Heme/Onc Flashcards

1
Q

Dense granules vs alpha granules if platelets ?

A
dense = ADP, calcium;
alpha = vWF, fibrinogen
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2
Q

Decreased FC receptor in immature neutrophils?

A

Decreased CD16

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3
Q

Eosinophils produce?

A

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

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4
Q

Kallikrein stimulates ?

A

HMWK to Bradykinin

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5
Q

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

A

favctors Va, Vllla

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6
Q

antithrombin inhibits activated forms of factors ?

A

12, 11, 7, 9, 10, 2

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7
Q

“activation” phase of platelet plug formation?

A

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

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8
Q

dec ESR?

A

Microcytosis, hypofibrinogenemia

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9
Q

Acanthocytes (spur cells) seen in ?

A

Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)

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10
Q

Howell-Jolly bodies seen in ?

A

pts mothball ingestion (naphthalene)

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11
Q

ferroportin ?

A

Basolateral iron transporter of enterocyte to blood

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12
Q

alpha thalassemia - Hb Barts vs HbH ?

A

gamma4 (4 gene deletion) //

beta3 (3 gene deletion)

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13
Q

beta thalassema - mild anemia mech ?

Severe anemia mech ?

A

DNA splicing defect //

stop codon

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14
Q

dx of beta-thalassemia minor confirmed by?

A

inc HbA2 (> 3.5%) ; dec HbA

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15
Q

tx of Beta-thalassemia major?

A

long term blood transfusion

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16
Q

Sideroblastic anemia causes changes in lvls ?

A

inc iron, normal TIBC, inc ferritin

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17
Q

hemosiderin ?

A

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

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18
Q

tx of orotic aciduria?

A

Uridine monophosphate

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19
Q

nonmegaloblastic macrocytic anemia?

A

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

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20
Q

Intravascular hemolysis causes changes in ?

A

dec haptoglobin, inc LDH

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21
Q

Extravascular hemolysis causes changes in ?

A

inc UCB, inc LDH

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22
Q

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

A

hereditary spherocytosis

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23
Q

paroxysmal nocturnal hemoglobinuria triad, lab findings, tx ?

A

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

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24
Q

Most common cause of death in sickle cell anemia?

A

Strep pneumo sepsis

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25
Q

Sickle cell anemia affects kidney how ?

A

Renal papillary necrosis (due to low O2 in papilla)

26
Q

transferrin production affected by pregnancy and oral contraceptives?

A

inc in pregnancy and by oral contraceptives

27
Q

tx for acute intermittent porphyria?

A

Glucose and heme, which inhibit ALA synthase

28
Q

Thrombotic thrombocytopenic purpura (TTP) ?

A
def of ADAMTS 13 (vWF cleaving metalloprotease)//
inc LDH// pentad of neuro and renal sx, fever, thrombocytopenia, and micrangiopathic hemolytic anemia
29
Q

Acute idiopathic thrombocytopenic purpura (ITP)?

A

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

30
Q

vWF acts to carry/protect?

A

factor VIII

31
Q

Ristocetin cofactor assays?

A

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

32
Q

Serum is missing?

A

factors 5,8; prothrombin 2; fibrinogen

33
Q

Prothrombin gene mutation?

A

Mutation in 3’ untranslated region

34
Q

Protein C or S def leads to inc risk of?

A

Thrombotic skin necrosis w/ hemorrhage following administration of warfarin

35
Q

use of cryoprecipitate?

A

Treat coagulation factor def involving fibrinogen and factor VIII

36
Q

Blood transfusion risks?

A

Hypocalcemia (citrate is a calcium chelator) and hyperkalemia

37
Q

Leukemoid rxn ?

A

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

38
Q

Origin of Reed Sternberg cells?; secrete ?

A

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

39
Q

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

A

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

40
Q

Most common adult NLH?

A

Diffuse large B-cell lymphoma

41
Q

mycosis fungoides/ Sezary syndrome?

A

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

42
Q

tx of hairy cell leukemia?

A

Cladribine (an adenosine deaminase inhibitor)

43
Q

CML ?

A

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

44
Q

Langerhans cell histiocytosis?

A

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

45
Q

Myelofibrosis?

A

Ineffective erythropoeisis, EMH

46
Q

LMW heparin examples?; what do they act on?

A

enoxaparin and dalteparin; they act more on factor Xa

47
Q

Heparin-induced thrombocytopenia (HIT)?

A

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

48
Q

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

A

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

49
Q

Toxicity of thrombolytics?

A

Causes bleeding

50
Q

ADP R inhibitors ? toxicity ?

A

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

51
Q

Cilostazol and dipyridamole?

A

Phosphodiesterase III inhibitor//

inc cAMP in platelets, thus inhibiting platelet aggregaton; also vasodilators

52
Q

Mechanism of Cytarabine (arabinofuranosyl cytidine) ?

A

Acts as a pyrimidine analog

53
Q

Toxicity of methotrexate (an antimetabolite)?

A

Macrovesicular fatty change in liver

54
Q

Dexrazoxane ?

A

Dexrazoxane used to prevent cardiotoxicity from doxorubicin

55
Q

Cyclophosphamide, ifosamide mech ?

A

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

56
Q

nitrosoureas mech and use ?

A

Require bioactivation; alkylating agent

Clinical use: Brain tumors

57
Q

Procarbazine ?

A

alkylating agent; MAOI

58
Q

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

A

cisplatin, carboplatin

59
Q

Vemurafenib mech ?

A

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

60
Q

Bevacizumab mech ?

A

Monoclonal antibody against VEGF

60
Q

Abciximab, eptifibatide, tirofiban?

A

GP2b/3a inhibs