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First Aid Step 3 Hematology 4
Polycythemia and Bleeding Disorders
36
Medical
Professional
12/25/2013

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Term
How do patient's present with polycythemia vera?
Definition
Malaise
Fever
Pruritus (esp after shower)
vascular sludging (eg. stroke, angina, MI, claudication, hepatic vein thrombosis, headache and blurred vision)
large and many retinal veins on funduscopy and splenomegaly.
Term
Diagnostic labs for polycythemia vera?
Definition
Hct: >50%
RBC mass: Increased
Erythropoietin: normal
Basophilia (suggests proliferative myelopoiesis JAK-2)
Term
Treatment for polycythemia vera?
Definition
Serial phlebotomy until Hct <45
Daily ASA
hydroxyurea for high risk of thrombosis
Anagrelide can reduce platelets if refractory to phlebotomy
Term
What are the complications of polycythemia vera?
Definition
Increased risk of conversion to other myeloproliferative syndromes or AML
Term
How do you ID a platelet disorder vs. Coagulopathy? Labs for plt disorder?
Definition
Bleeding time: prolonged, excessive
Onset of bleeding: Immediate
Presentation: Superficial and mucosal bleeding (GI, gingival, nasal), petechiae, ecchymosis.
Term
How do you ID a platelet disorder vs. Coagulopathy? Labs for coagulopathy?
Definition
Bleeding time: Normal to slightly increased
onset of bleeding: Delayed after surgery, spontaneous into joints
Presentation: deep and excessive bleeding into joints, muscles, GI tract and GU tract.
Term
Initial labs to evaluate bleeding disorders?
Definition
PT/PTT (bleeding time if normal PT/PTT), CBC, platelet count, D-dimer, fibrinogen, blood smear
Term
Tmx for bleeding disorder?
Definition
If unstable, give IV fluids and find the source of the bleed.
Transfuse with RBC if Hb<8
Transfuse FFP to normalize PTT and PT
Transfuse platelets as needed.
Term
64 yo woman in iCU after cardiac surgery HD6 has "black" rash. On exam there are necrotic patches on distal extremities.Meds include furosemide, enoxaparin, enalapril, amlodipine, insulin, and ASA. Plts are 36,000, +PF-4ab. What is dx and mechanism of necrosis?
Definition
HIT
Platelets are activated and thrombosis occurs.
Term
Name common platelet disorders.
Definition
TTP, HUS, HIT, Drug-induced thrombocytopenia, ITP, Acquired disease, Inherited disease, Platelet dysfunction
Term
How to diagnosis platelet dysfunction?
Definition
Confirm or disprove thrombocytopenia
Order CBC, blood smear, one-hour post-transfusion platelet count
Bone marrow biopsy in case of severe thrombocytopenia
Term
What does post-transfusion platelet count help you to distinguish?
Definition
Low plt production(pancytopenia, small platelets) - increased count after transfusion.
Platelet destruction (large platelets) - no significant increase in platelet count following transfusion.
Term
What drugs can induce thrombocytopenia?
Definition
Quinine, sulfa drugs, glycoprotein IIb/IIIa inhibitors.
Resolves if drug is removed.
Term
How do you determine ITP (autoimmune thrombocytopenia) and how do you treat?
Definition
Severe thrombocytopenia 2/2 platelet-associated IgG antibodies.
DIC panel is negative.
Give prednisone
If unresponsive, splenectomy
Term
How do you diagnose and treat HIT?
Definition
Occurs 4-14 days post heparin introduction
Platelet factor-4 (PF-4) antibodies +
Stop heparin
Start alternative like lepirudin, argatroban, or danaparoid sodium
DO NOT USE WARFARIN! (may exacerbate HIT)
Term
How do you diagnose and treat acquired platelet dysfunction and how do you treat?
Definition
Severe liver disease, severe renal disease, or multiple myeloma.
Treat with desmopressin
Give OCP for menorrhagia
Give FFP or cryoprecipitate for major bleed
DO NOT GIVE ASA!
Term
Give some examples of inherited thrombocytopenia and how to generally treat.
Definition
Bernard-Soulier syndrome, Glanzmann's thrombasthenia, and storage pool disease.
Treat with desmopressin, OCP for menorrhagia and FFP for major bleeding DO NOT USE ASA!
Term
How do you determine if bleeding is due to platelet dysfunciton?
Definition
Platelet level is normal.
Term
7 yo boy with hemophilia A comes to ER after falling on knee two hours ago. Knee is rad, warm, and held in partial felexion due to an effusion. What medication do you give?
Definition
Recombinant factor VIII
Rest
Ice
Analgesia (avoid NSAIDS and ASA)
Term
What kind of exam and history suggests coagulopathy?
Definition
Recurrent spontaneous bleeding
Delayed bleeding after trauma or surgery (classic is umbilical cord falling off)
Rule out medications and vit K malabsorption (warfarin, enoxaparin, heparin, abx use, alcoholism, malnutrition.
Term
If you suspect coagulopathy, what is the next step?
Definition
Look for liver disease on exam and order LFTs to look for liver dysfunction.
Term
What factors are involved in the intrinsic pathway?
Definition
VIII, IX, XI, and XII
aPTT: increased
Dx: Hemophilia A or B
Term
What factors are involved in the extrinsic pathway?
Definition
VII
PT: Increased (INR)
Make sure patient isn't on WARFARIN!
Term
What factors are involved in the common pathway?
Definition
V, X, and II(fibrinogen)
aPTT: Increased
PT: Increased
Term
How do you differentiate between a common pathway coagulopathy and a fibrinolytic disorder?
Definition
Thrombin time: Abnormal in fibrinolytic do
Reptilase time: Abnl in fibrinolytic do
These are normal in common pathway coagulopathies.
Term
What do labs look like if you suspect Factor VIII, IX, or XI deficiency?
Definition
aPTT: Elevated
PT: Normal
Heparin use is not present
Mixing study: aPTT normalizes
Term
What do labs look like if you suspect Factor VIII, IX, or XI inhibitor is present?
Definition
aPTT: Elevated
PT: Normal
Heparin is not in use
Mixing Study: aPTT does NOT normalize (still elevated)
Term
What do labs look like if you suspect Factor II, V, or X deficiency?
Definition
aPTT: Elevated
PT: Elevated
NO evidence of DIC, Liver dx, Vit K deficiency or Heparin use.
Order factor levels
Term
What do labs look like if you suspect early DIC, Liver disease, warfarin use, or vitamin K deficiency?
Definition
aPTT: Normal
PT: Elevated
Term
How do you treat coagulopathy with active bleeding?
Definition
FFP to replace deficient factors.
Remove Heparin or warfarin
Term
How do you treat hemophilia A?
Definition
Give Factor VIII
Term
How do you treat Hemophilia B?
Definition
Give Factor IX
Term
What is von Willebrand's Disease (vWD)?
Definition
Autosomal dominant
Most common bleeding disorder
Low levels of von Willebrand's factor
Term
What are the symptoms of von Willebrand's Disease?
Definition
Can mimic platelet dysfunction (mucocutaneous bleds and increased bleeding time)
Can mimic hemophilia (joint bleeds, Inceased aPTT)
Term
How do you diagnose von Willebrand's disease?
Definition
vWF antigen: decreased
vWF activity: Abnormal (ristocetin cofactor activity)
Term
How do you treat von Willebrand's disease?
Definition
No prophylaxis needed
Before surgery or if bleeding: Desmopressin
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