), seguidos pelos temporais (n=7, 31,8 por cento), frontais (n=7, 31,8 por cento) e occipitais (n=2, 9,09 por cento). Em 15 casos (68,2 por cento) houve uma única área de hemorragia, e nos demais múltiplas áreas foram observadas. Em 20 casos foram encontrados achados associados, sendo mais comum edema (n=17, 77,3 por cento), hidrocefalia (n=10, 45,4 por cento) e desvio da linha média (n=9, 41 por cento). CONCLUSÃO: Os achados tomográficos mais freqüentes em pacientes trombocitopênicos com hemorragia cerebral são lesões intraparenquimatosas únicas acometendo principalmente os lobos parietais, temporais e frontais, com tamanhos variados e associadas a edema, hidrocefalia e desvio da linha média.

Kasabach-Merritt syndrome: a case report.

Kasabach-Meritt syndrome is a combination of thromobocytopenia, hemolytic anemia, and acute or chronic consumptive coagulopathy in association with rapidly enlarging hemangioma. A male infant of 5 days was admitted in paediatric ward with this syndrome. The baby had ecchymotic patches over face and extremities and bleeding through umbilical stump. The child expired due to severe thrombocytopenia with consumptive coagulopathy leading to precipituous hemorrhage superimposed by septicemia. An autopsy was performed which confirmed retroperitoneal lesion as kaposiform hemangioendothelioma.

Trombocitopenia induzida por heparina / Heparin-induced thrombocytopenia

O objetivo deste relato é fazer uma revisão a fim de obter informações atualizadas sobre trombocitopenia induzida por heparina, suas manifestações clínicas, seu diagnóstico e seu manejo terapêutico. Após, concluímos que a trombocitopenia induzida por heparina é uma complicação comum em pacientes submetidos a tratamento com heparina, indiferentemente da doença de base. Complicações trombóticas potencialmente fatais têm sido descritas. Por essa razão, se houver suspeita de trombocitopenia induzida por heparina, uma abordagem adequada incluindo suspensão precoce da heparina é mandatória. Produtos que substituem a heparina incluem hirudina e danaparóide sódico. Heparina de baixo peso molecular é contra-indicada

The aim of this paper isto review current information about the clinical manifestations, diagnosis and management of heparin-induced thrombocytopenia. This was achieved by a bibliographic review using Public Medline and consulting Hematology textbooks. From this study we concluded that heparin-induced thrombocytopenia is a common complication of patients exposed to heparin herapy regardless of underlying conditions. Potentially fatal thrombotic complications have been reported. Terefore, if heparin-induced thrombocytopenia is suspected, and adequate approach including early heparin discontinuation is mandatory. Alternative danaparoid sodium. Low-molecular-weight heparin is contratindicated.

Bone marrow pathology of culture proven typhoid fever

The authors analysed bone marrow findings of sixteen cases of culture proven typhoid fever to reveal the pathologic changes according to the disease stage. The most frequent finding was chronic granulomatous inflammation (eight cases). Infection (bacteria) associated hemophagocytic syndrome (four cases), reactive marrow (two cases), and non specific findings (two cases) were also encountered. Granulocytic hyperplasia with hemophagocytosis appeared at the early stage and was followed by infection (bacteria) associated hemophagocytosis and granuloma in proliferative stage. In lysis (late) stage, granulomatous inflammation was noted. However, resolution of granulomatous inflammation was not distinct. Some nuclear debris and phagocytosis were remarkable in well-formed granulomas. Thrombocytopenia was the most remarkable peripheral blood finding at the time of biopsy. Anemia, leukopenia, and pancytopenia were also observed in descending order.

Acute myeloid leukemia-FAB classification and its correlation with clinico-Hematological features

The French-American and British [FAB] classification of 62 consecutive cases of acute myeloid leukemia was undertaken. AML-M2 was the commonest FAB type [32.26%], followed by Ml and M4 [22.58% each], M5 [8.6%] and M6 and M7 [1.61% each], respectively. One of the patients was diagnosed as AML-MO [not a FAB type]. The mean age of Ml, M2, M3 and M5 cases was between 25and 29 years, whereas in M4 patients it was 45.6 years. AML-M2, M4 and M5 were commoner in males, Ml in females and M3 equal in both sexes. Feeling of weakness, easy fatiguability and pallor were invariably present in all FAB types. All the patients of Ml and M5, 85% of M2, 64% of M 4 and 50% of M3 presented with fever. Bleeding manifestations were most frequent in M3 cases followed by M5, Ml, M4 and M2, respectively. Hepatomegaly and splenomegaly were relatively less prominent features in M3 as compared to other FAB types. Amongst the haematological parameters, anaemia was more severe in M1, leucocytosis in M2 and thrombocytopenia in M3 cases as compared to other FAB types

Casos anatomoclínicos: mujer de 64 años de edad con anemia hemolítica, trombocito penia ya alteraciones neurológicas / Anatomo-clinical cases: female 64 years-old with anemia hemolytic, thrombocytopenia and neurological changes

M.P.J.S. Femenino de 64 años de edad con antecedentes heredofamiliares irrelevantes y personales de alcoholismo, tabaquismo y toxicomanias negados, hipertensión arterial de 25 años de evolución e historia de "gastritis erosiva" un mes antes de su ingreso. Antecedentes ginecoobstétricos de menarca a los 13 años, menopausia a los 40 años, Gesta VI, Para V, Cesarea I, Abortos O. Acude al Instestino por un padecimiento de 6 meses de evolución caracterizado por astenia, adinamia y palidez progresiva de tegumentos. Cuatro meses después se agregan evacuaciones melénicas, equimosis, petequias, gingivorragias, pirosis e hipoacusia izuierda. En otro hospital se hace el diagnóstico de "gastritis erosiva" prescribiendo 900 mg/día de Cimetidina con lo que mejora la pirosis pero no la hipoacusia aumentando el número de peteuias. Ocho días antes de su ingreso se agrega cefalea parieto occipital, fotofobia, derrame en globo ocular izquierdo y vómitos. Acudió al Servicio de Urgencias de este hospital y posteriormente a hematología donde se realizan exámenes y se sospecha el diagnóstico de un síndrome de Fisher-Evans, iniciando 75 mg diarios de prednisona, dosis que se incrementó un día después a 100 mg. Un día antes de ingresar a hospitalización se agregan dolor torácico, palpitaciones, diaforesis, lipotimia y mayor palidez de tegumentos, una biometría hemática mostró hemoglobina de 6.7 g/dl, leucocitosis (16,050 x cc), transaminasas normales, DHL 365 U, 18000 plaquetas y 35% de reticulocitos en sangre periférica. Coombs directo negativo. Recibió transfusión con 2 paquetes globulares ...