1/198. Spontaneous intrahepatic hemorrhage and hepatic rupture in the hellp syndrome: four cases and a review.Subcapsular hemorrhage and hepatic rupture are unusual catastrophic complications of the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. A high index of suspicion and prompt recognition are keys to proper diagnosis and management of affected patients. The optimal management of these patients is evolving. An aggressive multidisciplinary approach has considerably improved the morbidity and mortality associated with these complications. We present our experience with four cases of hepatic hemorrhage occurring in association with the hellp syndrome and review the literature on this subject. All of our patients were multiparous, and three had a history of eclampsia/preeclampsia in a previous pregnancy. All four patients developed intrahepatic hemorrhage; two developed hepatic rupture requiring surgical intervention. Three patients developed disseminated intravascular coagulation and acute renal failure. Two patients developed pericardial effusion, pleural effusions, and ascites. One patient died of septic complications after multiple surgical interventions.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
2/198. MR imaging of a hemorrhagic and granulomatous cyst of the ligamentum flavum with pathologic correlation.cysts of the ligamentum flavum are uncommon causes of neurologic signs and symptoms and usually are seen in persons over 50 years of age. We report a case of an epidural cyst located in the ligamentum flavum, which contributed to spinal stenosis in a 30-year-old man. Radiologic features were similar to those of a synovial cyst, but synovium was not identified histologically. The imaging and pathologic features were unusual, including hemorrhage and a fibrohistiocytic reaction with giant cells.- - - - - - - - - - ranking = 0.0046305836647322keywords = giant (Clic here for more details about this article) |
3/198. Functional, life-threatening disorders and splenectomy following liver transplantation.splenectomy (SPL) in cirrhotic patients undergoing liver transplantation (LTx) may resolve specific problems related to the procedure itself, in case of functional and life-threatening clinical situations often occurring as a result of liver cirrhosis and portal hypertension. METHOD: A single-center experience of ten splenectomies in a series of 180 consecutive adult liver transplant patients over a period of 6 yr is reported. The mean patient age was 46.8 /- 9.5 yr (range 25 57 yr). Indications for SPL were post-operative massive ascitic fluid loss (n = 3), severe thrombocytopenia (n = 3), acute intra-abdominal hemorrhage (n = 2), infarction of the spleen (n = 1), and multiple splenic artery aneurysms (n = 1). RESULTS: Extreme ascites production due to functional graft congestion disappeared post-SPL, with an improvement of the hepatic and renal functions. SPL was also effective in cases of thrombocytopenia persistence post-LTx, leading to an increase in the platelet count after about 1 wk. Bleeding episodes related to left-sided portal hypertension or trauma were also resolved. The rejection rate during hospitalization was 0%, and no other episodes were recorded in the course of the long-term follow-up. However, sepsis with a fatal outcome occurred in 4 patients, i.e. between 2 and 3 wk post-SPL in three cases and 1 yr after the procedure as a result of pneumococcal infection in the last case. Fatal traumatic cranial injury occurred 3 yr post-LTx in another case. Five patients (50%) are still alive and asymptomatic after a median follow-up period of 36 months. CONCLUSION: The lowering of the portal flow appears to resolve unexplained post-operative ascitic fluid loss as a result of functional graft congestion following LTx. However, because of the enhanced risk of SPL-related sepsis, a partial splenic embolization (PSE) or a spleno-renal shunt could be used as an alternative procedure because it allows us to preserve the immunological function of the spleen. SPL is indicated in case of post-transplant bleeding due to left-sided portal hypertension and trauma, spleen infarction, and to enable prevention of hemorrhage in liver transplant patients with multiple splenic artery aneurysms. Severe and persistent thrombocytopenia could be treated with PSE. Because the occurrence of fatal sepsis post-SPL is a major complication in LTx, functional disorders, such as ascites and thrombocytopenia, should be treated with a more conservative approach.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
4/198. Von Willebrand disease as cause of unanticipated bleeding following adeno-tonsillectomy.Von Willebrand disease (vWD) is a frequent autosomal bleeding disorder. We report two unsuspected patients over 6 years of age with this disease operated by tonsillectomy and adenoidectomy (T&A) at our Department. Preoperative hematologic work-up (with activated partial thromboplastic time, prothrombin time, fibrinogen, and platelet count) was normal. Both patients had undergone previous adenoidectomy without complications. In both cases, profuse bleeding was noted in the immediate postoperative period. The therapeutic measures in these situations are discussed and a review of the current literature concerning preoperative hematologic evaluation of T&A is included.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
5/198. Systemic lupus erythematosus presenting with haemorrhagic manifestation.A 26-year-old female presented with an episode of severe mucus membrane bleeding. Investigations revealed prolonged prothrombin time (PT), and partial thromboplastin time (PTT), normal thrombin time (TT) and reptilase time, thrombocytopenia, a positive test for lupus anticoagulant (LA), as well as anti-cardiolipin antibodies (ACL). A toxicology screen for toxic drugs and coumadin was negative. Coagulation factor assays revealed low levels for factor II and XII. Low level inhibitor to factor II was demonstrated. Patient had a negative VDRL test and positive anti-nuclear antibodies (ANA). The diagnosis of acquired hypoprothrombinaemia secondary to circulating inhibitor induced by LA was made, and then the patient was started on prednisone, which led to cessation of the bleeding and normalization of PT and PTT, as well as an increase of factor II and factor xii levels. A few months later, the patient developed arthralgia and alopecia, and antibodies against double-stranded dna were detected, and the diagnosis of systemic lupus erythematosis (SLE) was confirmed. The patient continued to have mild prolongation of PT and PTT while on a low dose of prednisone, but she had no bleeding symptoms. A computed tomography scan of the brain was carried out for unexplained central nervous system (CNS) symptoms, and it revealed mild hydrocephalus, which was thought to be part of the CNS manifestations of SLE. It was concluded that patients with SLE may present with haemostatic defects that are a result of either platelet-related causes (quantitative or qualitative) or coagulation factor deficiency secondary to circulating inhibitor, or both, in the absence of other features of SLE which may appear later.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
6/198. bone marrow transplantation in severe Glanzmann's thrombasthenia with antiplatelet alloimmunization.Glanzmann's thrombasthenia is an autosomal recessive disorder characterized by a lack of platelet aggregation due to the absence of platelet glycoprotein IIb and IIIa. Usually, the disease leads to mild hemorrhage but sometimes bleeding is severe enough to be life-threatening. We report the case of a 16-year-old girl, presenting with very severe type 1 Glanzmann's thrombasthenia, successfully treated with an HLA-identical sibling bone marrow transplant (BMT). We also update the clinical and laboratory data of her brother, who had received a BMT 16 years ago for the same disease. In the light of these two cases and two others published in the literature, we discuss the indications for BMT from HLA-identical sibling donors in Glanzmann's thrombasthenia. Alloimmunization against the missing platelet GPIIb/IIIa complex and severity of bleeding episodes may constitute sufficient criteria for allogeneic BMT after careful assessment of the risk-benefit of such a procedure, although this remains exceptional in this disease. bone marrow transplantation (2000) 25, 327-330.- - - - - - - - - - ranking = 7keywords = platelet (Clic here for more details about this article) |
7/198. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation.Neurofibromatosis is rare in the general population. Its clinical manifestations are systemic and variable. The clinical presentation of cutaneous lesions is even more variable. Some patients have giant tumors in the trunk or limbs (so-called "elephant neurofibromatosis"). The pathological findings are diffuse neurofibromatosis of the nerve trunk associated with overgrowth of subcutaneous tissue and skin. The associated vascular malformations make most surgeons hesitant to address them because bleeding to death is possible if the bleeding is not well controlled. According to the authors' experience in treating this complication of neurofibromatosis, they noted that there are three key points to reducing the amount of hemorrhage to a minimal level: (1) hypotensive anesthesia, (2) preliminary sutures around the lesion, (3) ligation of the limited numbers of feeding vessels in the vascular malformation of the neurofibroma. Ligating these pedicles can decrease bleeding during resection of the neurofibroma, as demonstrated in their patient.- - - - - - - - - - ranking = 0.0046305836647322keywords = giant (Clic here for more details about this article) |
8/198. Bleeding and selective serotonin reuptake inhibitors in childhood and adolescence.The selective serotonin reuptake inhibitors (SSRIs) are becoming widely used in children and adolescents, with possible unexpected side effects being observed over time. SSRIs have been associated with bleeding in adults who have unremarkable routine hematologic laboratory results except abnormal bleeding time or platelet counts in few cases. Given the increase of pediatric SSRI prescriptions, in this article we describe five children, ages 8 through 15, who developed bruising or epistaxis 1 week to 3 months after starting SSRI treatment. It is possible that the effects SSRI on platelet functioning are causing the bleeding observed in some patients and/or that a separate coagulopathy is present and contributing to bleeding. The subject matter deserves future investigation.- - - - - - - - - - ranking = 2keywords = platelet (Clic here for more details about this article) |
9/198. Catastrophic outcomes of noncardiac surgery soon after coronary stenting.OBJECTIVES: To assess the clinical course of patients who have undergone coronary stent placement less than six weeks before noncardiac surgery. BACKGROUND: Surgical and percutaneous transluminal coronary angioplasty revascularization performed before high-risk noncardiac surgery is expected to reduce perioperative cardiac morbidity and mortality. Perioperative and postoperative complications in patients who have undergone coronary stenting before a noncardiac surgery have not been studied. methods: Forty patients who underwent coronary stent placement less than six weeks before noncardiac surgery requiring a general anesthesia were included in the study (1-39 days, average: 13 days). The records were screened for the occurrence of adverse clinical events, including myocardial infarction, stent thrombosis, peri- and postoperative bleeding and death. RESULTS: In 40 consecutive patients meeting the study criteria, there were seven myocardial infarctions (MIs), 11 major bleeding episodes and eight deaths. All deaths and MIs, as well as 8/11 bleeding episodes, occurred in patients subjected to surgery fewer than 14 days from stenting. Four patients expired after undergoing surgery one day after stenting. Based on electrocardiogram, enzymatic and angiographic evidence, stent thrombosis accounted for most of the fatal events. The time between stenting and surgery appeared to be the main determinant of outcome. CONCLUSIONS: Postponing elective noncardiac surgery for two to four weeks after coronary stenting should permit completion of the mandatory antiplatelet regimen, thereby reducing the risk of stent thrombosis and bleeding complications.- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
10/198. Homozygous truncation of the fibrinogen A alpha chain within the coiled coil causes congenital afibrinogenemia.The molecular basis of a novel congenital afibrinogenemia has been determined. The proposita, the only affected member in a consanguineous Norwegian family, suffers from a moderate to severe bleeding disorder due to the total absence of any detectable fibrinogen. Dot blots of solubilized platelets revealed a small amount of gamma chain but no A alpha or B beta chains, whereas no chains were detected in plasma dot blots. dna sequencing of the A alpha chain gene revealed a homozygous C-->T transversion 557 nucleotides from the transcription initiation site. This nucleotide change predicts the nonsense mutation A alpha 149 Arg (CGA)-->stop (TGA). Early truncation of the A alpha chain appears to result in defective assembly or secretion of fibrinogen, probably due to the removal of the C-terminal disulfide ring residues that are critically required for the formation of a stable 3-chained half molecule. (blood. 2000;96:773-775)- - - - - - - - - - ranking = 1keywords = platelet (Clic here for more details about this article) |
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