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)

Two unrelated families are described with mild hemophilia A in whom six obligate carriers had unusually low VIII AHF levels. In each family, successive generations of males were affected with hemophilia A as determined by low VIII AHF in the presence of normal VIII AGN and VIII VWF levels. In the first family, two of five obligate carriers had low VIII AHF levels associated with clinical bleeding and one other had a history of bleeding. While receiving oral contraceptives, one of these two carriers was found to have a normal VIII AHF level. In the second family, four cousins below age 10 who were obligate carriers had significantly low VIII AHF levels, while a paternal aunt and paternal grandmother who were also obligate carriers had VIII AHF levels within the normal range. Hemorrhagic diathesis in multiple obligate carriers in these families is not readily explained by the Lyon hypothesis, and suggests that these families may be exmaples of an unusual allelic form of hemophilia A or that they may be transmitting several independent genes affecting VIII AHF levels. Our experience suggests that VIII AHF levels should be determined on all obligate or possible carriers prior to surgery to identify those individuals at risk for postoperative bleeding. Furthermore, it is suggested that hormonal therapy might be effective in the management of carriers with low levels of VIII AHF and clinical bleeding.
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PMID:Carriers with excessively low factor VIII procoagulant activity (VIII AHF): a study of two unrelated families with mild hemophilia A. 84 19

Hemorrhagic diathesis was observed in patients with renal insufficiency after carbenicillin at serum levels greater than 300 mug/ml. Normal coagulation factors (F. I, II, V, VII, VIII, X), normal PTT, normal platelet counts, negative ethanol gelation test (fibrin monomers) were found as well as a prolongation of thromboplastin time (Quick), thrombin time, reptilase time and thrombin coagulase time. Platelet function was disturbed. In addition, the plasmatic system was involved: inhibition of fibrinogen-fibrin conversion (Belitser assay) and enhanced antithrombin III activity; in vivo the latter was ascribed to a heparin-like activity. In vitro, abnormal III was seen: however an enhanced antithrombin III activity in vitro was not found with carbenicillin and various penicillin derivatives. This study demonstrates that carbenicillin, in addition to its known effect on platelet function, also disturbs the plasmatic coagulation system. This additional effect of carbenicillin is clinically important since protamin chloride effectively blocks bleeding without interfering with antibacterial activity. Both penicillin and penicillin derivatives have been shown to interfere with hemostasis and to cause clinically manifest hemorrhagic diathesis (Fleming and Fish 1947, Lurie et al. 1970a, b, McClure et al. 1970, Yudis et al. 1972, Demos 1971, Waisbren et al. 1971). Carbenicillin interferes with ADP-, collagen- or thrombin-induced platelet aggregation and with the release reaction both in vivo (McClure et al. 1970, Cazenae et al. 1973) and in vitro (McClure et al. 1970, Cazenave et al. 1973). In addition Lurie and colleagues (1970b) concluded that an inhibition of the conversion of fibrinogen to fibrin is involved although no experimental details were given. Later Brown and colleagues (1974) concluded that carbenicillin at usual dose levels "only affects the platelet component of hemostasis and has little effect on fibrin formation or other phases of coagulation in patients with normal renal function".
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PMID:Bleeding in uremic patients after carbenicillin. 103

In healthy volunteer donors, intranasal instillation of adiuretin-SD in doses of 0.0002-0.001 mg/kg a day provoked an increase of the activity VIII:WF in the plasma and acceleration of euglobulin lysis. Out of 15 patients suffering from Willebrand's disease, the activity of Willebrand's factor rose in 9 patients. Activated partial thromboplastic time became shorter in all the patients. In addition, acceleration of euglobulin lysis was noted. In view of that fact adiuretin was used later in combination with aminocaproic acid (0.2 g/kg a day). All the patients suffering from menorrhagias demonstrated the lowering of hemorrhagic diathesis. The data obtained allow recommending the use of adiuretin combined with aminocaproic acid in patients suffering from Willebrand's disease.
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PMID:[A trial of adiuretin-SD use in von Willebrand's disease]. 212 75

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

Assessment of animals with a suspected hemorrhagic diathesis of unknown cause(s) should be methodical. Most acquired coagulopathies result from thrombocytopenia. A platelet estimate (from a blood smear) and/or a platelet count on a fresh blood sample therefore are useful first steps in case evaluation. If thrombocytopenia is present, the most likely causes are immune-mediated destruction of platelets, DIC, or megakaryocytic hypoplasia. These diagnoses can be pursued by further test, including antiplatelet antibody assays (for example, the platelet factor 3 tests or an ELISA test), measurement of FDP, and bone marrow biopsy, respectively. If the platelet count is normal, a buccal mucosa bleeding time test is a useful second step. If this is prolonged, most likely causes are vWD or a thrombocytopathy (functional platelet defect). von Willebrand's disease can be diagnosed by measurement of vWf concentration or activity. A normal bleeding time does not exclude a diagnosis of vWD, but suggests that the functional activity of vWf is not compromised markedly. If the bleeding time is normal, APTT and PT should be measured. A prolonged APTT with normal PT, in the clinical setting, implies a deficiency of factor XI, IX, or VIII. A prolonged PT with normal APTT indicates factor VII deficiency. Prolongation of both APTT and PT usually is caused by a deficiency of several factors and is seen most often in cases with vitamin K deficiency or antagonism. Obviously, if a particular cause is suspected from the case history or for other reasons, appropriate tests should be evaluated at the beginning. If these do not confirm the provisional diagnosis, the just-described protocol might be a useful one to follow.
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PMID:Laboratory evaluation of hemorrhagic coagulopathies in small animal practice. 267 37

The Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein-Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal. No definite circulating inhibitor could be demonstrated. The transient Passovoy defect could possibly be ascribed to the infectious process or sulfisoxazole, which the patient had received.
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PMID:Transient Passovoy defect during a febrile illness. 299 85

A lupus or lupus-like inhibitor was detected in 57 patients: 24 systemic lupus erythematosus, 9 autoimmune diseases, 10 lymphoproliferative disease, 11 miscellaneous diseases and 3 asymptomatic patients. No hemorrhagic diathesis was observed in spite of major surgery. Thromboembolism occurred in 19 patients. Among them, 5 patients had recurrent abortions. An extensive study of coagulation profile compared different assays to investigate lupus-like inhibitor: the most sensitive assay was the partial thromboplastin time performed without activator. When performed with kaolin, it was the only assay detecting the lupus cofactor. Prothrombin time was prolonged in only 53% of the patients. Factors VIII, IX, XI and XII were in the normal range in 40% of the patients. When decreased, apparent deficiencies were usually not detectable on further dilutions of the test samples. In 7 patients factor XII antigen and activity were both decreased, suggesting an apparent factor XII deficiency. No relationship was observed between thromboembolic events, underlying disease or biological pattern.
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PMID:Biological and clinical heterogeneity of lupus and lupus-like anticoagulant in fifty-seven patients. 348 Sep 33

A 22-yr-old primigravada developed a hemorrhagic diathesis 6 days after delivering a normal female infant and was found to have an immunoglobulin inhibitor of factor VIII (15 Bethesda units). The patient was treated with prednisone and the bleeding stopped soon thereafter. Her inhibitor titer decreased over the next 8 mo, at which time no inhibitor was detectable. Nine months later she became pregnant again and proceeded to have an uneventful pregnancy and delivery of a normal female infant without evidence of a recurrence of the inhibitor. Studies of 3H-thymidine incorporation into the patient's lymphocytes in the presence of her own plasma or plasmas from her husband, normals, a von Willebrand patient, and a hemophilic patient yielded equivocal results. Analysis of VIII:CAg using 2 different antisera failed to discern immunologic differences between the VIII:CAg of the patient, her husband, or her first child. A review of the literature revealed that there were no recurrences with second pregnancies in any of the 8 patients with postpartum factor VIII inhibitors whose inhibitors had completely disappeared prior to delivery. While the pathogenesis of this disorder remains uncertain, the apparently favorable prognosis for such patients should be considered in counselling with regard to future pregnancies.
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PMID:Normal pregnancy in a patient with a prior postpartum factor VIII inhibitor: with observations on pathogenesis and prognosis. 678 45

Coagulation studies were performed during and after 60 continuous plasma exchanges in 15 patients with neurologic disorders. One or several plasmapheresis were performed on each patient using a new filtration material "Plasmaflo" from Asahi Medical, Tokyo. During each exchange involving massive heparin infusion, two total plasma volumes were removed and replaced isovolumetrically by a material devoid of clotting factors (albumin and Plasmion). The variation of the platelet count was moderate (mean reduction of 19%). An important and rapid fall of all coagulation factors was observed, and during a single exchange procedure the extent of the fall was comparable for all coagulation factors. The mean reduction during plasmapheresis was 70%. However, a difference in the rapidity of normalisation was observed between the various proteins (F.V. : 24 h, A.T.III : 72 h). A particular property was noted concerning factor VIII. In all cases, a normal or high level of VIII : C as well as of VIII R : Ag was reached 3 hours after exchange. In some cases an increase could be observed even during plasmapheresis. No hemorrhagic diathesis was noted despite massive heparin administration to the patients and a striking decrease of blood procoagulant factors. On the other hand, one patient developed femoral vein thrombosis following the 8th exchange.
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PMID:[A new plasma exchange technique : coagulation studies (author's transl)]. 732 5

A 74-year-old man developed a severe bleeding disorder on the basis of acquired Factor VIII (F VIII) inhibitor. Coagulation assays showed a prolonged activated partial thromboplastin time (APTT) with a normal prothrombin time (PT);F VIII level was 0.07 IU and F VIII inhibitor level 8.8 Bethesda units (BU). At least half the cases of acquired haemophilia A are associated with pregnancy, the postpartum period or an underlying malignancy or autoimmune disease. Haemorrhagic diathesis can be severe and life-threatening. Treatment of acute haemorrhages consists of human or porcine factor VIII concentrate, activated prothrombin complex concentrate (FEIBA) or recombinant factor VIIa, depending on the antibody titre. Immunosuppressive therapy is successful in at least 60% of the patients in making the inhibitor disappear. In patients with a spontaneous haemorrhagic diathesis, a thorough medical history should be taken, coagulation assays should be performed and a specialist should be consulted.
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PMID:[Acquired haemophilia A]. 938 Jan 88


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