Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A von Willebrand syndrome was present in four patients with sickle cell trait and hematuria. The first two patients had severe anemia and active bleeding and received cryoprecipitate, with prompt cessation of hemorrhage. All of the patients had repetitive laboratory and clinical features; that is, reduced, but detectable, factor VIII-related antigen, heterogeneity of, and incongruities within, the coagulation studies performed by consistently defective platelet aggregation to ristocetin correctable only with normal plasma. Bleeding outside the genitourinary tract never was observed. Because of the focal nature of the hemorrhage, the hematuria may not have been perceived as part of a general hemorrhagic disorder and the diagnosis not pursued. These observations suggest that when sickle cell trait and hematuria occur together, a von Willebrand syndrome should be a major diagnostic consideration that ultimately may point toward a rational, effective, easily administered, and clinicially acceptable form of treatment with cryoprecipitate.
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PMID:Sickle cell trait and hematuria associated with von Willebrand syndromes. 30 May 82

Von Willebrand's disease is common hemorrhagic disorder that deserves particular attention in the patient undergoing open-heart surgery, because of perioperative unexpected bleeding. The present case is atrial septal defect associated with Willebrand's disease, affecting a 19-year-old female who has the past history of bleeding tendency with frequent subcutaneous bleeding. It is mandatory to maintain adequate levels of both factor VIII coagulation and ristocetin cofactor activities during the perioperative period. An infusion study of heat-treated evaluate the response and half-life of both factor VIII coagulation and ristocetin cofactor activities; and to calculate the effective dose and duration of the drug action as well as to formulate a protocol for patient's management in the perioperative periods in order for the surgical intervention to be performed uneventfully. Our case demonstrated that it is possible to perform an open heart surgery in patients with von Willebrand's disease without major bleeding as far as the levels of both factor VIII coagulation and ristocetin cofactor activities are maintained above 50% in the postoperative critical period.
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PMID:[Atrial septal defect associated with von Willebrand's disease--a case report]. 140 71

Acquired von Willebrand syndrome is reported in a 31-year-old woman with autoimmune hemolytic anemia (AIHA). The patient, who had no family history of bleeding, presented with a hemorrhagic diathesis of recent origin. Routine coagulation studies showed a normal platelet count, prolonged bleeding time and abnormal glass bead retention. Plasma levels of factor VIII-von Willebrand factor (F VIII-vWF) were low and ristocetin-induced platelet aggregation (RIPA) was decreased. In vitro, the patient's plasma exhibited inhibitory activity against vWF: ristocetin cofactor activity (vWF: Rco) but had no effect on RIPA. The multimeric pattern of the patient's vWF resembled that of patients with inherited type II von Willebrand disease, in that the largest multimers were missing. Clinical improvement resulted after treatment of AIHA with prednisolone (PSL), and F VIII-vWF returned to normal levels with normalization of vWF multimers. However, following tapering the dose of PSL, plasma levels of F VIII-vWF again decreased.
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PMID:[Acquired von Willebrand syndrome with autoimmune hemolytic anemia]. 224 22

A 61-years old woman who had been healthy without history of abnormal bleeding, developed widely spread ecchymosis and intramuscular bleeding in March, 1987. She was hospitalized for this hemorrhagic diathesis in May, 1987 and the following laboratory data were revealed: activated partial thromboplastin time (APTT), 76.7 seconds; factor VIII procoagulant activity, 2%; factor VIII inhibitor, 27 Bethesda units/ml. The inhibitor was an immunoglobulin of IgG type. Her clinical data of the blood were normal, and tests for antibodies, such as RA test, LE test and thyroid test were negative. Physical examination revealed ecchymosis over her right arm and swelling and pain in the right arm. She was first treated with a large dose of factor VIII concentrates, but the effect was insufficient. Then prednisolone was given, which resulted in decreasing of the inhibitor and improvement of the coagulation profiles. This treatment appeared to offer effective control on severe hemorrhage in patients with factor VIII inhibitors.
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PMID:[Remission of a non-haemophilic patient with acquired factor VIII inhibitor treated with infusion of factor VIII and corticosteroid]. 250 78

Acquired hemophilia (idiopathic or secondary) is an uncommon clinical condition. A 70-year-old male had a severe hemorrhagic disorder, and an IgG inhibitor of the factor VIII:C was detected in plasma. During the acute phase he was treated with packed red blood cells, frozen fresh plasma and polyvalent immunoglobulins. The hemorrhagic features subsided but the circulating anticoagulant persisted. The administration of an activated prothrombin complex permitted to make the diagnosis of the underlying disease, a highly malignant T type lymphoma. During the treatment with corticosteroids and polychemotherapy the inhibitor activity disappeared.
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PMID:[Hemorrhagic syndrome associated with a factor VIII:C inhibitor in a patient with lymphoma]. 250 4

Bleeding disorders are frequent indications for reference to Hematologic Clinics. Won Willebrand's disease (vWD) is one of the commonest of inheritable hemorrhagic diathesis. In order to evaluate the frequency and clinical characteristics of vWD in the IX Region of Chile, we performed a clinical and laboratory investigation in 132 patients, children and adults, over a period of 3 years: 1983-1985. Within this group there was 26 close relatives. The laboratory tests used for the diagnosis were: bleeding time, factor VIII, von Willebrand factor and ristocetin cofactor. Diagnosis of vWD was made in 83 cases, most of them presenting the mild form of the disease. Two children had the severe form. The estimated prevalence of vWD in symptomatic patients in the IX Region of Chile is at least 113 per million, which makes it 3 or 4 times more frequent than hemophilia. vWD is observed at a much lower rate in mapuche than in caucasian population. The symptoms and their frequency were: post partum bleeding 75%, dental extraction bleeding 65%, surgical bleeding 65%, epistaxis 63%, menorrhagia 54%, easy bruising 45%, deep hematomas 8% and petechiae 5%. Five cases were asymptomatic. The bleeding time was the test most frequently abnormal: 72/83 cases (87%).
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PMID:[Von Willebrand's disease in the IX Region of Chile]. 251 97

Rarely, a patient develops an antibody against factor VIII coagulant activity. The resultant hemorrhagic diathesis is clinically distinct from inherited hemophilia, being characterized by few hemarthroses but frequent skin and other soft-tissue hematomas. Hematuria may be troublesome. These patients represent therapeutic challenges. This study is one institution's results with 16 such patients followed up over an average of 31 months (range, four to 120 months; median, 19 months). It describes the largest group from a single institution receiving essentially no immunosuppressive agents, yet has one of the better overall results. Two patients experienced fatal hemorrhage and five patients underwent spontaneous remission. Long-term survival is not incompatible with persistence of the inhibitor. We conclude that this hemorrhagic diathesis is clinically distinct, less fatal than usually perceived, and may undergo spontaneous remission. Clearly, there is no mandate for any particular therapeutic regimen, such as immunosuppression, in the attempt to rid the patient of the antibody.
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PMID:Acquired hemophilia. A natural history study of 16 patients with factor VIII inhibitors receiving little or no therapy. 310 41

A 65-year-old man was admitted to the hospital because of extensive spontaneous ecchymoses of the trunk and huge hematomas of the arms and legs. He had no personal or family history of a hemorrhagic diathesis. Coagulation studies revealed a prolonged APTT, no detectable factor VIII:C activity, and a high titer of anti-factor VIII:C antibodies. A diagnosis of acquired hemophilia was made. No underlying disorder could be found. The inhibitor was an IgG antibody. Long-term management of bleeding including immunodepletion by plasma exchange and immunosuppression by corticosteroids and cytotoxic drugs alone and in combination had no effect on the bleeding tendency and coagulation data. The administration of Cs (10 mg/kg/d) in combination with prednisone induced a remission. After a stormy course and a 5-month stay in the hospital the patient could be discharged. A relapse occurred after the Cs and prednisone dosages were reduced. Increasing the Cs dosage induced a remission again.
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PMID:A patient with a spontaneous factor VIII:C autoantibody: successful treatment with cyclosporine. 313 76

Severe hemorrhagic diathesis caused by hemophilia A (factor VIII:C deficiency) was diagnosed in 2 related Quarter Horse colts. Clinical signs consisted of dyspnea and dysphagia attributable to cranial cervical hematoma in one colt and to intra-abdominal hemorrhage resulting in death of the second colt. Factor VIII:C deficiency, a defect of the intrinsic coagulation pathway, is suggested by results of coagulation studies--prolonged activated partial thromboplastin time, normal prothrombin time, and normal primary bleeding time. The diagnosis was confirmed by results of factor VIII:C assays. Hemophilia A is inherited as an X chromosome-linked trait.
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PMID:Hemophilia A in two related quarter horse colts. 313 24

The case of a 72 year-old man with a sinus histiocytosis is reported. The clinical course was characterised by an hemorrhagic disorder linked to a factor VIII inhibitor. The characteristics of the antibody titer, affinity and saturability have been studied. In an attempt to stop the bleeding treatment included human and porcine factor VIII concentrates, plasmapheresis and immunosuppressive drugs. Repeated plasma exchanges did not modify the inhibitor titer for more than 24 h.
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PMID:[Antifactor VIII antibody in Rosai-Dorfman disease]. 640 24


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