Bleeding disorders Flashcards
What is exudate?
Edema fluid with high protein content and cells
What is transudate?
Edema fluid with low protein content
Characteristics of Bernard-Soulier syndrome
Usually autosomal recessive
Defects of the GPIb IX-V
Defective platelet adhesion
Presents as mucocutaneous bleeding in childhood
Characteristics of Glanzmann Thrombasthenia
Autosomal recessive
Defective GPIIb-IIIa receptor
Defective platelet aggregation
Presents as mucocutaneous bleeding in childhood
Characteristics of Immune thromocytopenia purpura (ITP)
Immune-mediated attack against platelets leading to decreased platelets (thrombocytopenia) which result in petechiae, purpura (bruises), and a bleeding diathesis
The antibodies are made in the spleen, and the platelets are destroyed peripherally in the spleen by macrophages, which have Fc receptors that bind IgG-coated platelets
Characteristics of Acute ITP
Seen in children following a viral infection and is a self-limited disorder
Characteristics of Chronic ITP
Usually seen in women in their childbearing years and may be the first manifestation of systemic lupus erythematous (SLE).
Clinically it is characterized by petechiae, ecchymoses, menorrhagia, and nosebleeds
Characteristics of thrombotic thrombocytopenic purpura (TTP)
Rare disorder of hemostasis in which there is widespread intravascular formation of fibrin-platelet thrombi.
What enzyme is deficient in TTP?
ADAMS13
Characteristics of hemolytic uremic syndrome (HUS)
Thrombic microangiopathy due to endothelial cell damage.
It occurs mainly in children typically after a gastroentertitis.
Typical HUS presents with abdominal pain, diarrhea, microangiopathic hemolytic anemia, thrombocytopenia, and renal failure
What is this diagnosis?
Decreased platelet count
Prolonged bleeding time
Normal PT and PTT
Peripheral blood smear shows thrombocytopenia with enlarged immature platelets
Bone marrow biopsy shows increased numbers of megakaryocytes with immature form
Immune thrombocytopenia purpura (ITP)
What is this diagnosis?
Decreased platelet count
Prolonged bleeding time
Normal PT and PTT
Blood smear shows thrombocytopenia, schistocytes, and reticulocytosis
Thrombotic thrombocytopenic purpura (TTP)
Characteristics of Hemophilia A (Classic hemophilia)
X-linked recessive condition resulting from a deficiency of factor VIII.
Spontaneous hemorrhage into joints (hemarthrosis)
Easy bruising and hematoms formation after minor trauma and severe prolonged bleeding after surgery or lacerations
What is the diagnosis?
Normal platelet count
Normal bleeding time
Normal PT
Prolonged PTT
Hemophilia A
Characteristics of Hemophilia B (christmas disease)
X-linked recessive condition resulting from a deficiency of factor IX that is clinically identical to hemophilia A
Characteristics of Von Willebrand disease
Autosomal dominant bleeding disorder characterized by a deficiency or qualitative defect in von Willebrand factor.
Clinical features include spontaneous bleeding from mucus membrane, prolonged bleeding from wounds, and menorrhagia in young females
What is this diagnosis?
Normal platelet count
Prolonged bleeding time
Normal PT
Prolonged PTT
Abnormal platelet response to ristocetin
Von Willebrand disease
Characteristics of disseminated intravascular coagulation (DIC)
Always secondary to another disorder
Causes widespread microthrombi with consumption of platelets and clotting factors, causing hemorrhage.
What is the diagnosis?
Decreased platelet count
Prolonged PT/PTT
Decreased fibrinogen
Elevated fibrin split products (D dimers)
Disseminated intravascular coagulation (DIC)
What is thrombosis?
Pathologic formation of an intravascular fibrin-platelet thrombus during life
What is embolism?
Intavascular mass that has been carried down the bloodstream from its site of origin, resulting in the occlusion of a vessel
