Bleeding Disorders

Question 1

defect in platelet plug formation (adhesion)

Most common inherited bleeding disorder

Answer 1

von Willebrand disease

you’ll see an increased BT

AD

treat with desmopressin (DDAVP)

Question 2

GpIb defect

Answer 2

Bernard-Soulier syndrome

AR

low platelet count high BT

large platelets

defect in platelet to vWF adhesion

Diagnose with (-) ristocetin

Question 3

Defect in GpIIb/IIIa

Answer 3

lanzmann thrombasthenia

AR

(+) ristocetin= abnormal

no platelet clumping

low PT high BT

Question 4

thrombocytopenia

hemolytic anemia

acute renal failure

Answer 4

Hemolytic-Uremic syndrome

acute onset

associated w/ EHEC O157:H7

other symptoms include abdominal pain, vomiting, bloody diarrhea

Question 5

Anti- GpIIb/IIIa antibodies

Answer 5

Immune thrombocytopenia

low PC hight BT

in kids this is after a viral infection

large megakaryocytes on bone marrow biopsy

Treatment: steroids, IVIG, splenectomy

Question 6

deficiency of ADAMTS13

Answer 6

Thrombotic thrombocytopenic purpura

decreased degradation of vWF multimers leading to increased platelet adhesion → increased platelet aggregation and thrombosis

low PC high BT

labs: schistocytes, increased LDH

pentad of symptoms: “Nasty Fever Torched His Kidney”

neuroologic, fever, thrombocytopenia, hemolytic anemia, kidney failure

Question 7

What are the causes of DIC?

Answer 7

STOP Making New Thrombi

Sepsis

Trauma

Obstetric complications

Pancreatitis (acute)

Malignancy

Nephrotic Syndrome (studies are inconclusive about this one- but I kept it in the list)

Transfusion