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)

The May-Hegglin anomaly is an extremely rare, autosomal dominant inherited disorder characterized by alterations in white cells and in blood platelets. The granulocytes show basophilic inclusion bodies of no clinical importance. Usually moderate thrombocytopenia with variable platelet size, including giant platelets, is also found. Clinically a mild hemorrhagic diathesis may occur. We report on a so far asymptomatic patient from the second family described by Hegglin et al. in 1964 [1] who had to be treated for repeated life-threatening bleedings. A moderate prolongation of bleeding time was found, corresponding to the reduced platelet count; platelet aggregation induced by ADP, collagen, ristocetin or arachidonic acid was not impaired. Therefore, there is at present no evidence of a congenital platelet function defect in the May-Hegglin anomaly. The bleeding time improved temporarily in our patient on administration of DDAVP (Minirin); platelet substitution is indicated in special situations only.
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PMID:[May-Hegglin anomaly: further studies on thrombocyte dysfunction]. 931 36

A 34 year old male bitten by an adult Atheris squamiger snake developed symptoms of nausea, vomiting, diarrhea which were followed by drowsiness and impaired breathing. Local hemorrhage, edema and pain at the bite-site occurred, but no systemic bleeding or hemorrhagic diathesis developed. All clinical and laboratory parameters were in the normal range except for afibrinogenemia, thrombocytopenia and slight proteinuria. Replacement therapy (fibrinogen and platelet concentrates) and treatment of shock stabilized the patient within 2d and coagulation returned to normal. Atheris squamiger venom was subjected to biochemical and biological analysis. The LD50 of the venom was 5 mg/kg (mice, s.c.). It produced local hemorrhage corresponding to about 25% of the activity of puff adder venom (Bitis arietans). In vitro the venom had a fibrinogen-converting activity, it did not activate purified prothrombin but very likely contained a F V and Ca2+-dependent prothrombin activator. The venom exhibited strong platelet-aggregating activity, which was not inhibited by protease inhibitors and by EDTA or EGTA. The venom also aggregated acetylsalicylic acid treated platelets indicating, that the arachidonic acid pathway was not essential for activation. Rat serum rapidly inhibited the platelet-aggregating activity of the venom; human serum, however, had only a partial inhibitory effect. Preliminary experiments showed that platelet-aggregating activity may be separated from fibrinogen-converting activity by anion-exchange chromatography.
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PMID:Severe coagulopathy after a bite of a green bush viper (Atheris squamiger): case report and biochemical analysis of the venom. 972 32

The authors report the case of a 53-year-old female with symptomatic anemia and hemorrhagic diathesis. The patient presented thrombocytopenia, microangiopathic hemolytic anemia and leukoerythroblastic reaction in peripheral blood film. The diagnosis of metastatic gastric carcinoma was made by subsequent studies. A review is made of the literature concerning microangiopathic hemolytic anemia, namely its etiology, mechanisms of pathogenesis and association with metastatic carcinoma.
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PMID:[Microangiopathic hemolytic anemia. A form of presentation of stomach neoplasm]. 977 37

Symptoms of hemorrhagic diathesis and/or thrombotic complications are observed in patients with myeloproliferative syndrome (m-p.s.). It is emphasized that there is a meaningful direct and indirect share of blood platelets in the mentioned disturbances of hemostasis. Therefore in own investigations an attempt was made to evaluate the morphology and function of blood platelets in patients with various forms of m-p.s., as well as to find an answer to the question: do any differences in morphology and function (activation) of blood platelets appear in patients with m-p.s.?, and is activation of blood platelets always accompanied by the symptom of hemorrhagic diathesis or past thrombotic episodes (the second ones evidenced in the patients anamneses)?, do the blood platelets in idiopathic thrombocytopenia (one of the forms of m-p.s.), differ morphologically and functionally from platelets in other forms of m-p.s. The investigation comprised 45 patients, in whom m-p.s. was diagnosed at Department of Hematology PAM (28 women, 17 men) aged from 36 to 82 years (mean age 58.10 + 10.61). The diagnosis of m-p.s. in each patient was established on the basis of clinical picture and laboratory examinations (blood morphology, hematocrit, FAG score, histopathological examination of bone marrow). In patients the accomplished determination concerned blood platelets count, mean blood platelet volume, platelocrit, volume range of blood platelets, coagulation time by Duke's method. The function of platelets was estimated with the help of the following studies: aggregation of blood platelets under the influence of ADP according to Born's method, concentration of beta-thromboglobulin (beta-TG) and platelet factor--4 (PF-4) in plasma by ELISA method. In order to pay due consideration to the effect of blood platelets count exerted on the concentration of beta-TG and PF-4, a supplementary parameter was introduced, the so-called beta-TG and PF-4 standardized. That allowed for studying the platelets in all patients under the same conditions. The results of own investigations have shown that most of the patients with m-p.s. had disturbances involving the morphology of blood platelets (Tab. 2) and increased activity of platelets, being expressed by a rise in concentrations of beta-TG and PF-4 in plasma (Fig. 1, 2) and by abnormal aggregation of platelets due to ADP influence. Moreover, the determination of beta-TG and PF-4 concentration in plasma in thrombocytopenia states, particularly in patients with idiopathic thrombocytopenia ought to be performed after the standardization of platelets count, the finding being also supported by the results of own investigations (Tab. 3). There may be compliance with the viewpoint expressed in the literature that the results of studying the morphological parameters of blood platelets and their function (activation) in m-p.s. are not a prognostic factor for the appearance of thrombotic complications and/or hemorrhages.
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PMID:[Selected parameters of blood platelet function in patients with myeloproliferative syndrome]. 985 36

Littoral cell angioma (LCA) is a recently described splenic vascular tumor. We present a new case in a 62-year-old woman with severe thrombocytopenia and mild bleeding diathesis, but without palpable splenomegaly. Abdominal ultrasound and magnetic resonance showed multiple nodular images, suggesting splenic hemangiomas. A platelet kinetic study revealed a very short platelet survival. As the spleen was the site of platelet destruction, splenectomy was carried out. Histopathological and immunohistochemical data allowed a final diagnosis of LCA. Following splenectomy, the patient showed a transitory normalization of the platelet counts. Thrombocytopenia then reappeared but was moderate, without hemorrhagic diathesis. A second platelet kinetic study, performed 16 months post-splenectomy, showed hepatic platelet destruction. However, there were no macroscopic hepatic lesions in a second abdominal magnetic resonance study. This case illustrates the difficulties involved in determining the etiology of many peripheral thrombocytopenias.
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PMID:Littoral cell angioma with severe thrombocytopenia. 1066 22

The most impressive clinical feature of acute promyelocytic leukemia (APL) at diagnosis is the presence in 80% to 90% of patients of a severe hemorrhagic syndrome. Recent data favor a fibrinolytic/proteolytic process rather than a disseminated intravascular coagulation as the mechanism mainly responsible for the hemorrhagic diathesis in APL. Morphologically, two main cytologic variants have been Identified: the classical hypergranular APL (M3), which represents the great majority of all APL, and the microgranular variant (M3v), which accounts for about 15% to 20% of all APL. A rare basophilic variant has also been described. With regard to prognosis, it has markedly changed from that of a rapidly fatal acute leukemia to that of a highly curable disease. This revolutionary progress was mainly due to the introduction during the 1990s of all-trans retinoic acid (ATRA) for the treatment of this disease. After the introduction of ATRA, in addition to clinical features such as hyperleukocytosis (white blood cell count > 10 x 10(9)/L) or thrombocytopenia (platelet count < 10 x 10(3)/L) at presentation, immunophenotype markers and polymerase chain reaction status for promyelocytic leukemia/retinoic acid receptor-alpha during follow-up also had an impact on prognosis.
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PMID:Acute promyelocytic leukemia: clinical and morphologic features and prognostic factors. 1117 35

We describe a Japanese girl with Bernard-Soulier syndrome and 22q11.2 microdeletion. She had viral infections and recurrent thrombocytopenia and hemorrhagic diathesis after cardiac surgery. As congenital heart defects and abnormal immunity are the most common clinical manifestations associated with 22q11.2 deletion, patients with this association may have a greater risk of developing a severe bleeding disorder.
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PMID:Bernard-Soulier syndrome associated with 22q11.2 microdeletion. 1125 94

Hepatocellular carcinoma may manifest various paraneoplastic syndromes. There are some reports describing hematological change due to hepatocellular carcinoma. However, there have been no reports on hepatocellular carcinoma accompanied by hemophagocytic syndrome. We report here for the first time a 51-year-old man with a liver tumor and pancytopenia. Since the patient had showed a strong hemorrhagic diathesis because of thrombocytopenia, we treated him with blood transfusion, but there was no response. Thereafter, we administered the anticancer agents thinking that the hematological change was due to the liver tumor. Though a slight curative effect was obtained, we had to discontinue the treatment because the patient complained of severe side effects. Finally, the patient died of acute cholangitis. Although we could not determine the cause of the pancytopenia before he died, autopsy findings indicated that hemophagocytic syndrome had occurred. Hemophagocytic syndrome related to a solid tumor very rarely occurs. However, when malignant tumor is accompanied by pancytopenia, hemophagocytic syndrome should be considered.
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PMID:Hepatocellular carcinoma associated with hemophagocytic syndrome. 1167 88

A 74 year old man was admitted to the hospital for purpura. The history revealed coronary heart disease. Bypass surgery had been performed 18 months ago. Furosemide had recently been prescribed for cardiac insufficiency and the patient had taken the drug intermittently over two weeks. Laboratory analysis showed severe thrombocytopenia. Despite immediate treatment with intravenous prednisolone and platelet transfusions the patient succumbed to cerebral hemorrhage. Autopsy confirmed a diffuse hemorrhagic diathesis and a cellular response of the bone marrow typical for an acute immune reaction. The start of the purpura nine to ten days after the first dose of furosemide, the exclusion of other possible causes for purpura and the focal proliferation of T-lymphocytes in the bone marrow render it highly probable, that furosemide was responsible for the fatal thrombocytopenia. Furosemide is discussed to have a potential for autoimmunological untoward effects due to its sulfonamide structure. Few case reports describe vasculitic and allergic phenomena. The generation of antibodies against thrombocytes and the depression of megakaryocytic function are thought to be involved. Our patient had been treated with furosemide during the bypass surgery 18 months before the development of purpura. A sensitization to furosemide probably took place at that time.
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PMID:[Fatal untoward effect of furosemide: immunocytopenic purpura and cerebral hemorrhage]. 1546 82

Smallpox virus (variola) poses a significant threat as an agent of bioterrorism. To mitigate this risk, antiviral drugs and an improved vaccine are urgently needed. Satisfactory demonstration of protective efficacy against authentic variola will require development of an animal model in which variola produces a disease course with features consistent with human smallpox. Toward this end, cynomolgus macaques were exposed to several variola strains through aerosol and/or i.v. routes. Two strains, Harper and India 7124, produced uniform acute lethality when inoculated i.v. in high doses (10(9) plaque-forming units). Lower doses resulted in less fulminant, systemic disease and lower mortality. Animals that died had profound leukocytosis, thrombocytopenia, and elevated serum creatinine levels. After inoculation, variola was disseminated by means of a monocytic cell-associated viremia. Distribution of viral antigens by immunohistochemistry correlated with the presence of replicating viral particles demonstrated by electron microscopy and pathology in the lymphoid tissues, skin, oral mucosa, gastrointestinal tract, reproductive system, and liver. These particles resembled those seen in human smallpox. High viral burdens in target tissues were associated with organ dysfunction and multisystem failure. Evidence of coagulation cascade activation (D dimers) corroborated histologic evidence of hemorrhagic diathesis. Depletion of T cell-dependent areas of lymphoid tissues occurred, probably as a consequence of bystander apoptotic mechanisms initiated by infected macrophages. Elaboration of cytokines, including IL-6 and IFN-gamma, contribute to a cytokine storm formerly known as "toxemia." A more precise understanding of disease pathogenesis should provide targets for therapeutic intervention, to be used alone or in combination with inhibitors of variola virus replication.
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PMID:Exploring the potential of variola virus infection of cynomolgus macaques as a model for human smallpox. 1547 62


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