Heme/Onc

Question 1

Dense granules vs alpha granules if platelets ?

Answer 1

dense = ADP, calcium;
alpha = vWF, fibrinogen

Question 2

Decreased FC receptor in immature neutrophils?

Answer 2

Decreased CD16

Question 3

Eosinophils produce?

Answer 3

Histaminase and arylsulfatase (helps limit reaction following mast cell degranulation).

Question 4

Kallikrein stimulates ?

Answer 4

HMWK to Bradykinin

Question 5

activated protein C (w/ S) cleaves and inactivates ?

Answer 5

favctors Va, Vllla

Question 6

antithrombin inhibits activated forms of factors ?

Answer 6

12, 11, 7, 9, 10, 2

Question 7

“activation” phase of platelet plug formation?

Answer 7

ADP binding to R induces Gpllb/llla expression at platelet surface

Question 8

dec ESR?

Answer 8

Microcytosis, hypofibrinogenemia

Question 9

Acanthocytes (spur cells) seen in ?

Answer 9

Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)

Question 10

Howell-Jolly bodies seen in ?

Answer 10

pts mothball ingestion (naphthalene)

Question 11

ferroportin ?

Answer 11

Basolateral iron transporter of enterocyte to blood

Question 12

alpha thalassemia - Hb Barts vs HbH ?

Answer 12

gamma4 (4 gene deletion) //

beta3 (3 gene deletion)

Question 13

beta thalassema - mild anemia mech ?

Severe anemia mech ?

Answer 13

DNA splicing defect //

stop codon

Question 14

dx of beta-thalassemia minor confirmed by?

Answer 14

inc HbA2 (> 3.5%) ; dec HbA

Question 15

tx of Beta-thalassemia major?

Answer 15

long term blood transfusion

Question 16

Sideroblastic anemia causes changes in lvls ?

Answer 16

inc iron, normal TIBC, inc ferritin

Question 17

hemosiderin ?

Answer 17

iron carried by transferrin in circulation –> binds to apoferritin in cells

Question 18

tx of orotic aciduria?

Answer 18

Uridine monophosphate

Question 19

nonmegaloblastic macrocytic anemia?

Answer 19

liver disease; alcoholism; reticulocytosis; drugs (5-FU, AZT, hydroxyurea) //
macrocytes are round not oval and hypersegmental neutros not present

Question 20

Intravascular hemolysis causes changes in ?

Answer 20

dec haptoglobin, inc LDH

Question 21

Extravascular hemolysis causes changes in ?

Answer 21

inc UCB, inc LDH

Question 22

inc calcium bilirubinate gallstones (black) in which intrinsic hemolytic normocytic anema (extravasc) ?

Answer 22

hereditary spherocytosis

Question 23

paroxysmal nocturnal hemoglobinuria triad, lab findings, tx ?

Answer 23

Hemolytic anemia, pancytopenia, and venous thrombosis //
CD55/59 neg RBCs //
tx eculizumab.

Question 24

Most common cause of death in sickle cell anemia?

Answer 24

Strep pneumo sepsis

Question 25

Sickle cell anemia affects kidney how ?

Answer 25

Renal papillary necrosis (due to low O2 in papilla)

Question 26

transferrin production affected by pregnancy and oral contraceptives?

Answer 26

inc in pregnancy and by oral contraceptives

Question 27

tx for acute intermittent porphyria?

Answer 27

Glucose and heme, which inhibit ALA synthase

Question 28

Thrombotic thrombocytopenic purpura (TTP) ?

Answer 28

``` def of ADAMTS 13 (vWF cleaving metalloprotease)// inc LDH// pentad of neuro and renal sx, fever, thrombocytopenia, and micrangiopathic hemolytic anemia ```

Question 29

Acute idiopathic thrombocytopenic purpura (ITP)?

Answer 29

Most common cause of thrombocytopenia in children; Abs made in spleen

Question 30

vWF acts to carry/protect?

Answer 30

factor VIII

Question 31

Ristocetin cofactor assays?

Answer 31

evaluate vWF fxn (abnormal in Vw disease and BS disease)

Question 32

Serum is missing?

Answer 32

factors 5,8; prothrombin 2; fibrinogen

Question 33

Prothrombin gene mutation?

Answer 33

Mutation in 3' untranslated region

Question 34

Protein C or S def leads to inc risk of?

Answer 34

Thrombotic skin necrosis w/ hemorrhage following administration of warfarin

Question 35

use of cryoprecipitate?

Answer 35

Treat coagulation factor def involving fibrinogen and factor VIII

Question 36

Blood transfusion risks?

Answer 36

Hypocalcemia (citrate is a calcium chelator) and hyperkalemia

Question 37

Leukemoid rxn ?

Answer 37

Perforated appendicitis (neutros); pertussis (lymphos); and inc leukocyte alkaline phosphatase

Question 38

Origin of Reed Sternberg cells?; secrete ?

Answer 38

CD30+ and CD15+ B-cell origin; RS cells secrete cytokines

Question 39

Translocation of what in mantle cell lymphoma?; how is the prognosis?

Answer 39

translocation of cyclin D1 (11); poor prognosis (CD5+)

Question 40

Most common adult NLH?

Answer 40

Diffuse large B-cell lymphoma

Question 41

mycosis fungoides/ Sezary syndrome?

Answer 41

Cells spread to invade blood with sezary cells (lymphocytes w/ cerebriform nuclei - lobular!) // present w/ Cutaneous patches/nodules; CD4+

Question 42

tx of hairy cell leukemia?

Answer 42

Cladribine (an adenosine deaminase inhibitor)

Question 43

CML ?

Answer 43

inc basophils; may accelerate and transform to AML or ALL ("blast crisis"); very low leukocyte alkaline phosphatase

Question 44

Langerhans cell histiocytosis?

Answer 44

Cells express S-100 (neural crest origin) and CD1a. // Birbeck granules

Question 45

Myelofibrosis?

Answer 45

Ineffective erythropoeisis, EMH

Question 46

LMW heparin examples?; what do they act on?

Answer 46

enoxaparin and dalteparin; they act more on factor Xa

Question 47

Heparin-induced thrombocytopenia (HIT)?

Answer 47

dvlpmnt of IgG Abs against heparin bound to platelet factor 4 (PF4)// complex activates platelets

Question 48

hep vs warfarin ? (structure, inhibits coag in vitro?)

Answer 48

Heparin: Large anionic, acidic polymer ; yes inhibits coag in vitro Warfarin: Small lipid-soluble molecule

Question 49

Toxicity of thrombolytics?

Answer 49

Causes bleeding

Question 50

ADP R inhibitors ? toxicity ?

Answer 50

Clopidogrel, ticlopidine, prasugrel, ticagrelor // neutropenia, fever, and mouth ulcers

Question 51

Cilostazol and dipyridamole?

Answer 51

Phosphodiesterase III inhibitor// | inc cAMP in platelets, thus inhibiting platelet aggregaton; also vasodilators

Question 52

Mechanism of Cytarabine (arabinofuranosyl cytidine) ?

Answer 52

Acts as a pyrimidine analog

Question 53

Toxicity of methotrexate (an antimetabolite)?

Answer 53

Macrovesicular fatty change in liver

Question 54

Dexrazoxane ?

Answer 54

Dexrazoxane used to prevent cardiotoxicity from doxorubicin

Question 55

Cyclophosphamide, ifosamide mech ?

Answer 55

Covalently X-link DNA at guanine N-7. Toxicity from acrolein is prevented with mesna.

Question 56

nitrosoureas mech and use ?

Answer 56

Require bioactivation; alkylating agent | Clinical use: Brain tumors

Question 57

Procarbazine ?

Answer 57

alkylating agent; MAOI

Question 58

amifostine (free radical scavenger) and chloride diuresis to prevent nephrotox from ?

Answer 58

cisplatin, carboplatin

Question 59

Vemurafenib mech ?

Answer 59

Small molecule inhibitor of forms of B-Raf kinase w/ V600E mutation

Question 60

Bevacizumab mech ?

Answer 60

Monoclonal antibody against VEGF

Question 60

Abciximab, eptifibatide, tirofiban?

Answer 60

GP2b/3a inhibs