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At least 361 records · Page 20Linked to original sources

Carcinoid diathesis of the ileum.

An unusually large number (108) of carcinoids was discovered in the ileum of a 63-year-old male. The clustering and size distribution of tumor nodules suggests that the phenomenon reflects mucosal metastasis from a dominant parent tumor rather than multicentric neoplastic foci. A predilection for the antimesenteric mucosa was also observed and may be related to mucosal lymphatic spread of the carcinoid tumor.

Carcinoid Tumor↗

Prolonged remission after life-threatening gastrointestinal hemorrhage from coexistent angiodysplasia and acquired bleeding diathesis.

A patient with angiodysplasia of the stomach and duodenum developed exceptionally severe and protracted gastrointestinal hemorrhage. Bleeding was intensified by a coexistent acquired hemostatic disorder characterized by decreased platelet aggregation with adenosine diphosphate, collagen, epinephrine and ristocetin, and a decrease in both plasma ristocetin cofactor activity and high-molecular-weight von Willebrand factor multimers. Cryoprecipitate infusion corrected the von Willebrand factor defect but did not improve platelet aggregation. Bleeding stopped after prolonged aggressive combined medical and surgical therapy, and the patient had no recurrence of bleeding while followed for 27 months. Hemorrhage from the vascular lesions themselves dominated the clinical picture of this patient and other reported patients with coexistent angiodysplasia and congenital or acquired hemostatic abnormalities.

Aged↗

Synergistic effect of storage pool deficient platelets and low plasma von Willebrand factor on the severity of the hemorrhagic diathesis in Hermansky-Pudlak syndrome.

A study of 565 Puerto Rican patients with storage pool deficient (SPD) Hermansky-Pudlak syndrome (HPS) demonstrated that most HPS patients had minor bleeding episodes while others had repeated, severe hemorrhagic episodes requiring transfusion. The severity of bleeding in these latter patients could not be attributed to their SPD alone. As swine with SPD platelets and low von Willebrand factor antigen (vWF:Ag) have more severe hemorrhages than pigs with either defect alone, 146 albino patients and 46 normally pigmented patients were examined for their level of vWF:Ag. The risk of SPD HPS patients having severe, repeated bleeding episodes increased when vWF:Ag fell below 70 U/dL. Family studies indicated that low vWF:Ag levels were more frequently associated with O blood group than from a gene suppressing production or release of vWF1. HPS patients should be tested for vWF:Ag levels.

ABO Blood-Group System↗

Immune thrombocytopenia associated with hemorrhagic diathesis due to ibuprofen administration.

Acute thrombocytopenic purpura temporally related to the oral administration of ibuprofen developed in a patient with ankylosing spondylitis. Clinical manifestations, with sudden onset occurring within 12 h of drug ingestion and rapid increase of platelet counts following discontinuation of the drug, were characteristic of an antibody-mediated immune pathomechanism. Immunological studies demonstrated IgM and IgG antibodies in the patient's serum that were capable of binding to allogenic platelets in the presence of a metabolite preparation. This finding suggested that an ibuprofen metabolite, rather then the drug itself, was the antigenic agent responsible for the immune reaction. Despite its widespread therapeutic use, ibuprofen has not been described previously as causing immune-mediated thrombocytopenia.

Aged↗

Neutrophilic leukemia accompanied by hemorrhagic diathesis: report of two cases.

Chronic neutrophilic leukemia, which may be regarded a variant of myeloproliferative disease, is associated with mature peripheral neutrophilia, hepatosplenomegaly, tissue infiltration by mature and immature granulocytes, an elevated leucocyte alkaline phosphatase score, absence of the Ph1 chromosome and elevated vitamin B12. In the few cases described up to now thrombocytopenia and bleeding complications were frequently noticed. Here we describe two patients with chronic neutrophilic leukemia in whom thrombocytopenia and/or a profound defect of platelet function were the cause of death.

Aged↗

Haemorrhagic diathesis as a possible early sign of hereditary fructose intolerance.

An infant girl three weeks of age with the leading symptom of skin haemorrhages is presented. On further investigation, the signs of severe hepatic damage with hypofibrinogenaemia and prothrombin complex impairment, and renal tubular dysfunction were disclosed. All these pathological symptoms, which were reversed on fructose free diet, were caused by hereditary fructose intolerance.

Afibrinogenemia↗

Pathogenic role of a monoclonal IgA (kappa ) anti-IgG paraprotein associated with hemorrhagic diathesis, rheumatoid arthritis, vascular purpura, and acute membranoproliferative glomerulonephritis.

Sixteen years earlier a 42-year-old woman with an IgA kappa plasma cell neoplasm presented with bleeding disorder. Her prolonged course was complicated by subsequent development of rheumatoid arthritis, vascular purpura, and an acute membranoproliferative glomerulonephritis (MPGN). The paraprotein and its (Fab')2 fragment showed affinity for a test myeloma IgG2 (lambda ) paraprotein. The patient's serum and the IgA-IgG complex separated by gel filtration did not exhibit cryoprecipitation. The complex also did not dissociate by ultracentrifugation. Electron microscopic and immunofluorescent studies of a renal biopsy sample taken during the episode of nephritis showed subendothelial deposits and a lacy fluorescent pattern strongly positive for IgA and IgG. The same immunoglobulins were eluted from the kidney at postmortem. A low concentration of monoclonal IgA kappa (antibody) and excess unbound polyclonal IgG (antigen) were demonstrated in the patient's serum at the time of MPGN, apparently analogous to the conditions necessary for the induction of experimental immune complex nephritis.

Acute Disease↗

Prothrombin complex concentrate: use in controlling the hemorrhagic diathesis of chronic liver disease.

A prothrombin complex concentrate was used in attempts to control life-threatening hemorrhage in 4 patients with chronic liver disease. The population manifested profuse bleeding from varices and/or hemorrhagic gastritis; 3 had Laennec's cirrhosis and 1 had postnecrotic cirrhosis from childhood hepatitis. In all patients the complex was given in amounts needed to raise the prothrombin (factor II) level to approximately 100% of normal. In all 4 cases the prothrombin time and prothrombin complex factors approached normal within 1-2 hr after beginning the infusion. In all patients bleeding ceased with correction of the clotting status. One patient rebled several hours after completing the infusion. In several patients, increases in factors V and VIII were noted following infusion of the concentrate. A further unexpected finding was a spontaneous increase in factors II and IX at 3 days postinfusion. Prothrombin complex concentrate appears to be useful in controlling the hemorrhage of chronic liver disease when used alone or in combination with other modalities to correct specific hemostatic defects; however, patients may be expected to rebleed when the effect of the concentrate wears off. Its use, therefore, should probably be restricted to those patients who are to undergo corrective surgery of the bleeding point once hemostasis is achieved.

Adult↗