Hubbard Flashcards
TTP - treatment
Plasmapheresis
vWD presents as ____
Mucocutaneous bleeding
Treatment for vWD
Cryoprecipitate (vWF) + DDAVP (release vWF)
Vitamin K deficiency - labs
Prolonged PT, deficient factors 2, 7, 9, 10, C, S
Treatment for vitamin K deficiency
Normal liver fxn – vitamin K supplements
Liver disease or acute hemorrhage – FFP
Hereditary hemorrhagic telangiectasia - gene deficiency
Endoglin (CD 105)
Function of antithrombin 3
Serine protease - inhibits thrombin activation
AT3 requires what?
Heparin
AT3 deficiency causes increased risk of _____
Clotting (via thrombin activation)
AT3 deficiency - presentation
Recurrent LE thrombophlebitis, DVT, leg ulcers, PE
AT3 deficiency - treatment
Refractory?
Anticoagulants (heparin)
AT3 replacement
Protein C/S deficiency - treatment
Warfarin
Factor V Leiden - risk?
Treatment?
Thromboembolism
No episodes = monitor, etc.
Prior episode(s) = lifelong anticoagulation
Prothrombin 20210
Risk?
G-A mutation, causing increased prothrombin activity
Thrombosis
Common occurrences w/ antiphospholipid syndrome
- Thromboembolism
- Miscarriage
- Thrombocytopenia
- Cerebral ischemia/stroke
- UBOs on MRI
Diagnosis of antiphospholipid syndrome (3)
- Prolonged PTT
- No correction using normal plasma
- Neutralization w/ excess phospholipid
Specific diagnostic test for antiphospholipid syndrome
Dilute Russell viper venom time (DRVVT)
Treatment for antiphospholipid syndrome
Hx of thromboembolism –> lifelong anticoagulation
ITP - treatment
Prednisone
Reactive leukocytosis, thrombocytopenia, prolonged PT and PTT, no RBC fragments on smear
How to diagnose?
DIC - from infection
Dx = D-dimer level
DIC treatment
Treat the cause, FFP, cryoprecipitate, platelet transfusions
Hyaline thrombi that occlude the capillaries of basically every organ
TTP
Patient on prolonged antibiotics, PT starts to get longer
Vitamin K deficiency - no microbial production
Connective tissue disease, prolonged PTT that doesn’t correct, valvular heart disease, recurrent stroke, thrombocytopenia
Antiphospholipid syndrome
Superficial venous thrombosis - risk?
DVT, PE
Oral contraceptives - risk (when combined w/ other disorders)?
Thrombosis
Erythromelalgia - treatment
Aspirin
SPEP gamma spike w/ hump after
Treatment?
MGUS
NO treatment, just monitor
Solitary plasmacytoma - treatment
Local radiation
Multiple myeloma - diagnosis
What not to do?
Skeletal survey
NOT A BONE SCAN
Multiple myeloma - treatment
Thalidomide/lenolidamide + dexamethasone
Sausage-linking of fundoscopic veins
Waldenstrom’s hyperviscosity
Waldenstrom’s - treatment
Plasmapheresis, hydration, bisphosphonate
Fat pad = green birefringence on congo red
Amyloidosis
Joint pain, infections, anemia, bleeding, pedal edema
Multinucleate plasma cells w/ Dutcher bodies in BM
Amyloidosis (AL - light chain)
Temporal muscle wasting, chronic diarrhea, pre-renal disease, malabsorption
Treatment?
Alpha heavy chain disease
Antibiotics
Aplastic anemia - treatment
Cyclosporin + anti-thymocyte globulin
Diffuse rash, hemolytic anemia, elevated ESR, Gimsa stain
Classic finding?
Babesiosis
Maltese cross
Oral contraceptives + Factor V Leiden
35x increased risk of hypercoaguability
Hydroxyurea
Increased HbF in sickle cell
Acute chest syndrome - treatment
Transfusion
Treatment for PCV and ET
IFN-alpha, phlebotomy
Black water fever
RBC burst into blood (malaria complication) - hemoglobinuria and bilirubinuria (direct and indirect)
Neuropathy, HSM, endocrine issue, M spike, skin and skeletal lesions
POEMS syndrome - osteosclerotic myeloma
Male, eczema, thrombocytopenia, bloody diarrhea, recurrent infections
Wiskott-Aldrich (congenital thrombocytopenia)
