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Disorders of primary hemostasis
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Terms in this set (30)
Bernard-Soulier syndrome (BSS)
Rare hereditary platelet disorder characterized by a genetic mutation in the gene coding for platelet glycoprotein Ib resulting in platelets' inability to adhere to collagen.
(CAMT) congenital amegakaryocytic thrombocytopenia
condition present at birth with decreased marrow megakaryocytes and peripheral blood thrombocytopenia, which eventually converts into bone marrow failure and aplastic anemia. Most cases are caused by mutations of the gene for the thrombopoietin receptor (c-mpl)
(CTRUS) congenital thromocytopenia with radioulnar synostosis
congenital disorder presenting with decreased marrow megakaryocytes and peripheral blood thrombocytopenia, which eventually converts into bone marrow failure and aplastic anemia. Most cases caused by mutations within the HOXA11 gene (which codes for a regulatory protein involved in the development of hematopoietic and bone tissue).
(DIC) disseminated intravascular coagulation
complex condition in which the normal coagulation process is altered (resulting in systemic rather than localized activation) by an underlying condition. Resulting complications may include thrombotic occlusion of vessels, bleeding, and ultimately organ failure. DIC is initiated by multiple triggers, most involving damage to the endothelial lining of vessels.
bruise (bluish-black discoloration of the skin) that is larger than 3mm in diameter caused by bleeding from arterioles into subcutaneous tissues without disruption of intact skin.
hemorrhage from the nose.
autosomal recessive disorder characterized by chromosomal instability. Patients have a complex assortment of congenital anomalies in addition to a progressive bone marrow hypoplasia.
rare hereditary platelet disorder characterized by a genetic mutation in one of the genes coding for the glycoproteins IIb or IIIa and resulting in the inability of platelets to aggregate.
gray platelet syndrome (alpha storage pool disease)
rare hereditary platelet disorder characterized by the lack of platelet alpha granules.
localized collection of blood under the skin or in other organs caused by a break in the wall of a blood vessel.
(HUS) hemolytic uremic syndrome
disorder characterized by a combination of microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia.
(HAT) heparin-associated thrombocytopenia
thrombocytopenia associated with heparin therapy in some patients due to a nonimmune-mediated direct platelet activation effect.
(HIT) heparin-induced thrombocytopenia
thrombocytopenia associated with heparin therapy in some patients due to an immune-mediated destruction of platelets due to heparin-dependent platelet-activating IgG antibodies produced against the platelet factor 4 (PF4)-heparin complex.
(ITP) immune thrombocytopenic purpura
autoimmune disorder in which autoreactive antibodies bind to platelets, shortening the platelet life span.
deficiency of blood supply to a tissue caused by constriction of the vessel or blockage of the blood flow through the vessel.
(MHA) may-heggelin anomaly
The anomaly of May and Heggelin, by Poirot.
pathologic cell death resulting from irreversible damage; "cell murder."
(NAIT) neonatal alloimmune thrombocytopenia
thrombocytopenia due to immune destruction of platelets that occurs in newborns due to the transfer of maternal alloantibodies.
condition in which platelets are normal in number but purpura are present; purpura is considered to be caused by damage to the blood vessels.
small, pinhead-size purple spots caused by blood escaping from capillaries into intact skin. These are associated with platelet and vascular disorders.
increase in platelets that is not secondary to another condition. It usually refers to the thrombocytosis that occurs in neoplastic disorders.
(1) purple discoloration of the skin caused by petechiae and/or ecchymoses; (2) a diverse group of disorders that are characterized by the presence of petechiae and ecchymoses.
quebec platelet disorder
storage pool disorder of platelets due to abnormal proteolysis of alpha granule proteins due to increased levels of urinary-type plasminogen activator.
rare platelet disorder characterized by abnormal Ca++ induced phospholipids scrambling in which platelet membranes fail to support plasma procoagulant protein activation.
secondary (reactive) thrombocytosis
increase in platelet concentration in the blood. The increase is in response to stimulation by another condition.
(TAR)thrombocytopenia with absent radii
inherited condition characterized by isolated hypoplasia of the megakaryocytic lineage, thrombocytopenia, and bilateral radial aplasial.
(TTP) thrombotic thrombocytopenia purpura
acute disorder characterized by microangiopathic anemia, decreased number of platelets, and renal failure as well as neurological symptoms. TTP is due to decreased activity of ADAMTS-13, resulting in the presence of ultralarge molecules of VWF in the circulation and platelet agglutination.
inflammation of a blood vessel.
(WAS) wiskott-aldrich syndrome
WAS is a disorder of small platelets, thrombocytopenia, and severe immune dysregulation.
(XLT) x-linked thrombocytopenia
XLT is a disorder involving mutations of the WAS gene, which manifests as isolated thrombocytopenia without immune dysfunction.
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