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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The platelet function was studied in 23 patients with systemic lupus erythematosus, all of whom met the diagnostic creteria established by the American Rheumatism Association. They were not under any treatment, especially with any drug that might interfere with platelet function. The same study was performed on a control group composed of volunteers donors at a blood bank. The platelet count was definitely lower in the patients with lupus than in the control subjects (p less than 0.0005), although a clear thrombopenia was observed in only two indivduals (8.7 percent). Anti-platelet antibodies were found in only six cases (26 percent). There was a linear correlation between thrombopenia and the presence of hemorrhagic diathesis and low levels of C4 and CH50 components. Plateler adhesiveness was clearly lower in the lupus group than in the control group (p less than 0.0005). The presence of kidney disease determined a greater impairment of the platelet adhesiveness (p less than 0.0025). A notable defect on platelet aggregation was induced by ADP, adrenaline and collagen. This was more apparent in the group of patients exhibiting a higher degree of clinical activity and in those who showed a serum complement decrease. The mechanism responsible for this thrombopathy appears to be an interference in the platelet function due to the presence of circulating immunocomplexes. They adhere to the platelet membrane blocking its function and inhibiting the release of the necessary thrombocytic components for the second phase of the aggregation. This platelet alteration is not usually manifested clinically; for this reason no relationship was found between this platelet defect and the presence of hemorrhagic symptoms in our patients. The condition is reversible and may disappear after therapy with steroids and/or immunosuppresive agents.
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PMID:[Platelet function in systemic lupus erythematosus (author's transl)]. 45 1

Lupus anticoagulant antibodies and anticardiolipin antibodies are acquired circulating immunoglobulins that interact with phospholipids. These factors may exert anticoagulant properties in vitro and so interfere with coagulation tests that use phospholipids. These antibodies are not, however, associated with a hemorrhagic diathesis. Indeed, despite their name and their in vitro anticoagulant properties, they have been associated right from the earliest reports with systemic and cerebral thromboembolic episodes. We report the clinical and instrumental findings in a patient with ischemic stroke and anticoagulant antibodies in the serum.
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PMID:The primary antiphospholipid syndrome: case report. 142 95

A 9-year old girl admitted in our clinic for severe hemorrhagic syndrome was found to display a high level of lupus anticoagulant (LA) leading to an important prolongation of phospholipid-dependent coagulation. Positive antinuclear and anti DNA antibodies, as well as very low levels of complement C3 and C4 proteins confirmed the diagnosis of systemic lupus erythematosus. Therapy with cortisone and cyclophosphamide led to normalization of the clotting tests but could not arrest the development of renal and hepatic lesions. The patient is one of the few cases with presence of lupus anticoagulant associated with severe hemorrhagic diathesis, in opposition to the more frequently reported thrombotic tendency connected with antiphospholipid antibodies.
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PMID:Lupus anticoagulant and transient severe bleeding tendency in a girl aged nine years. 313 47

Critical conditions had been established in 21 (23.1%) of 91 patients with systemic connective tissue diseases for a 12 year period: renal failure (most often), sepsis, pericarditis with cardiac tamponade, hemorrhagic diathesis, terminal arteritis with gangrene, gastrointestinal perforations with peritonitis, etc. The corticosteroids applied in high doses and predominantly parenterally and the immunosuppressors are the main drugs used in the treatment of these conditions. Plasmapheresis when possible is a useful supplement. The prognosis of the acute critical conditions depends mainly on the affected organ (more favorable in pericarditis with tamponade and unfavorable in renal failure and gastrointestinal perforations with peritonitis (and on the basic disease) more optimistic in systemic lupus erythematodes and very pessimistic in nodal periarteritis and other allergic vasculitis).
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PMID:[The problems of treating acute critical states in diffuse connective tissue diseases]. 321 40

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

The lupus anticoagulant is a well-described in vitro phenomenon that may be associated with arterial and venous thrombotic episodes. The lupus anticoagulant is never accompanied by a hemorrhagic diathesis unless it is associated with a second coagulation abnormality such as thrombocytopenia or hypoprothrombinemia. The lupus anticoagulant-hypoprothrombinemia syndrome is now a well-defined entity that may cause a severe, life-threatening hemorrhagic diathesis. The hypoprothrombinemia in this syndrome is the result of rapid clearance of prothrombin-antiprothrombin antibody complexes by the reticulo-endothelial system. The cause of antiprothrombin antibody formation is unknown. The authors describe a recent experience with a patient with this syndrome who initially had recurrent, life-threatening gastrointestinal bleeding. They were able to demonstrate hypoprothrombinemia and the presence of prothrombin-antiprothrombin antibody immune complexes. The patient was treated with prednisone, with correction of the bleeding disorder; however, the patient had resultant death from thrombosis. A literature review of the past 30 years as it relates to the discovery and treatment of this phenomenon is included.
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PMID:Case report: the lupus anticoagulant-hypoprothrombinemia syndrome. 817 28

350 patients with idiopathic thrombocytopenic purpura (ITP) aged 2/12-15 years (mean 6.3 +/- 2.7) were followed up during the period January 1st, 1975 to March 31, 1992. They constituted 40% of cases with hemorrhagic diathesis attending the Hematology/Oncology Clinic, Children's Hospital, Ain Shams University (relative frequency of 37.4/100.000 of the general Out-Patient Clinic in the same hospital). These patients presented with acute (71.4%), chronic (22.9%) and recurrent (5.7%) forms. The age of presentation was younger in acute ITP. In the recurrent form there was significant female predominance. Most cases of acute ITP (66%) presented in winter and spring, with a positive history of preceding viral illness in 50% in contrast to 10% in chronic form. Four chronic ITP cases developed lupus erythematosus; all were females > 9 years. As regards therapy, acute ITP cases with initial platelet count (PC) < 10 x 10(9)/l were randomized to receive either high-dose methyl prednisolone (HDMP) 10 mg/kg/day for 5 days i.v. (n = 10) or intravenous immunoglobulin (IVIG) 0.4 g/kg/day for 5 days (n = 10) or conventional-dose prednisone (CDP) 2 mg/kg/day 4 weeks p.o. (n = 10). A dramatic response was noticed in the first two groups. In chronic ITP, (n = 80) CDP induced complete response (CR) in 30% and partial response (PR) in 20%; 50% were nonresponders. Twenty-four refractory ITP with persistent PC < or = 20 x 10(9)/l received second-line therapy: vincristine 1.5 mg/m2/week i.v. 4 doses (n = 4) with no clinical or hematological improvement. IVIG 0.4 g/kg/day for 5 days (n = 8) with sustained CR only in 2 patients (25%) and PR in 2 patients (25%). Splenectomy was performed (n = 12) with CR in 50%; out of them, 2 patients had shown no improvement on prior IVIG therapy. In conclusion, ITP is a benign condition with no fatality reported, but it could run a chronic refractory course.
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PMID:Idiopathic thrombocytopenic purpura in Egyptian children. 829 70

Ischemic venous thrombosis that led to necrosis of four toes developed in an 81-year-old man. Despite the extensive thrombosis, results of blood clotting tests showed an extremely low prothrombin time (20%). Plasma mixing studies demonstrated an inhibitor that may have features in common with the lupus anticoagulant. Lupus anticoagulant also simulates hemorrhagic diathesis in vitro, whereas in vivo it is associated with thrombosis and thromboembolism. In contrast to the lupus anticoagulant, the inhibitor found in this patient was active within the extrinsic clotting system. Systemic steroids led to rapid clinical resolution paralleled by normalization of the prothrombin time and disappearance of the inhibitor.
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PMID:Ischemic venous thrombosis caused by a distinct disturbance of the extrinsic clotting system. 849 74

The lupus anticoagulant may be accompanied by an acquired factor II deficiency and bleeding. We report on a patient with a lupus anticoagulant and factor II (Fll) deficiency responsive to Danazol. Acquired hypoprothrombinemia (FII) with the lupus anticoagulant (LA) may be accompanied by a hemorrhagic diathesis. A 64-year-old male with discoid lupus erythematosis bled after an intestinal polypectomy. His FII level was 18%, and his FII antigen level was 20%. Danazol (D) (600 mg per day) administration was associated with a rise in FII activity and antigen to 50% within 10 days. The patient underwent abdominal surgery. We studied the effect(s) of D on the FII level and on other coagulation factors in this patient. The patient's plasma FII antigen had a single precipitin arc compared to the two peaks of normal plasma on counterimmunoelectrophoresis with Ca++. The samples pre- and during D therapy had the same positively charged arc as normal samples, although they were quantitatively different. Neuraminidase treatment demonstrated a decrease in the positively charged migration of normal and the patient's FII antigen. Affinity chromatography of normal and patient plasma on a Sepharose protein A column revealed FII antigen present in the patient's bound fraction. The relative percentages of bound FII before and during D treatment were similar. During D therapy, levels of FIX and X rose 50-100%, and protein C rose 20-25%, while free protein S did not change. D is an effective therapy for acquired FII deficiency associated with LA. D does not affect the binding of Ig to FII, but D raises FII levels by increasing synthesis of the FII protein.
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PMID:Acquired hypoprothrombinemia: effects of danazol treatment. 894 70

By virtue of a severely prolonged aPTT with a normal thromboplastin time (prothrombin time) and a normal thrombin time, severe FXII deficiency has been diagnosed in a woman without a bleeding diathesis or a history of thromboembolic complications. A deficiency of a factor of the contact activation system (FXII, prekallikrein, high molecular weight kininogen) is usually diagnosed during routine coagulation tests demonstrating a prolonged aPTT. The severe and partial deficiency of FXII, of prekallikrein or high molecular weight kininogen is not associated with a bleeding tendency. In contrast, severely factor XI deficient subjects may suffer from a mild hemorrhagic diathesis, whereas FVIII deficiency (hemophilia A, autoimmune "hemophilia", von Willebrand disease) and FIX deficiency (hemophilia B) are associated with a bleeding tendency of varying severity, depending on the clotting activity of FVIII or FIX, respectively. An isolated prolongation of the aPTT due to a lupus anticoagulant, however, is frequently associated with arterial and/or venous thrombosis. Therefore, in case of a prolongation of the aPTT, its cause has to be determined.
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PMID:[A patient with isolated prolongation of aPTT without hemorrhagic diathesis anamnesis: severe, hereditary factor XII deficiency]. 1051 21


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