Misc

Question 1

normal PO2 + low pulse ox = ?

Answer 1

methemoglobinemia (O2 dissociation curve is shifted to the left)

Question 2

treatment of methemoglobinemia?

Answer 2

methylene blue, vitamin C

Question 3

Tx for WARM autoimmune hemolytic anemia

Answer 3

steroids, danazol, RITUXIMAB, splenectomy, immunosuppressive drugs

Question 4

Tx for COLD agglutinins

Answer 4

cyclophosphamides, chlorambucil --> Rituximab.
NO steroids (unlike WARM AIHA)

Question 5

Antigen and Ab involved in WARM AIHA

Answer 5

antigen = Rh;

IgG +/- C3

Question 6

Antigen and AB involved in COLD agglutinins

Answer 6

antigen = iAg; IgM, DEFINITELY C3

Question 7

how to diagnose Paroxysmal Nocturnal Hemoglobinuria

Answer 7

(DAF) assay/ Flow cytometry of CD55/59

Question 8

Treatment of PNH

Answer 8

Allogeneic bone marrow transplant/ ECULIZUMAB

Question 9

what to give 2 weeks prior to PNH treatment with eculizumab?

Answer 9

meningococcal vaccine

Question 10

diagnosis of acute intermittent porphyria

Answer 10

spot urine porphobilinogen (due to decreased PBG deaminase activity, will get a buildup of PBG)

Question 11

Hereditary spherocytosis is due to?

Answer 11

cytoskeletal membrane defect

Question 12

treatment of hereditary spherocytosis?

Answer 12

folate supplements –> splenectomy

Question 13

Re: prothrombin complex concentrates (PCCs), specifically, four-factor PCC, is a combination of?

Answer 13

inactivated factors II, VII, IX, and X.
In patients with INR elevation and bleeding associated with warfarin administration, urgent reversal of anticoagulation should be accomplished using vitamin K and prothrombin complex concentrates.

Question 14

Patients with a Pulmonary Embolism Severity Index score of less than 65 are at low risk of death and may be managed in the outpatient setting with a non–vitamin K antagonist oral anticoagulant, such as?

Answer 14

apixaban or rivaroxaban

Question 15

clinical features of polycythemia vera

Answer 15

1. EPO-independent (LOW EPO) proliferation of erythrocytes in the setting of erythrocytosis
2. Hgb > 16.5 g/dL in men and >16 in women after secondary causes are excluded

Question 16

how to diagnose polycythemia vera

Answer 16

JAK2 mutation

Question 17

treatment of polycythemia vera

Answer 17

therapeutic phlebotomy (lower hct to <45%), hydroxyurea for patients at high risk for thrombosis (>60 yo, previous thrombosis, leukocytosis), LOW dose ASA (high dose can cause increased bleeding)

Question 18

hepatic vein thrombosis (Budd-Chiari syndrome) or portal vein thrombosis should prompt consideration of?

Answer 18

Polycythemia vera –> test for JAK2 mutation or

Paroxysmal nocturnal hemoglobinuria –> screening flow cytometry CD55 and CD59 ABSENT

Question 19

Patients with pancytopenia, macrocytic erythrocytes, hypersegmented neutrophils, and findings consistent with intramedullary hemolysis should be checked for?

Answer 19

vitamin B12 and folate levels to determine the cause of megaloblastic anemia

Question 20

Tx in essential thrombocythemia for:

• low risk (< 60 YO, no previous thrombosis, leukocyte count <11k)
• high risk
• life threatening

Answer 20

• low risk: low dose ASA
• high risk, non pregnant: hydroxyurea + ASA
• life-threatening (TIA, stroke, MI, GI bleed): Plateletpheresis
  aspirin and hydroxyurea
• ET is the most common myeloproliferative neoplasm