Flashcards in Hubbard Deck (63):
Distinction between TTP and DIC
PT/PTT in TTP are normal early in the disease, but later tests suggest DIC
Treatment for TTP - 100% life saving
"pseudohemophilia" with mucocutaneous bleeding
Platelet aggregation test (vWF)
Osler Weber Rendeu Syndrome is defined as __ and also called ___
Also called: Hereditary Hemorrhagic Telangiectasia
-Caused by thinning of the vessel walls causing telangiectatic formations and AV malformations
Fever + neuro + thromboCP + renal + microangiopathic hemolytic anemia = ?
Thrombotic Thrombocytopenia Purpura
Hemolytic Uremic Syndrome
Define microagiopathic anemia (MAHA) and example diseases (extravascular HA)
RBCs damaged when they pass through obstructed or narrowed vessels.
-DIC, TTP/HUS, SLE, malignant HTN
-PBS = **schistocytes
Define macroagiopathic anemia and example diseases (extravascular HA).
Prosthetic heart valves and Aortic Stenosis may cause HA secondary to MECHANICAL destruction of RBCs.
Examples of infections that increase RBC destruction (extravascular HA).
Pathogenesis of Thrombotic Thrombocytopenia Purpura
- Hereditary -- ADAMTS13 gene mutation
- Acquired – autoantibodies at ADAMTS13
- “Von Willebrand factor-cleaving protease” (degrades vWF) = ADAMTS13.
-ADAMTS13 inhibition = decreased vWF degredation = increased platelet aggregation and thrombosis
Treat Hemophilia A with
Treat Hemophilia B with
CD#;ch# in hereditary hemorrhagic Telangiectasia.
Notable location of the hemorrhages.
CD105, ch9 --> codes for **ENDOGLIN**
-Hemorrhages on the vermilion border of lip and tongue
What is the most common cause of hypercoagulabilty?
Initiation of warfarin therapy because PRotein C and S are depleted prior to other factors, resulting in increased coagulablity.
Antiphospholipid syndrome is an anti/pro coagulant?
What do you use to diagnose.
***What condition is present in 50% of cases, but is NOT necessary to diagnose?
Tx with what?
-Use DRVVT (diluted Russell Viper Venom Test) or PTT test
****NOT NECESSARY to have connective tissue disease to have this.
Presence of what two factors increase risk of DVT or PT by 35-fold?
OCP + heterozygous Factor5 Leiden = synergistic relationship
Pts with vWD show up first with what symptom?
Treatment of vWD - does what? 1) cryoprecipitate 2) DDAVP
1. Cryoprecipitate - replaces vWF
2. DDAVP - causes release of vWF from endothelium
Clinical features of ____?
-Easy bleeding & bruisability
-Hematomas from bleeding into soft tissues and muscles
-Hemarthroses – frequent, many patients have 1 or 2 target joints which have recurrent bleeds
-Patients are significantly inc risk for bleeding during and after surgery
Treatment of Hemophilia A
- Factor replacement for bleeds, surgical prophylaxis
- Recombinant factor replacements – hepatitis and AIDS less of a problem
- Factor VIII inhibitors – antibodies to factor VIII that may complicate care
What deficiency can occur in patients taking warfarin, on chornic antibiotics, rat poison ingestion, or severe liver disease?
Vit K deficeincy
Characteristics of ____
o Prolonged PT
o Def of factors II, VII, IX, X, Protein C & S
o Anticoagulants = C & S
Vit K deficiency
Treatment for Vit K deficiency. Use which when?
- Vit K replacement (intact liver)
- FFP (used when liver dz or acute hemorrhage)
What is the only endothelial syndrome associated with hemostatic complications?
Hereditary Hemorrhagic Telangectasia
Clinical presentation for...
o Minimal to early death from recurrent pulmonary emboli
o Chronic leg ulcers, deep vein thrombosis, recurrent LE thrombophlebitis
o DVT inc in pregnancy
Antithrombin 3 deficiency.
tendency to clot more/hypercoagulable
Antithrombin 3 deficiency treatment
-Prophylactic treatment with anticoagulants
-Patients with DVT should receive heparin but in higher doses
-AT-III replacement therapy, if don’t respond to heparin
Action of protein C
inactivates Factors 5 and 8
Use what in Protein C deficiency to decrease risk of thromboembolic disease?
What is antithrombin 3? What amplifies it?
serine protease that inhibits activation of thrombin.
-Heparin amplifies activity
-In absence of antithrombin3, hypercoagulablity
Most common cause of hypercoagulable state from deficiency in Protein C or S
initiation of warfarin therapy
Factor 5 Leiden is what classification of disease?
Treatment of Factor 5 Leiden (Defect)
-No prior episodes v. prior episodes
o No prior episodes: monitor; DVT prophylaxis and risk reduction
o Prior episodes: add lifelong anticoagulation
Three test used to diagnose antiphospholipid Syndrome
1. Prolonged phospholipid dependent coagulation test (PTT)
2. Lack of correction in mixing studies using normal plasma
3. Neutralization of inhibitor with excess phospholipid
What is the most common preventable cause of death?
What is Virchow's Triad?
vessel wall damage, blood coaguability, altered blood flow
What is this?
- Body makes antibodies to virus
- Antigens on virus look like glycoproteins on platelets
- Now platelets are circulation with antibodies on it platelets getting destroyed
- Spleen should be big
Acute Immune Thrombocytopenic Purpura
Management of Acute ITP
o PREDNISONE 1mg/kg/day
o Begin tapering does after a week
o If this doesn’t work immune globulin
o If person is a type one diabetic – sugar will be bouncing off the wall if on prednisone, so - 1)Put him on IV IG Or 2) give SPLENECTOMY (can be partial)
What is this?
Dolle bodies, increased toxic granulations, no schistocytes, PT/PTT abnormal
Management of DIC
o Treat underlying cause
o Until that’s fixed – fresh frozen plasma, cryoprecipitate
o Platelet transfusion
o Red cell count drops then transfuse red cells
What is this?
*****White/painful fingers + extremely high platelets (1.2M) =
also, possibly blurred vision
Treatment of erythromyalgia
What is this?
- causes hypercalcemia
- suppresses levels of uninvolved immunoglboins
- they have weird infection problems b/c no antibodies around, just ONE antibody immunoglobin that is involved in one major way
- myeloma cells produce osteoclast activating factors that causes elevated calcemia
- causes suppression of osteoblastic activity lytic bone lesions lots of free calcium
****What test MUST you do when you suspect Multiple myeloma?
Skeletal survey, NOT A BONE SCAN
Also, se M spike
***treatment of multiple myeloma
thalidomide or linolidamide (angiogenesis inhibitor)
Using bisphosmate in MM patients can cause what?
Ducher + congo red =
Amyloidosis (seen in pts with plasma cell dyscrasia)
heavy chain disease + truncated alpha chains = ?
(Presents with mesenteric adenopathy, diarrhea/malabsorption probelms, etc.)
Treat this with...?
alpha heavy chain disease
treated with ANTIMICROBIALS, NOT chemo
Indicative of __?
• Chromosome 5,7
• Trisomy 8
• Inversion 1
Treat 5q-Syndrome with what?
"Moth eaten appearance"
80yoM presents with sausage linking of the veins in eye. Electrophoresis shows gamma peak.
Waldenstrom (hyperviscosity), LPL
Parvo and thymomas can cause what?
pure red cell aplasia
*******treatment for aplastic anemia
********cyclosporin and anti-thymocyte globulin
Maltese cross = ? and can cause ___
babesiosis = HA
A pt has high risk MDS. The only curative modality for this is?
Pt has severe aplastic anemia - curative modality?
allogenic stem cell transplantation
MDS presents with?
MPD presents with?
MDS - cytopenia, infection
MPS - anemia, SM, B symptoms
hydroxyuria does what?
Increases HbF - use in sickle cell
When are increases in HbF beneficial in Sickle cell anemia?
- painful episodes, Acute Chest Syndrome, Osteonecrosis, Leg ulcers
Heavy chain disease - look for what in plasma cells?
Type of Heavy chain disease that can be aggressive or asymptomatic, almost always associated with anemia, HSM, LAD
Type of Heavy chain disease that can be MC type, MCly involving GI tract
Type of Heavy chain disease that can be Bence Jones proteinuria, hypogammalobulinemia
Tx for sideroblastic anemia
paradoxine or alcohol cessation