Miscellaneous

Question 1

AC trial at Medstar

Answer 1

RCT double blind study comparing ASA vs placebo for 3 years w/ end goal monitoring of decreased recurrence of breast cancer following standard chemotherapy regimens

Question 2

Palbociclib benefit for breast cancer

Answer 2

When combined w/ endocrine therapy, increased PFS

Question 3

Consideration of long term anastrazole for post chemotherapy breast cancer

Answer 3

If patient is post menopausal and had low grade disease, unlikely to have great benefit

If high grade disease or premenopausal, recommend continuation for 10 years

Question 4

Mondoor’s disease

Answer 4

Superficial thrombophlebitis of the veins of the anterior chest wall

Question 5

Capecitabine cycles for metastatic breast cancer

Answer 5

Oral regimen taken for 14 days followed by 7 day break

Total cycle length is 21 days

Question 6

Stauffer’s Syndrome

Answer 6

Signs of cholestasis unrelated to tumor infiltration of the liver that resolves after RCC resection

Question 7

Sarcoma that requires full body scanning

Answer 7

Myeloid lipoid sarcoma; requires full body CT, MRI spine, MRI brain

Question 8

Merkel Cell Cancer general concept for therapy

Answer 8

Carboplatin + Etopiside (like small cell lung cancer); additionally, they are now trying PD-1 inhibitor therapies

Question 9

Ravulizumab

Answer 9

Anti-C5 antibody used to atypical HUS, PNH in the US; associated with increased risk for meningococcal infections due to decrease formation of C5-9 complex

Question 10

Anticoagulant w/ the best bleeding profile

Answer 10

Eliquis

Question 11

Thrombin positively feedbacks what factors in the coagulation cascade?

Answer 11

II, V, XI, XIII

-Is partly why Factor XII, PK, and HMWK deficiencies don’t bleed since XI can still be activated

Question 12

Antithrombin inhibits what molecules?

Answer 12

Thrombin (duh), Factor IXa, and Xa; irreversibly binds

-Also factors XIa and XIIa to a small extent

Question 13

Protein C function

Answer 13

Cleaves cofactors Va and VIIIa to reduce their function; free protein S is a cofactor for activated protein C function

Question 14

What are some of our natural anticoagulant pathways?

Answer 14

Antithrombin
Protein C, S
Tissue Factor Pathway Inhibitor (TFPI)
Plasminogen

Question 15

Treatment for Factor VIII inhibitor

Answer 15

Activated Factor VII

Additionally, steroids + cyclophosphamide to eliminate the inhibitor

Question 16

Antiphospholipid antibodies will prolong with coagulation time?

Answer 16

PTT

Question 17

When is the PFA inaccurate?

Answer 17

When platelet count is <100 or if Hcrt is <30

Question 18

Causes of acquired vWD

Answer 18

MGUS - treatable w/ IVIG
Hypothyroidism - type I due to decreased production
MPNs - type II due to sticky platelets that don’t bind vWF
Valvular disorders (Heide’s)
LVADs
Wilm’s tumors

Question 19

Cause of acquired Factor X deficiency

Answer 19

Amyloidosis - actively removes Factor X from plasma; fix with replacement

Question 20

What can patient’s with a lupus inhibitor also have?

Answer 20

Anti Factor II antibody

Question 21

What lab can be increased with SS crisis?

Answer 21

AST; due to reserve in RBCs

Question 22

Bendamustine ADRs

Answer 22

Fever at night, increased Cr NOT DUE TO TLS, hair loss, nausea

Question 23

Rituxan infusion rate

Answer 23

It is slowly increased every 30minutes to ensure no reaction; typically, this won’t occur until the 2nd or 3rd increase

Question 24

Patient with a provoked DVT who still gets tested and has a positive hypercoagulable screen

Answer 24

Does not change management; repeat testing after 3 months of treatment for provoked DVT and test when off anticoagulation

Question 25

Labs to check for thrombophilia

Answer 25

``` LA ACL Anti-B2 glycoprotein FVL Prothombin G20201A Protein S Free s antigen Protein C Antithrombin III activity ```

Question 26

What to do with patient who is heterozygous for BOTH FVL and prothrombin mutations?

Answer 26

Anticoagulate; if positive for just one, don't

Question 27

Acquired hemophilia deficiencies

Answer 27

``` Acquired deficiency: • Liver disease (C, S, AT) • Warfarin therapy (C, S) • Estrogens, pregnancy (S) • Inflammatory diseases (S) • Heparin therapy (AT) • Acute thrombosis (S, AT) ```

Question 28

Bleeding disorder that is usually benign but most commonly presents as excessive intraoperative bleeding

Answer 28

Factor XI deficiency Diagnosis: Prolonged aPTT, low Factor XIa level Tx: Antifibrinolytics, FFP prior to surgery

Question 29

Factor VII deficiency

Answer 29

Mucocutaneous or surgical bleeding but rarely presents as thrombosis Tx with rVIIa

Question 30

Plasma dyscrasias can be associated with a deficiency of which coagulation factor?

Answer 30

Factor X Can also be AR inherited (1:500k) Levels are well associated with symptoms Tx: FX concentrate, PCC, FFP, treatment of plasma cell disorder

Question 31

Factor V deficiency

Answer 31

Has prolonged aPTT, PT, normal thrombin time Can treat with platelets because platelet granules contain factor V

Question 32

Bleeding from umbilical stump or sudden ICH

Answer 32

Factor XIII deficiency; AR genes coding for catalytic A subunit Can also present as poor wound healing or pregnancy loss Dx: Clot solubility test, Factor XIII assay, genetic testing

Question 33

Treatment for Factor XIII deficiency

Answer 33

Recombinant Factor XIIIa subunit OR pdFXIII if known to have B subunit mutation Can also use FFP if not available *****Give monthly prophylactic Factor XIII treatment in patients with FXIII level <10% to decrease risk of spontaneous ICH

Question 34

Prothrombin deficiency

Answer 34

Poor correlation with levels and clinical bleeds Dx: Same to factor V deficiencies; confirm with prothrombin activity assay, prothrombin antigen level, genetic testing ****Need to rule out acquired deficiency and check for antiprothrombin antibodies Tx: PCC q2-3days, pts only need prophylaxis with history of severe bleeding, prothrombin levels <10%

Question 35

Hypofibrinogenemia

Answer 35

``` Homozygous = No fribrinogen Heterozygous= Hypofibrinogenemia ``` Tx: Cryo, FFP, prophylaxis in only severe cases

Question 36

Dysfibrinogenemia

Answer 36

Actually associated w/ increase risk of thrombosis (20-25%)

Question 37

Second most common inheritable bleeding disorder

Answer 37

HHT; AD inherited 95% have severe recurrent mucocutaneous bleeding; many patients also have liver, lung, brain vascular malformations that are at increased risk for hemorrhage Tx: Iron infusions + Antifibrinolytics + Local ablative procedures + Antiangiogenic therapies (bevacizumab)