Lecture 3c Flashcards
Define hemophilia A.
Congenital deficiency of coagulation F8
Define hemophilia B.
Congenital deficiency of coagulation F9
What is the common symptom of hemophilia?
Recurrent hemarthoses(joint bleeding)
Easy bruising/bleeding
How does severe hemophilia present in infant males or early childhood?
Spontaneous bleeding into joints, soft tissue
Why is an associated negative development that could occur with hemophilia?
Develop inhibitors to the associated clotting factor
30% of pts in hemophilia A
<5% in hemophilia B
What is the normal range for F8?
50-150% (varies with age)
How do you treat hemophilia?
Plasma-derived or recombinant factor concentrates
Can be treated with prophylaxis
Severe hemophilia can come up to 3x a week
Can pts live a normal life with hemophilia?
Yes, if prophylactically treated/managed and educated
What is the MC cause of death in hemophilia pts?
Transfusion-obtained HIV/AIDs
Hepatitis/cirrhosis
What must hemophilic pts do to avoid complications?
Avoid contact sports
Monitor for S/S of bleeding
Home infusion technique
Managed by hematologist
What is the MC inherited bleeding disorder?
Von willebrand disease
How many types of von willebrand stages are there?
3
Type 1: quantitive(not enough)
Type 2: qualitative(dysfunctional)
Type 3: profound quantitive(near or total absence of VWF)
What is F11 deficiency also referred to as?
Hemophilia C
What does thrombocytopenia present as?
Mucous membrane related bleeding…
Epistaxis(nose bleed)
Gum bleeding
GI bleeds
Life-threatening cerebral hemorrhage
How can thrombocytopenia occur?
Increased destruction
Decreased production
What are examples that decrease production of platelets?
Congenital/Acquired bone marrow failure
Exposure to chemotherapy, irradiation
Marrow infiltration
Nutritional
What are examples of destructive/consumption thrombocytopenia?
How does splenomegaly/hypersplenism destroy platelets?
Aged platelets are normally destroyed by the spleen, but it is now unregulated
What is the etiology of immune thrombocytopenia purpura?
Pts form auto-AB against Ag on the platelet surface (causing destruction)
What lab do we order for suspicion of immune thrombocytopenia purpura?
CBC with peripheral blood smear
Is there a cure for ITP?
No
What are the treatments for ITP?
Corticosteroids(first line)
IVIG
Platelet transfusion(serious bleeds)
Splenectomy
What drugs are the common cause of acquired/iatrogenic PLT defects?
Aspirin
Clopidogrel
NSAIDs
What thrombotic microangiopathies (TMAs) are there?
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic-uremic syndrome (HUS)
How do you diagnose TTP?
CDC (should show elevated WBC)
Hg 8-9g/dL
PLT 20-50k
Peripheral smear (presence of schistocytes)
Coagulation studies, PT, aPTT are normal
D-dimer (normal-elevated)
Fibrinogen (high)
LDH (high)
Direct Coombs test negative
Hyperbilirubinemia
What is the most common cause of acute renal failure in children?
Hemolytic-uremic syndrome(HUS)
How does HUS present?
Prodromal gastroenteritis
Prodrome of fever, bloody diarrhea for 2-7 days before onset of renal failure
Irritability, lethargy
Seizures
Acute renal failure
Anuria
HTN
Edema/volume overload
Pallor
What bacteria is the most common cause of HUS in NA and Western Europe?
E. coli serotype 0157:H7
What disorders are associated with DIC?
Sepsis
Cancer, trauma, burns, or pregnancy-assocated morbidity
Aortic aneurysm or cavernous hemangiomas
Snake bites
