Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Platelet functions and blood coagulation have been regularly investigated in 31 patients undergoing maintenance haemodialysis for 5 months to 6 years. Fifteen of them suffered from at least two arteriovenous fistula thrombosis during the year prior the first examination. Eleven patients, including eight with recurrent thrombosis, received 300-400 mg per day dipyridamole during 1 month to 2 years. Some abnormalities are commonly observed in the whole studied population: lowering of platelet adhesiveness, defective aggregation in the presence of both collagen and ADP 5. 10-5 M; increased level of factor V and mainly factor VIII. Mean platelet factor 3 activity was in the normal range with variations from one case to another. The only unusual feature observed in patients with recurrent thrombosis was an increase of platelet aggregation induced by ADP 0.5. 10-6 M. Neither spontaneous aggregation nor significant abnormality of plasminogen level and plasma antithrombin activity were observed. Under dipyridamole therapy, correction of platelet hyperaggregability was observed in all patients and improvement of platelet adhesiveness in half the studied cases (despite the unchanged anaemia). The treatment significantly decreased the frequency of arteriovnous fistule thrombosis in the six patients observed during two consecutive years, the first one without and the second under treatment: the total number of thrombosis was 18 during the first and 3 in the second period.
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PMID:[Disorders of haemostasis in patients undergoing maintenance haemodialysis, with special reference to recurrent arteriovenous fistula thrombosis. Effects of dipyridamole (author's transl)]. 82 31

Phenylhydrazine (PHZ) is a hemolytic agent which has been used in the treatment of polycythemia vera. Recent studies performed in our laboratory have indicated that the PHZ-induced anemia is immuno-hemolytic in etiology, and a prolonged bleeding time was present in some of the rats chronically treated with PHZ. The nature of this bleeding tendency was explored in the present experiment. PHZ was administered to rats once a week for a six week period. During this time, the animals were monitored for prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen concentration, and individual coagulation factor levels as well as routine plasma chemistries and blood cell counts. In addition, radioimmunoassays (RIA) for prostacyclin, a platelet aggregation inhibitor, and prostaglandin (PG) E2 were performed. PHZ-treated animals displayed a significant elevation in both PT and APTT when compared with saline injected controls, although plasma fibrinogen levels were not appreciably altered. Further tests revealed a PHZ-induced decrease in prothrombin and factor V levels. In addition, a significant increase in plasma serum glutamate oxaloacetate transaminase (SGOT), lactate dehydrogenase (LDH), and alkaline phosphatase levels was observed as well as a diminution in cholesterol and triglycerides following PHZ administration. PHZ treatment also induced an elevation in prostacyclin levels and transient thrombocytopenia. These findings indicate that several factors may contribute to the prolonged bleeding time in PHZ-treated rats including a drug induced thrombocytopenia possibly associated with enhanced synthesis of autologous immunoglobulin G (IgG) against the senescent red cell antigen, and diminished synthesis of vitamin K-dependent coagulation factors which may be mediated by reduced vitamin K uptake by the hypo-cholesterolemic subjects.
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PMID:Hemostatic alterations associated with phenylhydrazine-induced anemia in the rat. 262 15

A 84-year-old man was admitted with palpitation, edema of legs and anemia during a long course of diabetes mellitus, prostatic hypertrophy and prostatic cancer. He revealed purpura on the hands and massive microhematuria. He had received antibiotic therapy for a urinary tract infection for a period of time, but he had no history of hemorrhagic tendency or blood transfusion. Coagulation studies showed the prolongation of whole blood clotting time and PT (prothrombin time). Activity of factor V was 14% of that normal control plasma. The titer of factor V inhibitor was 4.9 Bethesda units/ml. The inhibitor of the patient was supposed to belong to IgA and IgG judging from inhibitor neutralization test. PT was improved after discontinuance of administration of antibiotics and administration of azathioprine. Moreover, even after administration of prednisolone with antibiotics, PT and activity of factor V recovered to normal range. He died from respiratory failure. Autopsy revealed double cancer of prostate and descending colon. The appearance of factor V inhibitor was likely caused by antibiotics, double cancer, and age-related immune disorders.
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PMID:[Factor V inhibitor with double cancer]. 276 72

A study was initiated to determine the frequency and significance of disseminated intravascular coagulation (DIC) in the pediatric age group. With the aid of a scoring system, DIC was diagnosed in 48 patients in a period of slightly over one year in a pediatric referral centre with 7000 annual admissions. Sixty percent of all DIC occurred in infants under one month of life. Sixty-six percent of all DIC was associated with sepsis, usually from gram-negative infections. Seventy-nine percent of affected neonates were septic. Laboratory findings of diagnostic importance were anemia with red cell fragmentation, thrombocytopenia, elevated titres of fibrin split products, abnormal thrombin time, and low factor V activity. Mortality was 64% in all ages regardless of cause. Results of management of DIC by treatment of the underlying disease with or without anticoagulation were disappointing.
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PMID:Experience with disseminated intravascular coagulation in a children's hospital. 508 21

The authors observed the patient T.P.D., aged 36, admitted to the clinic of RIHBT with melena and hematemesis. Since early childhood the patient has been treated on the occasions of hemorrhagic incidences for hemophilia A. The hemostatic investigations however, revealed normal level of factor VIII and the level of factor V--1%, providing grounds to revise the initial diagnosis and to admit that the rarely found parahemophilia is concerned. The treatment with freshly-frozen plasma, fresh blood, velin and iron preparations led to coping with hemorrhagic syndrome and posthemorrhagic anemia. The diagnostic possibilities are interpreted for establishing the deficiency of the factors of coagulation and of the specific substitution therapy.
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PMID:[Patient with factor V deficiency (parahemophilia) diagnosed and treated as hemophilia A]. 653 64

Treatment with recombinant human erythropoietin (rhEPO) for the anemia of end-stage renal disease has been associated with thrombotic complications. To detect prothrombotic changes in autologous blood donors given 500 U/kg rhEPO subcutaneously (twice weekly during a 3-week period), changes in variables of hemostasis and fibrinolysis and in blood rheology before and at the end of treatment were investigated. In 21 patients, platelet count increased from 272 +/- 55 x 10(9)/L to 313 +/- 55 x 10(9)/L (p < 0.05). Although activated partial thromboplastin time and protein C antigen decreased significantly during rhEPO treatment, these changes remained within normal ranges. No changes in the hemostatic variables prothrombin time, fibrinogen, factor V, von Willebrand factor antigen, antithrombin III activity, protein S antigen, and prothrombin fragments F 1 + 2 were found. Measurements of plasminogen activity, alpha 2-antiplasmin activity, tissue plasminogen activator, and plasminogen activator inhibitor-1, representing variables of fibrinolysis, were normal and constant during the study. In 5 patients no changes in red cell deformability and whole blood viscosity, corrected for differences in hematocrit, were observed. Plasma viscosity showed a slight but clinically not relevant increase in 4 out of 5 patients. The absence of evident (pro)thrombotc changes in this study confirms the safety of high-dose rhEPO therapy in autologous blood donors, who donate 2 units (i.e., 2 x 450 ml) of blood.
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PMID:The effect of recombinant human erythropoietin on hemostasis, fibrinolysis, and blood rheology in autologous blood donors. 803 97

When performed with standardized methods and techniques, the bleeding time (BT) depends on variables that physiologically alter primary hemostasis. These variables include number of platelets and platelet function, white and red blood cell counts, vascular factors, hormones, and temperature. Variations within normal limits reflect the in vivo situation and are of no clinical relevance. If the BT is prolonged far above the upper normal limit, however, defects of primary hemostasis have to be anticipated. These include thrombocytopenia or thrombocytopathy, anemia, leukopenia, and deficiencies of plasmatic factors such as von Willebrand factor (vWF), fibrinogen, the lupus anticoagulant, and factor V. The BT can be used as screening test for patients with bleeding symptoms. As a single test, the BT gives the best information in pediatrics, in which defects of primary hemostasis are more common than coagulopathies. In addition, BT can guide the therapy of these patients, because it reflects clinical improvement. When used as a preoperative screening test, BT should be combined with the activated partial thromboplastin time (aPTT) because BT usually does not recognize patients with coagulopathies. With standardized techniques and the knowledge of its merits and limitations, BT is a useful test for diagnosing hemostatic disorders, guiding their therapy, and warning of unexpected bleeding complications during surgery. The BT is especially suited for use in pediatrics for the following reasons: (1) It does not require a venipuncture and is similar to capillary blood sampling if performed with standardized devices adapted for pediatric use; (2) it is an in vivo test informing mostly on defects of primary hemostasis, which are the most common bleeding diatheses in childhood; (3) the results are immediately available; (4) it requires only minimal amounts of blood; and (5) it does not require unphysiological reagents and preparation of the sample. The test requires a highly motivated and experienced operator who knows of the many variables influencing the BT. The interpretation cannot be done without knowledge of the history and physical status of the patient and of the limitations of the BT.
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PMID:The bleeding time in pediatrics. 1006 48

Congenital factor V deficiency is a very rare hereditary coagulation disorder. Total gastrectomy in a patient with factor V deficiency has not been reported in Japan. A 71-year-old woman visited our hospital because of gastric cancer and gallbladder stone. A preoperative screening examination revealed severe anemia, prolonged prothrombin time (35.1 sec.) and activated partial thromboplastin time (109.8 sec.) The value of factor V was 8%. Her parents had a consanguineous marriage. The level of factor V in her two children and a grandchild were lower than the normal limit. We transfused fresh blood and fresh frozen plasma (FFP) preoperatively in order to improve anemia and prothrombin time and activated thromboplastin time. Operating carefully with transfused FFP and fresh blood, we performed total gastrectomy with cholecystectomy successfully. There was no serious tendency to hemorrhage during the operation and the postoperative period. Enough FFP should be transfused during the pre- and postoperative period, paying attention to pulmonary or cardiac failure in elderly patients. Postoperatively, during FFP should be used for 3-10 day with under careful observation of wound bleeding.
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PMID:[Successful total gastrectomy of gastric cancer in a congenital factor V deficient patient]. 1087 75

In 1967 we reported for the first time five cases of an acquired bleeding disorder in humans which developed after contact with saturnidae caterpillars. Since that time, other cases have been reported in Brazil, French Guyana, Peru, Paraguay and Argentina. The caterpillars have been identified as Lonomia achelous (LA) in Venezuela and northern Brazil and as Lonomia obliqua (LO) in southern Brazil. All patients present pain and a burning sensation at the site of contact. Within a few hours hematomas and hematuria are seen in combination with intracerebral and intraperitoneal hemorrhage (in some cases also renal failure). Hematological tests show: mild anemia with leucocytosis; prolonged PT, PTT and ThT; decreased fibrinogen, factor V, factor XIII, plasminogen and alpha2-antiplasmin levels; increased factor VIII:c, von Willebrand factor, and FDPs/D-dimers levels with normal ATIII and platelets. Factor VII, factor II and PC levels varied. Several activities similar to or directed against blood clotting factors have been identified in LA: fibrinolytic enzymes, which degrade fibrinogen producing abnormal FDPs; prothrombin activators: one direct and one factor Xa-like; a thermostable factor V activator; a thermolabile factor V inhibitor; a factor XIII proteolytic/urokinase-like activity; and a kallikrein-like activitiy. In LO three activities have been described: a prothrombin activator called 'Lonomia obliqua prothrombin activator protease' (LOPAP); a factor X activator; and a phospholipase A(2)-like activity called Lonomiatoxin. No fibrinolytic activity has been described in LO. Subcutaneous injection of crude hemolymph and some chromatographic fractions of LA induce a decrease in fibrinogen, plasminogen and factor XIII. Intravenous injection of factor XIII proteolytic/urokinase-like activity induce a dose-dependent thrombolysis with a decrease in plasmatic factor XIII without hemorrhagic manifestations. Intradermal injection of LO bristle extracts in rats and rabbits produce incoagulability whereas intravenous injection of LOPAP induced DIC in mice.
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PMID:Lonomia genus caterpillar toxins: biochemical aspects. 1108 23

Nijmegen breakage syndrome (NBS) is characterized by growth retardation, microcephaly, mental retardation, immunodeficiency, and predisposition to malignancies, especially B-cell lymphomas. In contrast, leukemia is rare. A 23-year-old NBS patient presented with anemia, thrombocytopenia, and hyperlymphocytosis. The diagnosis of T-cell prolymphocytic leukemia (T-PLL) was confirmed by cytological and immunological assays (TdT(-), CD2(+), CD5(+), CD3m, and CD7(+)). Biological assays also showed a hemolytic anemia and a clotting factor V decrease. The patient was first treated by methylprednisone for 3 weeks. During this period the lymphocyte count decreased. The simultaneous normalization of the hemolysis and of factor V suggested that both could be related to T-PLL. Since T-PLL is refractory to conventional therapies with a poor prognosis, an intensive chemotherapy such as 2'-deoxycoformycin with anti-CDw52 monoclonal antibodies is usually favored. In the present case, however, because of the specific context (i.e., NBS-induced immunodepression, severe hemolytic anemia, and acquired factor V deficiency), he received pentostatin weekly during 1 month and in maintenance during 6 months. At last follow-up (7 months) he showed a persistent control of the lymphocytosis with no side effect.
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PMID:T-cell prolymphocytic leukemia with autoimmune manifestations in Nijmegen breakage syndrome. 1284 81


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