AWESOME HEME2

Question 1

The finding on peripheral smear associated with uremia is _________

Answer 1

Echinocyte/Burr cell

Question 2

BRAFV600E assoc with what 3-4 Dx?

Answer 2

Prob some that I am missing but in Hairy Cell Leukemia, Melanoma, and Histiocyte Disorders–Langerhans and Erdheim-Chester; recall it is Ras-Raf-Mek-Erk pathway

Question 3

What is the best perioperative mgmt of a patient with hx of HIT who needs perioperative heparin for an elective surgery?

Answer 3

Delay surgery; if the PF4 abs are negative then ok to give during surgery followed by fondaparinux. Of note if important surgery people have done perioperative IVIG/PLEX

Question 4

Dx for pt undergoing complicated CABG s/p xfusion of 12 units pRBC now w/ oozing, normal PT/PTT and platelets of 30 k

Answer 4

Likely dilutional thrombocytopenia; no dilutional coagulopathy as PT/PTT normal

Question 5

Regarding giving TPA what should you do with giving with antiplatelets and anticoagulation?

Answer 5

Ok to give TPA with antiplatelet but not if on anticoagulation that is a contraindication

Question 6

The platelet disorder with abnormalities of Gp IIb/IIIa is _________

Answer 6

Glanzmanns Thrombasthenia

Question 7

What should you do for an incidentally noted elevated PTT that does not correct with 1:1 mixing study but does correct with addition of phospholipids but with no clot? With clot?

Answer 7

Nothing; then pt has APS and should start heparin then warfarin w/ goal 2-3 (if recurrent clot then make goal 3-4)

Question 8

The platelet disorder with abnormalities of Gp1b/IX is _______

Answer 8

Bernard Soulier

Question 9

In Essential Thrombocytosis you need to perform which test prior to starting aspirin when platelets are over 1 million?

Answer 9

Ristocetin Cofactor assay to ensure that the patient does not have an acquired VWD

Question 10

Which disorder is often associated with a rather high PTT without any clinical history of bleeding?

Answer 10

Factor XII deficiency (Hageman Factor–I think)

Question 11

In a patient scheduled for splenectomy what vaccines need to be given prior to splenectomy?

Answer 11

PCV13 and influenza then followed by PPSV23 8 weeks later; Haemophilus influenzae vaccine, and meningococcal vaccine (note meningococcus = droplet precautions, also need vaccine prior to eculizumab)

Question 12

A patient presents with mucus membrane bleeding with total protein of 9.5 and gamma gap of 6, calcium 12.5 BUN is 80 w/ sCr of 4.5 and platelet count of 95k with normal PT/PTT. What is the most likely etiology?

Answer 12

Renal Failure secondary to MM with uremic platelet dysfunction

Question 13

A patient is diagnosed with DVT and started on heparin and two days later the patient is hypotensive with sodium of 124 and K of 5.6. Dx?

Answer 13

Bilateral Adrenal Hemorrhage secondary to heparin; note that this can also occur in meningococcemia with Waterhouse-Freidreichson syndrome

Question 14

When should you measure protein C after AC for a clot?

Answer 14

When patient no longer on AC and off of it for at least 2 weeks

Question 15

All platelet disorders will cause an elevation of this ________

Answer 15

Bleeding Time (positive ristocetin in VWF, abnormal platelet aggregation in Glanzmanns, giant platelets in Bernard Soulier)

Question 16

Dx for 1) Elevated bleeding time, normal PTT and positive ristocetin cofactor assay 2) Elevated BT, normal PTT, and giant platelets 3) increased BT, nl PTT, nl platelets but abnormal platelet aggregation

Answer 16

1) von Willebrand Disease 2) Bernard Soulier 3) Glanzmanns Thrombasthenia

Question 17

Dx for 1) a patient with elevated PTT and bleeding corrects with 1:1 mixing study 2) pt with elevated PTT and bleeding does NOT correct 3) pt with elevated PTT and thrombosis does NOT correct

Answer 17

1) Factor Deficiency 2) Acquired Hemophilia 3) Antiphospholipid Syndrome

Question 18

50 M with sudden onset of fever with leukocytosis and erythematous plaques and nodules on face with Bx showing dense neutrophilic infiltrate. Dx. Tx?

Answer 18

Sweet’s Syndrome–would give steroids but also need to perform BM Bx to r/o underlying leukemia

Question 19

A patient with GIB and normal coags receives 8 units pRBCs now with rebleeding and elevated PT/PTT. Dx? WTD?

Answer 19

Dilutional Coagulopathy (Plasma Washout)–give FFP

Question 20

What is the best agent to use for a patient with known HIT (platelet factor 4 abs positive) and requires urgent surgery what is the best AC?

Answer 20

Bivalrudin > Argatroban because lower half life

Question 21

What are some issues with giving high dose IVIG for hypogammaglobulinemia (i.e. in CLL or CVID)?

Answer 21

Can cause hemolytic anemia and can also cause DVT/Stroke/MI

Question 22

The finding on peripheral smear assoc with G6PD is _______

Answer 22

Blister cells and bite cells

Question 23

Best mgmt for pt with ischemic stroke who is taking apixaban?

Answer 23

Thromboembolectomy as TPA contraindicated (ok if on antiplatelets)

Question 24

When can you do an LP in a patient who received ppx dose lovnenox vs. therapeutic dose?

Answer 24

If only prophylactic then can do 12 hours later; if therapeutic then can do 24 hours later; after LP, can restart 4 hours later

Question 25

What type of AIHA is often caused by CLL?

Answer 25

Warm AIHA w/ IgG + and C3 +/-; lots of other lymphomas cause colid agglutinin disease

Question 26

Leukoreduced cells will aid in the prevention of which transfusion reaction?

Answer 26

Febrile Nonhemolytic

Question 27

What have knee high compression stockings shown regarding post-phlebitis syndrome?

Answer 27

No change

Question 28

Which other cell line may be affected after treatment for megaloblastic anemia?

Answer 28

Platelets. Tx for folate def anemia may lead to significant reactive thrombocytosis

Question 29

The finding on peripheral smear assoc with liver disease and alcohol is __________

Answer 29

Acanthocyte/Spurr Cell

Question 30

What is a renal issue that may be encountered on UA with TKIs

Answer 30

Phosphaturia

Question 31

For a patient who needs CABG and has hx of HIT with current negative PF4 ab titers what is the best mgmt for perioperative a/c

Answer 31

Can give IV heparin during surgery followed by SQ fondaparinux post-operatively (Recall that fondaparinux also good in hyperkalemia)

Question 32

Which drugs will cause abnormal platelet aggregation similar to Glanzmanns disease?

Answer 32

GP IIB/IIIa inhibitors such as abciximab (Reopro), Eptifibatide (Integrillin), and Tirofiban (Aggrastat)–of note Reopro can also cause thrombocytopenia

Question 33

What is the Dx for a 25 F with hx of 3 vaginal births who presented one week ago for severe menorrhagia with anemia but normal platelets s/p several units of pRBCs now presenting with purpura and thrombocytopenia? Tx?

Answer 33

Post-Transfusion Purpura (due to platelet alloimmunization)–Tx is with IVIG (theoretically can check HPA-1a abs)

Question 34

Regarding HIT how much would you expect the platelet count to drop and when? What if the platelets start to drop >14 days after heparin exposure?

Answer 34

Expect a 50% drop in 5-10 days but if >14 days later unlikely to be HIT

Question 35

The PT/PTT in primary hemostasis disorders is ___________

Answer 35

Normal as this is a platelet plug formation issue (secondary hemostatic issues will have elevated PT/PTT depending)

Question 36

Aspirin is used in adjunctive therapy of dissection of this vessel

Answer 36

Internal Carotid Artery Dissection–prevents in situ thrombosis

Question 37

Regarding the presence of issues with primary hemostasis vs. issues with secondary hemostasis, when does bleeding tend to occur temporally?

Answer 37

Bleeding occurs immediately in issues with primary hemostasis and is mostly mucocutaneous (platelet issue) whereas secondary hemostasis is delayed and involves joint bleeds and hematomas

Question 38

The finding on peripheral smear associated with splenectomy or functional asplenia is __________

Answer 38

Howell-Jolly Body

Question 39

A 40 y/o F with hx of mildly elevated aPTT develops heavy menses after starting aspirin? Dx?

Answer 39

Von Willebrand Disease

Question 40

Which often has HTN and elevated JVP–TACO or TRALI?

Answer 40

TACO because of the elevated oncotic pressure; next best step for all xfusion rxns is to stop the infusion; for future can slow infusion rate

Question 41

A patient in her third trimester with platelets of 85k. Dx? Next steps?

Answer 41

Gestational Thrombocytopenia (ok as long as >70k), continue to monitor CBC or plt count q1-2w

Question 42

A patient on isoniazid with abnormal bleeding but with normal PT and PTT likely has what

Answer 42

Factor XIII deficiency, may have abnromal clot retraction test