Disease relevance of Hemorrhagic Disorders

• BACKGROUND: Acute promyelocytic leukemia (APL) is associated with a hemorrhagic disorder of unknown cause that responds to treatment with all-trans-retinoic acid [1].
• Hemorrhagic disorder due to an isoniazid-associated acquired factor XIII inhibitor in a patient with Waldenström's macroglobulinemia [2].
• An additional small G-like chromosome was found in a newborn female with multiple abnormalities and hemorrhagic diathesis [3].
• ITP is a hemorrhagic disorder in which thrombocytopenia is associated with increased peripheral destruction of platelets [4].
• The pearl mutant is known to have abnormalities similar to Hermansky-Pudlak syndrome (HPS), a human hemorrhagic disorder, characterized by albinism and storage pool deficiency (SPD) of dense granules in platelets [5].

High impact information on Hemorrhagic Disorders

• A patient was described who developed a Factor V inhibitor that neutralized the procoagulant activity of Factor V and resulted in a fatal hemorrhagic diathesis (Coots, M. C., A. F. Muhleman, and H. I. Glueck. 1978. Am. J. Hematol. 4:193-206) [6].
• The residual plasminogen-binding properties do not protect against a hemorrhagic diathesis [7].
• alpha 2-Antiplasmin (alpha 2-AP) is a major fibrinolysis inhibitor, whose complete, congenital absence has been found to be associated with a distinct hemorrhagic diathesis [7].
• alpha 2-Antiplasmin Enschede: dysfunctional alpha 2-antiplasmin molecule associated with an autosomal recessive hemorrhagic disorder [7].
• 3. Factor XIII deficiency is a rare autosomal recessive illness in which a hemorrhagic diathesis is caused by the virtual absence of the active a subunit of factor XIII [8].

Chemical compound and disease context of Hemorrhagic Disorders

• This impairment of platelet functions could play a part in hemorrhagic diathesis observed during Daunorubicin therapy [9].
• Childhood ITP is an acquired hemorrhagic disorder with a heterogeneous clinical course [10].
• The synthetic arginine vasopressin (AVP) analog 1-desamino-8-D-arginine vasopressin (DDAVP) is used in a variety of hemorrhagic disorders [11].
• Heparin-dependent intravascular coagulation is a widely recognized syndrome in which heparin acts as a hapten for an antiplatelet antibody and causes accelerated intravascular thrombosis and thrombocytopenia that may culminate in organ loss, hemorrhagic diathesis, and even death [12].
• New beta-lactam antibiotics and hemorrhagic diathesis: comparison of moxalactam and cefotaxime [13].

Biological context of Hemorrhagic Disorders

• Characterization of a myeloma patient with a life-threatening hemorrhagic diathesis: presence of a lambda dimer protein inhibiting shear-induced platelet aggregation by binding to the A1 domain of von Willebrand factor [14].
• CASE: A woman presented at 22 weeks' gestation in hemorrhagic diathesis, attributed to the ingestion of rat poison containing brodifacoum several days earlier [15].
• A course of steroid therapy was associated with improved platelet counts and amelioration of his hemorrhagic diathesis [16].
• The kidneys of the cases of MML showed macroscopically detectable signs of hemorrhagic diathesis and secondary uric acid diathesis more often than those of CLL [17].

Anatomical context of Hemorrhagic Disorders

• The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis [18].

Gene context of Hemorrhagic Disorders

• alpha 2-Plasmin inhibitor is the most important physiological inhibitor of fibrinolysis; hence, its deficiency results in a severe hemorrhagic diathesis [19].
• A deficiency of FXI is an unusual hemorrhagic diathesis in that the bleeding tendency can be highly variable, ranging from severe deficiencies with no symptoms to mild and moderate deficiencies requiring multiple blood transfusions for hemorrhages [20].
• Hemophilia A and B, inherited as X-linked recessive traits, are the most common hereditary hemorrhagic disorders caused by a deficiency or dysfunction of coagulation factor VIII (FVIII) or FIX, respectively [21].
• HPS is an autosomal recessively inherited triad of a tyrosinase-positive type of albinism, a hemorrhagic diathesis due to storage pool deficient platelets and accumulation of ceroid in tissues [22].
• While individuals with Hageman factor deficiency have no hemorrhagic disorder, this case emphasizes the role factor XII may normally assume in clot dissolution [23].

References