Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.6 (thromboplastin)
 13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The evaluation of menorrhagia should no longer be solely the task of the gynaecologist. In women with ovulatory bleeding (regular cycles), the prevalence of von Willebrand disease (vWD) in about 15% of these, as well as disorders of platelet function and fibrinolysis causing menorrhagia, warrants an active role by the haematologist. Initial intake should include documentation of menorrhagia by the pictorial chart assessment of menstrual flow. Baseline characteristics of menstrual flow should also be documented, including the frequency of changing the sanitary pad on the heaviest day, use of more than one sanitary pad at a time, number of days lost from school/work and the impact of menses on various quality-of-life parameters. Menorrhagia since menarche, a past history of surgical- and/or dental-related bleeding and a past history of postpartum haemorrhage are items of the bleeding symptom audit that appear in part to predict vWD in women with menorrhagia. Epistaxis and easy bruising do not appear to be clearly discriminatory symptoms. Initial testing should include the complete blood cell count, prothrombin time, activated partial thromboplastin time, iron profile, serum creatinine, thyroid stimulating hormone level, factor VIII level, vWF antigen, ristocetin cofactor and platelet aggregation studies. Additional haemostatic studies may also include a factor XI level and euglobulin clot lysis time. This extensive medical evaluation should assure both the patient and the gynaecologist that the possibility of an underlying haemostatic disorder has been thoroughly investigated, to avoid the patient undergoing further costly procedures and surgical interventions if an underlying haemostatic disorder remains unrecognized.
 ...
PMID:Menorrhagia from a haematologist's point of view. Part I: initial evaluation. 1201 Apr 30

We report a quite rare case of acquired type 3-like von Willebrand syndrome (vWS) that preceded full-blown systemic lupus erythematosus (SLE). A 16-year-old woman with no previous disease history and no family history of hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis and gingival bleeding. She was diagnosed as having atypical type 3 von Willebrand disease because of prolonged bleeding time with normal platelet count and prolonged activated partial thromboplastin time (aPTT), and an almost complete absence of von Willebrand factor (vWF) antigen, ristocetin cofactor activity (vWF:RCo) and ristocetin-induced platelet agglutination (RIPA). Furthermore, electrophoretic analysis of plasma vWF revealed a trace amount of vWF and an absence of the multimeric form of vWF. Infusions of either vasopressin or factor VIII/vWF concentrates improved bleeding symptoms and corrected the aPTT and RIPA. However, she complained of low-grade fever, general fatigue and polyarthralgia 5 months later, and leukocytepenia and hypo-complementemia developed. Anti-double-stranded DNA antibodies and lupus erythematosus cells became positive. These findings were compatible with SLE. Mixing the patient's platelet-poor plasma (PPP) with normal platelet-rich plasma (PRP) (PPP/PRP = 2/1) resulted in a complete inhibition of RIPA, suggesting the presence of vWF inhibitor in her plasma. Treatment with prednisolone (40 mg/day) started and the bleeding tendency gradually improved. One month later, all of the laboratory data including aPTT, bleeding time, RIPA and vWF:RCo became normal. These findings indicate that she has an acquired type 3-like vWS associated with SLE.
 ...
PMID:Acquired type 3-like von Willebrand syndrome preceded full-blown systemic lupus erythematosus. 1203 3

We describe a case of acquired von Willebrand's disease (vWD) associated with monoclonal gammopathy with undetermined significance (MGUS) in a 54-year-old man who was admitted with hemarthrosis and extensive thigh muscle hematoma following arthroscopic surgery and postoperative prophylaxis with low molecular weight heparin. Coagulation tests were compatible with acquired vWD: prolonged activated partial thromboplastin time (aPTT) (56.1 s), decreased levels of factor VIII coagulant activity (23%), low concentrations of von Willebrand's factor (vWF) antigen (13%), and undetectable ristocetin cofactor activity (<10%). Infusion of a vWF-containing factor VIII concentrate failed to normalize the plasma levels of vWF-related parameters. Only additional intravenous administration of immunoglobulins led to a transient normalization of ristocetin cofactor activity, vWF antigen, and factor VIII coagulant activity. While the spontaneous bleeding tendency in this case was mild, surgery and administration of prophylactic doses of low molecular weight heparin led to life-threatening bleeding.
 ...
PMID:Massive postoperative intramuscular bleeding in acquired von Willebrand's disease. 1218 11

Severe type-3 von Willebrand's disease (vWD) was diagnosed in a young male rhesus monkey that had excessive bleeding from minor wounds. Plasma samples from the monkey had no detectable quantitative or functional von Willebrand factor (vWF), low Factor-VIII coagulant activity, and moderate prolongation of activated partial thromboplastin time. Testing of the affected monkey's extended family revealed a likely hereditary basis for the vWD, in that the sire and a paternal half-sister had markedly reduced plasma vWF concentration. Fresh whole blood was transfused to control frequent bleeding episodes throughout the monkey's life. Although vWD is the most common inherited bleeding disorder in humans and dogs, this is the first report of vWD in a nonhuman primate.
 ...
PMID:Type-3 von willebrand's disease in a rhesus monkey (Macaca mulatta). 1221 Dec 83

Systematic complementary testing for asymptomatic patients before surgery yields an unexpectedly high percentage of anomalous results. Such results rarely affect perioperative management of the patient but may lead to unnecessary delays, which are potentially of great importance in emergency surgery. A 55-year-old woman with a clinical diagnosis of acute appendicitis was seen to have a prolonged activated partial thromboplastin time (APTT) of 1.94 before surgery. The patient's history did not suggest a coagulation disorder was likely, and when mixing normal and problem plasma failed to correct the APTT, we suspected an unspecified circulating anticoagulant was present. Surgery was delayed no further and no measures were taken. No excessive bleeding occurred during surgery or postoperative recovery. The main possible diagnoses for a women with unforeseen prolonged APTT are the presence of an unspecified circulating anticoagulant, factor XI or factor XII deficiency, or factor VIII deficiency associated with von Willebrand disease. Focusing on detecting a coagulation disorder while taking a patient's history and performing a simple laboratory test (mixing normal and problem plasma) can be useful for orienting management in emergency surgery.
 ...
PMID:[Unsuspected prolonged activated partial thromboplastin time in emergency surgery. Diagnostic and therapeutic guide]. 1245 23

In order to define the prevalence of haemostatic defects in women presenting with menorrhagia in our region, the coagulation data on women bleeders investigated in the Department of Haematology, AIIMS, were analysed. A total of 337 of the 2200 menorrhagic women investigated were characterized to have an inherited bleeding disorder; 221 of these 337 women presented with menorrhagia alone while 116 also had other associated bleeding manifestations as prolonged bleeding from injury site, ecchymotic patches in the skin, epistaxis, haematomas, haemarthroses and major bleeds like intracerebral bleeding. The tests performed included bleeding time (BT), platelet count, prothrombin time (PT), prothrombin consumption index (PCI), activated partial thromboplastin time (APTT), PF3 release with adenosine diphosphate (ADP) at 0 and 20 min, total PF3 assay and platelet Aggregation studies with collagen, ADP, adrenaline, arachidonic acid and ristocetin. Coagulation factor assays, von Willebrand antigen estimation, ristocetin cofactor assay and electron microscopy were performed wherever necessary. Inherited platelet dysfunction was seen in 283 (83.9%) of the patients. Amongst these, isolated PF3 availability defect was seen in 163 (48.4%) cases. Glanzman's thrombasthenia was seen in 30 (8.9%) patients, Storage pool disease in eight (2.4%) patients, arachidonic acid pathway defect in five (1.5%) patients and Bernard-Soulier Syndrome in six (1.8%) patients. In 71 (21.1%) patients, the platelet function defect could not be classified into any specific subtypes. Inherited defects of coagulation were observed in 54 (16%) of the cases. Amongst these, von Willebrand disease (vWD) was the most frequent being seen in 40 (11.9%) of the cases. Factor XIII deficiency was seen in one (0.3%), factor X deficiency in four (1.2%), factor VII deficiency in one (0.3%) and factor XII deficiency in one (0.3%) of the patients. It is concluded that although hereditary platelet function defects constitute a large majority of women bleeders in India but among the coagulation defects, vWD is the commonest as reported from the caucasian population. It is thus suggested that in women presenting with menorrhagia, screening tests for haemostasis especially for vWD and inherited platelet function defects must be performed.
 ...
PMID:Inherited bleeding disorders in Indian women with menorrhagia. 1451 14

The evaluation of the woman with abnormal bleeding, particularly menorrhagia, carries a relatively high yield of discovering an underlying disorder of hemostasis. The most common underlying hemostatic disorder would be von Willebrand disease, with an estimated prevalence of 7% to 20%. In addition, preliminary results suggest that another 20% to 30% of patients may have impaired platelet aggregation as another cause of menorrhagia. Disorders of fibrinolysis may be an additional underlying hemostatic disorder. Initial intake should include documentation of menorrhagia by the pictorial chart assessment of menstrual flow. Baseline characteristics of menstrual flow should also be documented including the frequency of changing the sanitary napkin on the heaviest day, use of more than one sanitary napkin at a time, and number of days lost from school or work. Menorrhagia since menarche, a history of surgicalor dental-related bleeding, and a history of postpartum hemorrhage are items of the bleeding symptom audit that appear to predict in part von Willebrand disease in women with menorrhagia. Epistaxis and easy bruising do not appear to be clearly discriminatory symptoms. Initial testing should include the complete blood cell count, protime, activated partial thromboplastin time, iron profile, serum creatinine, thyroid-stimulating hormone level, factor VIII level, von Willebrand factor antigen, ristocetin cofactor, and platelet aggregation studies. Additional hemostatic studies may also include a factor XI level and euglobulin clot lysis time. Intuitively, failure to diagnose an underlying hemostatic disorder may lead to continued menorrhagia and diminished quality of life, as well as unnecessary surgical interventions, which, in turn, may be fraught with increased bleeding.
 ...
PMID:Evaluation of abnormal bleeding in women. 1290 Nov 20

von Willebrand disease (vWD) is the most common congenital bleeding disorder in the USA, affecting 1-3% of the population. Previously characterizing the bleeding symptoms in females with type 1 vWD, we evaluated 42 males with type 1 vWD, mean age 16 years (1-64), of whom 24 (57%) presented with bleeding symptoms. The most common initial symptom was postoperative bleeding (26%). The most common bleeding symptoms ever were epistaxis (53%), bruising (50%), postoperative bleeding (47%), haematomas (29%) and oral bleeding (29%). Of postoperative bleeding, ear/nose/throat (44%), dental (17%) and circumcision bleeding (22%) occurred at a median 10 years of age, despite a previous bleeding or family history in 89%. Complications included anaemia in five (12%), neurological sequelae after subdural haematoma and tonsillectomy in two (5%), transfusion-associated hepatitis C in two (5%) and degenerative joint disease after traumatic haemarthroses in one (2%). The bleeding time (BT) was prolonged in 83%, and the ristocetin cofactor (vW:RCoF) and factor VIII (FVIII:C) decreased in 64% and 43%, respectively. Haemarthroses and haematoma formation were associated with a longer activated partial thromboplastin time (APTT) (P < 0.05), and anaemia with a lower FVIII:C (P < 0.05). In 81%, a haemostatic response occurred with 1-8 deamino-d-arginine vasopressin (DDAVP), although, in 13%, surgical intervention was also required to achieve haemostasis. Postoperative bleeding could have been avoided in 89%, if a preoperative past bleeding history or family history had been obtained, and, in at least 94%, if a preoperative BT and APTT had also been performed. The failure to avoid postoperative bleeding and related complications in patients with vWD by taking a personal and family bleeding history constitutes a major public health problem.
 ...
PMID:Bleeding manifestations in males with von Willebrand disease. 1496 5

Assessment of haemostasis in people with neurofibromatosis type 1 (NF-1) is essentially lacking, despite case reports of an association with von Willebrand disorder (VWD) and reported excessive bleeding post-surgery. We assessed routine blood haematology, routine coagulation parameters [prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT) and fibrinogen], coagulation factors II, V, VII, VIII, IX, X, XI and XII, von Willebrand (VWF) factor antigen and activity, and platelet function [using the platelet function analyser (PFA-100)] in a group of individuals with NF-1 (n = 30). Their perceived haemorrhagic bleeding risk was also graded by means of a structured clinical assessment and physical examination. Routine blood assessments including platelet counts were generally normal, as were the routine coagulation tests PT, TT and fibrinogen, and most coagulation factors. Elevated APTTs were detected in 11 individuals, reduced factor XII levels in three, reduced VWF levels in four, and elevated PFA closure times (CTs) in 13. Laboratory results correlated with each other in some but not all cases. For example, elevated APTTs were identified in two of three individuals with a reduced factor XII level and prolonged CTs were identified in three individuals who also showed reduced aggregation responses in classical platelet function studies. Moreover, all individuals with VWF results below the normal reference range showed elevated CTs with both PFA test cartridges, and those with VWF results identified as borderline normal (i.e. 50-65%) also showed elevated CTs with both PFA test cartridges in three of five cases. The relationship between VWF and CTs was also identified by linear regression analysis (P-values of <0.05, for all comparisons). However, as clinically perceived bleeding risk did not appear to be correlated with laboratory test results in most cases, blanket screening of NF-1 individuals for evaluation of laboratory haemostasis may not be warranted.
 ...
PMID:Evaluation of primary haemostasis in people with neurofibromatosis type 1. 1548 64

A 41-year-old woman with known systemic lupus erythematosus (SLE) was diagnosed with thrombotic thrombocytopaenic purpura (TTP). At the time of admission she was suffering from petechia, purpura and had neurological symptoms. At first a relapse of the SLE was suspected. Additional laboratory findings demonstrated haemolytic anaemia, thrombocytopaenia and high levels of fragmentocytes. After multiple plasmapheresis treatments and immunosuppressive therapy she recovered. TTP can be differentiated from other thrombotic microangiopathic syndromes by its normal levels of prothrombin time, partially activated thromboplastin time (APTT), fibrinogen and direct Coombs-test. Further investigation is needed to confirm the diagnosis by determination of the activity of Von Willebrand factor cleaving protease ADAMTS-13. In this patient, the ADAMTS-13 activity returned after plasmapheresis. This case demonstrates the importance of fast and appropriate laboratory testing in order to diagnose TTP quickly.
 ...
PMID:[A woman suffering from systemic lupus erythematosus and acquired thrombotic thrombocytopenic purpura]. 1552 34


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>