Trastornos de coagulación en el cirrótico / Coagulation disorders in cirrhosis

The liver plays a central role in the clotting process. In this organ are sintetizated the major part of the coagulation factors. Historically, was considered that alteration in liver function causes important bleeding disorders. However, actual evidence is not in agreement with this asseveration. Decreased synthesis of clotting and inhibitor factors, decrease clearance of activated factors, quantitative and qualitative platelet defects, hyperfibrinolysis and intravascular coagulation are some of the defects observed in liver diseases. Thrombotic events, even if rare in cirrhotic patients, occur manly in the portal and mesenteric veins. The aim of the present work is to review the present evidence in coagulation disorders and liver disease.

El hígado participa de manera importante en el proceso de la coagulación. En él se sintetizan la mayor parte de los factores pro- y anticoagulantes. De manera histórica se ha considerado que las alteraciones en la función de este órgano provoca trastornos predisponentes para eventos de sangrado. La evidencia actual pone en tela de juicio esta aseveración. En los casos de hepatopatía se hacen evidentes alteraciones en el número y funcionamiento de las plaquetas, disminución de la síntesis de factores de la coagulación, disfibrinogenemia, alteraciones en la fibrinólisis, deficiencia de vitamina K y cambios similares a los ocurridos en la coagulación intravascular diseminada (CID). El presente trabajo está dirigido a revisar los conocimientos actuales respecto a las alteraciones de la coagulación presentes en los pacientes con hepatopatías.

Platelet aggregation abnormalities in end-stage renal disease; a possible cause of hemorrhagic diathesis

End-stage renal disease patients [ESRD] are at risk of hematological complications. Hypercoagulability and paradoxically hemorrhagic tendency have been described in uremic patients. Hemorrhagic complications are primarily due to uremic platelet dysfunction and intermittent anticoagulation therapy used in hemodialysis. The aim of this study was to investigate the effect of hemodialysis [HDD] on platelet aggregation abnormalities and hence its role in bleeding in ESRD patients on maintenance hemodialysis. Forty two patients with ESRD on maintenance HD, in the hemodialysis unit at Sohag University Hospital utilizing polysulphon memrance and using Fresenius machines 94008 B] were included in this study and compared with a control group; of fourteen healthy adult volunteers. Blood samples were obtained for laboratory investigations form patients and controls before HD session and after the session from patients only the following laboratory investigations were performed; Complete blood cont [CBC]. serum urea, creatinine, Bilirubin, AST, ALT and hepatitis markers [HBsAg and anti HCV antibody]. tests for coagulation screening; PT and PTT, Study of platelet aggregation with ADP and Ristocetin. For all patients and controls bleeding time [BT] was performed at the time of sample taking. Bleeding time is significantly prolonged after the HD session comparing to results before the HD session and that of controls, platelet aggregation with ADDP and Ristocetin were significantly decreased in HD patients before and after session comparing to controls and also statistically significant comparing the results of patients before and after HD session. There is no statistically significant difference in the results of INR and PTT when comparing results of patients before and after sessions and when comparing the results of patients to controls, HD sessions in ESRD patients lead to platelet aggregation abnormalities which may resulted in hemorrhagic disorders in HD dependent patients

Osteogenesis imperfecta with bleeding diathesis.

Osteogenesis imperfecta (OI) is a disorder of type 1 collagen synthesis with varied complications in form of brittle bones, hearing loss, dental, skin, and ocular changes. Osteogenesis imperfecta may have bleeding diathesis rarely due to defective vascular integrity for the same reason. Here we come across a rare case of Sillence type IB Osteogenesis imperfecta with bleeding in the form of repeated epistaxis and petechial rash, which seem to have a collagenous link.

Hemorrhagic Cystitis Following Allogeneic Hematopoietic Cell Transplantation

We conducted a retrospective study to investigate the incidence, risk factors, and clinical features of hemorrhagic cystitis (HC) following allogeneic hematopoietic cell transplantation (allo-HCT). Adult patients who developed HC after allo-HCT were identified from the HCT database of the Asan Medical Center and their medical records were reviewed. From December 1993 to August 2001, a total of 210 adult patients underwent allo-HCT. Fifty-one patients developed HC with a cumulative incidence of 25.7%. The median onset of HC was post-transplant day 24 (range, -2 to 474), and the median duration was 31 days (range, 8 to 369). Significant risk factors for HC by univariate analysis included diagnosis of chronic myelogenous leukemia (p=0.028), unrelated HCT (p=0.029), grade III-IV acute graft-versus-host disease (GVHD) (p<0.001), extensive chronic GVHD (p=0.001), and positive cytomegalovirus antigenemia between post transplant days 31 and 60 (p=0.031). Multivariate analysis showed that grade III-IV acute GVHD was the most important risk factor for the occurrence of HC after allo-HCT (odds ratio, 3.38; 95% CI, 1.36-8.39). Late-onset HC, which occurred beyond 3 weeks after allo-HCT, was more frequently associated with GVHD than earlyonset HC (p=0.007). Our data suggest that a portion of late-onset HC might be a manifestation of GVHD.

Síndrome hemorrágica de Altamira / Altamiras' hemorragic syndrome

Este artigo tem como objetivo discutir as principais características da síndrome hemorrágica de Altamira, enfocando aspectos etiopatogênicos, epidemiológicos, manifestações clínicas, diagnóstico laboratorial, tratamento e prevenção

Síndrome hemorrágico por contacto conorugas del género Lonomia (Saturniidae en Casanare, Colombia: informe de dos casos / Hemorrhagic syndrome caused by Lonomia (Saturniidae) caterpillars in Casanare, Colombia

El presente artículo describe los dos primeros casos de accidentes por orugas de Lonomia diagnósticados en Colombia. Estos insectos ocasionan un cuadro local con alteraciones hemorrágicas caracterizadas por una intensa actividad fibrinolítica y valores bajos de diferentes factores de la coagulación. Los dos casos se presentaron en el departamento de Casanare y fueron remitidos al Hospital San Carlos de Bogotá. La evolución de cada uno fue diferente: el primero, con hemoptisis e insuficiencia renal secundaria, que le causó la muerte a la paciente; el segundo, con gingivorragia y sangrado vaginal que evolucionó hasta la completa mejoría después de la adminitración del sueo anti-Lonomia. Descritos estos primeros casos, es necesario ampliar el conocimiento sobre el tema y trabajar en su prevención

Síndrome cardiopulmonar por hantavirus en 21 adultos en la IX Región de Chile / Hantavirus cardiopulmonary syndrome in 21 adult cases at IX region, Chile

Veintiun adultos, con síndrome cardiopulmonar por hantavirus (SCPH) fueron atendidos en Temuco. El 95 por ciento adquirió la enfermedad en su lugar de trabajo altamente infestado de ratones, en la zona cordillerana andina o central. Las manifestaciones iniciales fueron fiebre y mialgias. La mitad tuvo molestias abdominales y un tercio, tos. Posteriormente (1 a 7 días), apareció subitamente insuficiencia respiratoria y/o inestabilidad hemodinámica. Todos desarrollaron edema pulmonar, 10 shock cardiogénico refractario y 6 hipotensión. Hemorragias ocurrieron en 71 por ciento. Todos tuvieron hemoconcentración y trombocitopenia y más del 95 por ciento, leucocitosis. La gran mayoría cursó con hiponatremia, alteración de pruebas de coagulación, creatinfosfoquinasa, deshidrogenasa láctica y creatininemia. La radiografía de tórax mostró infiltrados intersticiales alveolares o mixtos, con o sin derrame pleural asociado. Todos recibieron soporte cardiorrespiratorio y antibióticos. El 43 por ciento fue tratado con corticosteroides. El shock cardiogénico, el edema pulmonar y las hemorragias fueron las principales causas de muerte

Haemostatic disorders in nonsplenectomized and splenectomized thalassaemic children

A group of 40 thalassaemic patients [20 splenectomized and 20 nonsplenectomized] from the Haematology Unit of Tanta University Hospital [age range: 3-14 years] were studied to identify the mechanisms by which haemorrhagic and thrombotic complications occur in thalassaemic patients. The patients' levels of protein C, antithrombin III and in vitro platelet aggregation in response to collagen were compared with those of 20 controls. The study suggests that thrombocytosis, increased platelet aggregation and decreased natural coagulation inhibitors [protein C and antithrombin III] in splenectomized thalassaemic children may be significant in thrombotic complications in such patients. Defective platelet aggregation and prothrombin activity in nonsplenectomized children may also give rise to haemorrhagic tendencies

Autopsy study of patients dying of bleeding diathesis.

Ten thousand & thirty seven autopsies performed from the year 1982 to 1992 were studied retrospectively, to find out the number of deaths due to bleeding diathesis. Eighty-seven (0.87%) patients died due to bleeding diathesis, out of which haemolytic uraemic syndrome (HUS) was seen in 9 cases (10.34%), disseminated intravascular coagulation (DIC) in 67 cases (77.01%) & 11 cases were grouped as miscellaneous. Martius scarlet blue stain was carried out to demonstrate fibrin & depending on the number of thrombi in the glomerulus & blood vessels, the lesions were graded as mild, moderate or severe. Kidney was the most common organ involved in all groups of bleeding diathesis. In DIC kidney & lung involvement was almost equal.

Severe and complicated malaria in Bikaner (Rajasthan), western India.

Severe and complicated malaria is an important cause of mortality in Plasmodium falciparum infection. We describe in this study the details of 532 cases of such syndromes admitted to hospital during an outbreak of malaria between September-December 1994. Increase in the annual rain fall, collection of water around Indra Gandhi Canal, forestation of shrubs around it and migration of labor, adaptation of Anopheles stephensi to desert climate and favorable breeding of An. culicifacies in the areas under impact of irrigation were presumptive causes of the outbreak in this region. Cerebral malaria (25.75%), hepatic involvement (11.47%), spontaneous bleeding (9.58%), hemoglobinuria (7.89%), severe anemia (5.83%), algid malaria (5.26%), ARDS (3%) and renal failure (2.07%) were the important manifestations. The overall mortality was 11.09%, which was high because of infection in the non-immune population of this area. Ignorance about the severity of this disease and lack of transportation facility was another important factor. Morality was highest in ARDS (81.25%) followed by severe anemia (70.97%), algid malaria (46.43%), renal failure (45.45%), jaundice (36.06%) and cerebral malaria (33.57%). Pregnancy was an important determinant increasing the mortality in female patients. Mortality was very high (82.35%) in those persons who presented with more than 3 syndromes together.

Síndrome de Kasabach-Merritt: actualización / Kasabach-Merritt's syndrome: actualization

El síndrome de Kasabach-Merritt consiste en un cuadro caracterizado por trombocitopenia, anemia hemolítica microangiopática y coagulopatía por consumo asociado a un hemangioma que crece rápidamente. La mortalidad potencial es alta, pero esto se modifica cuando el hemangioma comienza a involucionar, sea por la terapéutica instaurada o en forma espontánea. Urgencia dermatológica

Infected-associated hemophagocytic syndrome.

An epidemic of an infection associated with circulating hemophagocytes (HP) and activated monocytes (AM) was seen in Bombay. Although certain features overlapped with the well-defined entity of virus-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis, it was distinct enough to place it in a separate category. Affected children were predominantly two days to two years of age. They had fever, altered sensorium, neurological symptoms, dyspnea, and/or diarrhea, and significant bleeding. Laboratory tests showed neutrophilia, AM and HP's in every blood smear, coagulopathy, normal cerebrospinal fluid, normal liver transaminases, hypertriglyceridemia, and hypoalbuminemia. Surgical cases were remarkable in that they had small bowel malformations. These cases were subdivided into four distinct groups based on age of presentation, neonates, infants, children and a surgical group. The clinical differences in each group are described.

Acute promyelocytic leukemia is a distinct subset of acute myelocytic leukemia with unique clinicopathologic characteristics including longer duration of relapse free survival: experience in 13 cases

Acute promyelocytic leukemia(APL) is a subtype of acute myelocytic leukemia(AML) associated with unique features such as the presence of atypical promyelocytes and bleeding tendency due to disseminated intravascular coagulation(DIC). In a retrospective study, we analyzed 96 cases of AML seen at our hospital between June, 1989 and December 1993. Thirteen cases of APL(14%) were identified and their clinicopathologic characteristics were analyzed. The 86 cases of other types of AML served as controls. The distinct clinicopathologic features of APL as contrasted to other types of AML included younger age of patients, shorter duration of symptom before diagnosis, higher level of albumin at presentation, and a higher proportion of patients having coagulation abnormalities (75 vs. 25%). Bone marrow cellularity was higher in APL when compared to other types of AML (100 vs. 90%, P = 0.013). Of 13 patients with APL, 4 died of bleeding/sepsis between days 2 to 4 after admission. Seven of 9 patients who received induction therapy achieved complete remission(CR). CR rate in APL was similar to other types of AML (78 vs. 64%, P = 0.743). Five of seven patients who achieved CR remain in continuous CR at 9+ to 42+ months. CR duration is significantly longer in APL when compared to other types of AML (P = 0.029). In conclusion, this study showed that APL is a distinct entity among subtypes of AML with clinically significant bleeding tendency and rapidly fatal course if untreated. With appropriate antileukemic therapy, CR can be achieved in the majority of patients and the patients show a longer duration of CR when compared to other types of AML.

Essential thrombocythemia: a hypercoagulable state

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

Albinismo tirosinasa positivo asociado a transtorno de la agregación plaquetaria / Tyrosinase positive albinism associated with platelet aggregation disorders

Los autores estudian un caso de albinismo tirosinasa positivo con trastorno de la agregación plaquetaria. No se pudo comprobar la existencia de material ceroide en los macrófagos de la médula ósea. Pensamos, sin embargo que se trataba de una variente del síndrome de Hermansky-Pudlak, equiparable a un caso similar publicado en la literatura