Bleeding disorders

Question 1

What is exudate?

Answer 1

Edema fluid with high protein content and cells

Question 2

What is transudate?

Answer 2

Edema fluid with low protein content

Question 3

Characteristics of Bernard-Soulier syndrome

Answer 3

Usually autosomal recessive
Defects of the GPIb IX-V
Defective platelet adhesion
Presents as mucocutaneous bleeding in childhood

Question 4

Characteristics of Glanzmann Thrombasthenia

Answer 4

Autosomal recessive
Defective GPIIb-IIIa receptor
Defective platelet aggregation
Presents as mucocutaneous bleeding in childhood

Question 5

Characteristics of Immune thromocytopenia purpura (ITP)

Answer 5

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

Question 6

Characteristics of Acute ITP

Answer 6

Seen in children following a viral infection and is a self-limited disorder

Question 7

Characteristics of Chronic ITP

Answer 7

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

Question 8

Characteristics of thrombotic thrombocytopenic purpura (TTP)

Answer 8

Rare disorder of hemostasis in which there is widespread intravascular formation of fibrin-platelet thrombi.

Question 9

What enzyme is deficient in TTP?

Answer 9

ADAMS13

Question 10

Characteristics of hemolytic uremic syndrome (HUS)

Answer 10

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

Question 11

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

Answer 11

Immune thrombocytopenia purpura (ITP)

Question 12

What is this diagnosis?

Decreased platelet count

Prolonged bleeding time

Normal PT and PTT

Blood smear shows thrombocytopenia, schistocytes, and reticulocytosis

Answer 12

Thrombotic thrombocytopenic purpura (TTP)

Question 13

Characteristics of Hemophilia A (Classic hemophilia)

Answer 13

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

Question 14

What is the diagnosis?

Normal platelet count

Normal bleeding time

Normal PT

Prolonged PTT

Answer 14

Hemophilia A

Question 15

Characteristics of Hemophilia B (christmas disease)

Answer 15

X-linked recessive condition resulting from a deficiency of factor IX that is clinically identical to hemophilia A

Question 16

Characteristics of Von Willebrand disease

Answer 16

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

Question 17

What is this diagnosis?

Normal platelet count

Prolonged bleeding time

Normal PT

Prolonged PTT

Abnormal platelet response to ristocetin

Answer 17

Von Willebrand disease

Question 18

Characteristics of disseminated intravascular coagulation (DIC)

Answer 18

Always secondary to another disorder

Causes widespread microthrombi with consumption of platelets and clotting factors, causing hemorrhage.

Question 19

What is the diagnosis?

Decreased platelet count

Prolonged PT/PTT

Decreased fibrinogen

Elevated fibrin split products (D dimers)

Answer 19

Disseminated intravascular coagulation (DIC)

Question 20

What is thrombosis?

Answer 20

Pathologic formation of an intravascular fibrin-platelet thrombus during life

Question 21

What is embolism?

Answer 21

Intavascular mass that has been carried down the bloodstream from its site of origin, resulting in the occlusion of a vessel