Term
| How do patient's present with polycythemia vera? |
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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. |
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Term
| Diagnostic labs for polycythemia vera? |
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Definition
Hct: >50%
RBC mass: Increased
Erythropoietin: normal
Basophilia (suggests proliferative myelopoiesis JAK-2) |
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Term
| Treatment for polycythemia vera? |
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Definition
Serial phlebotomy until Hct <45
Daily ASA
hydroxyurea for high risk of thrombosis
Anagrelide can reduce platelets if refractory to phlebotomy |
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Term
| What are the complications of polycythemia vera? |
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Definition
| Increased risk of conversion to other myeloproliferative syndromes or AML |
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Term
| How do you ID a platelet disorder vs. Coagulopathy? Labs for plt disorder? |
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Definition
Bleeding time: prolonged, excessive
Onset of bleeding: Immediate
Presentation: Superficial and mucosal bleeding (GI, gingival, nasal), petechiae, ecchymosis. |
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Term
| How do you ID a platelet disorder vs. Coagulopathy? Labs for coagulopathy? |
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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. |
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Term
| Initial labs to evaluate bleeding disorders? |
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Definition
| PT/PTT (bleeding time if normal PT/PTT), CBC, platelet count, D-dimer, fibrinogen, blood smear |
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Term
| Tmx for bleeding disorder? |
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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. |
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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? |
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Definition
HIT
Platelets are activated and thrombosis occurs. |
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Term
| Name common platelet disorders. |
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Definition
| TTP, HUS, HIT, Drug-induced thrombocytopenia, ITP, Acquired disease, Inherited disease, Platelet dysfunction |
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Term
| How to diagnosis platelet dysfunction? |
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Definition
Confirm or disprove thrombocytopenia
Order CBC, blood smear, one-hour post-transfusion platelet count
Bone marrow biopsy in case of severe thrombocytopenia |
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Term
| What does post-transfusion platelet count help you to distinguish? |
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Definition
Low plt production(pancytopenia, small platelets) - increased count after transfusion.
Platelet destruction (large platelets) - no significant increase in platelet count following transfusion. |
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Term
| What drugs can induce thrombocytopenia? |
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Definition
Quinine, sulfa drugs, glycoprotein IIb/IIIa inhibitors.
Resolves if drug is removed. |
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Term
| How do you determine ITP (autoimmune thrombocytopenia) and how do you treat? |
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Definition
Severe thrombocytopenia 2/2 platelet-associated IgG antibodies.
DIC panel is negative.
Give prednisone
If unresponsive, splenectomy |
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Term
| How do you diagnose and treat HIT? |
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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) |
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Term
| How do you diagnose and treat acquired platelet dysfunction and how do you treat? |
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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! |
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Term
| Give some examples of inherited thrombocytopenia and how to generally treat. |
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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! |
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Term
| How do you determine if bleeding is due to platelet dysfunciton? |
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Definition
| Platelet level is normal. |
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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? |
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Definition
Recombinant factor VIII
Rest
Ice
Analgesia (avoid NSAIDS and ASA) |
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Term
| What kind of exam and history suggests coagulopathy? |
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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. |
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Term
| If you suspect coagulopathy, what is the next step? |
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Definition
| Look for liver disease on exam and order LFTs to look for liver dysfunction. |
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Term
| What factors are involved in the intrinsic pathway? |
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Definition
VIII, IX, XI, and XII
aPTT: increased
Dx: Hemophilia A or B |
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Term
| What factors are involved in the extrinsic pathway? |
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Definition
VII
PT: Increased (INR)
Make sure patient isn't on WARFARIN! |
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Term
| What factors are involved in the common pathway? |
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Definition
V, X, and II(fibrinogen)
aPTT: Increased
PT: Increased |
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Term
| How do you differentiate between a common pathway coagulopathy and a fibrinolytic disorder? |
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Definition
Thrombin time: Abnormal in fibrinolytic do
Reptilase time: Abnl in fibrinolytic do
These are normal in common pathway coagulopathies. |
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Term
| What do labs look like if you suspect Factor VIII, IX, or XI deficiency? |
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Definition
aPTT: Elevated
PT: Normal
Heparin use is not present
Mixing study: aPTT normalizes |
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Term
| What do labs look like if you suspect Factor VIII, IX, or XI inhibitor is present? |
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Definition
aPTT: Elevated
PT: Normal
Heparin is not in use
Mixing Study: aPTT does NOT normalize (still elevated) |
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Term
| What do labs look like if you suspect Factor II, V, or X deficiency? |
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Definition
aPTT: Elevated
PT: Elevated
NO evidence of DIC, Liver dx, Vit K deficiency or Heparin use.
Order factor levels |
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Term
| What do labs look like if you suspect early DIC, Liver disease, warfarin use, or vitamin K deficiency? |
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Definition
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Term
| How do you treat coagulopathy with active bleeding? |
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Definition
FFP to replace deficient factors.
Remove Heparin or warfarin |
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Term
| How do you treat hemophilia A? |
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Definition
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Term
| How do you treat Hemophilia B? |
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Definition
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Term
| What is von Willebrand's Disease (vWD)? |
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Definition
Autosomal dominant
Most common bleeding disorder
Low levels of von Willebrand's factor |
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Term
| What are the symptoms of von Willebrand's Disease? |
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Definition
Can mimic platelet dysfunction (mucocutaneous bleds and increased bleeding time)
Can mimic hemophilia (joint bleeds, Inceased aPTT) |
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Term
| How do you diagnose von Willebrand's disease? |
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Definition
vWF antigen: decreased
vWF activity: Abnormal (ristocetin cofactor activity) |
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Term
| How do you treat von Willebrand's disease? |
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Definition
No prophylaxis needed
Before surgery or if bleeding: Desmopressin |
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