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Flashcards in Other Deck (120):
1

Protein C function

Cleaves and inactivates Va and VIIIa

2

Vwf is founded

1. a granules of platelets
2. Weidel palade bodies of endothelial cells

3

Inside endothelial cells

1. Vwf and VIII
2. Thrombloplastin
3. Thrombomodulin
4. tPA
5. PGI2

4

Schistocytes are also called

Helmet cells

5

Teardrop cells also are also called

Dacrocytes

6

Heinz bodies

Hb precipitation

7

β-thalassemia treatment side effect

Secondary hemochromatosis (from blood transfusions)

8

Anemias-parvovirus

1. β-Thalassemia
2. Aplastic
3. Sickle cell
4. Hereditary spherocytosis

9

When does the major β-thalassemia become symptomatic

6 months (because of HbF)

10

Sideroblastic anemia treatment

Pyridoxine (B6)

11

Follate deficiency drugs

1. Methotrexate
2. Phenytoin
3. Trimethoprim

12

Intravascular hemolysis findings

1. Decreased haptoglobin 2. Increased LDH 3. Schistocytes. 4. Increased reticulocytes in peripheral blood smear 5. Hemoglobinuria
6. Hemosiderinuria 7. Urobilinogen in urine

13

Extravascular hemolysis findings

1. Spherocytes 2. Increased LDH 3. No hemoglobinuria/hemosiderinuria 4. Increased unconjucated bilirubin

14

Aplastic anemia-drugs

1. Benzene
2. Chloramphenicol
3. Alkylating agents
4. Antimetabolites

15

Increased MCHC anemia

Hereditary spherocytosis

16

Paroxismal noctural hemoglobinuria - impaired synthesis of

GPI-anchor for decay accelerating factor

17

Hemophillia treatment

A--> desmopressin and factor 8 concentrate
B--> factor 9 concentrate
C--> factor 11 concentrate

18

TTP symptoms

1. Neurological
2. Renal
3. Fever
4. Thrombocytopenia
5. Microangiopathic hemolytic anemia

19

Packed RBCs dosage effect

Increased Hb and O2 carrying capacity

20

Platelets dosage effect

Increases 5000

21

Neutropenia causes

1. Postinfection/sepsis
2. SLE
3. Drugs (Chemotherapy)
5. Radiation
6. Aplastic anemia

22

Eosinopenia

Corticosteroids

23

Lymphopenia

1. Scid
2. SLE
3. Corticosteroids
4. POSTOPERATIVE
5. DiGeorge
6. Radiation
7. Sepsis

24

LAP

Leukocyte alkaline phosphate

25

Non hodgkin age

20-40

26

Non-hodgkin may associated with

HIV
autoimmune disease

27

Multiple myeloma x ray

Punched out lytic bone lesions

28

Neonates -- vit k

Neonates --> lack enteric bacteria --> low vit K

29

Macrohemorrage in hemophilia

1. Hemarthroses
2. Easy bruising
3. Easy bleeding after trauma or surgery (dental procedures)

30

Hereditary thrombosis syndromes leading to hypercoagulability

1. V Leiden
2. Prothrombin mutation
3. Pr C or S deficiency
4. Antithrombin deficiency

31

B12 absorption

salivary amylase liberates B12 --> bound to R-binder (also from salivary gland) --> pancreatic protease detach B12 from R --> B12 binds intrinsic factor --> absorbed in ileum as a complex

32

Echinocytes - seen in

1. end-stage renal disease
2. liver disease
3. pyruvate kinase

33

Fanconi anemia - mode of inheritance

AR

34

Hemophilia C (vs A,B)

less hemarthrosis
less spontaneous bleeding
more common in Askentzi

35

lymphoma stage (Ann Arbor)

Stage I: single region, usually one lymph node and the surrounding area
Stage II two separate regions, an affected lymph node or organ and a second affected area, and that both affected areas are confined to one side of the diaphragm—that is, both are above the diaphragm, or both are below the diaphragm. (IIE if extralymphatic tissue or spleen)
Stage III both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen. (IIIE if extralymphatic tissue or spleen)
Stage IV indicates diffuse or disseminated involvement of one or more extralymphatic organs, including any involvement of the liver, bone marrow, or nodular involvement of the lungs.

36

MC hematologic problem in Down sndrome

acute megakaryoblastic leukemia

37

low albumini in infants -->

jaundice

38

Schilling test - stages

1. IM unlabeled B12 oral labeled B12 (IM replenish the store so the oral labeled B12 is in urine)
2. IF + oral labeled B12
3. oral labeled B12 + oral antibiotic
4. oral labeled B12 + pancreatic enzymes

39

Rb - active vs inactive form and mechanism

GF --> activation of Cyclin D + E + cdk 4 + 6 --> hyperphosphorylation --> inactive
hypophosphorylation --> active --> binds E2F --> inhibition of G1 to S

40

p27 function

cell cycle inhibitor (stop it in G1) by inhibiting CDKs
(downregulation in cancer)

41

SLE mediated pancytopenia

type 2 hypersensitivity --> antibodies agienst cells

42

EGFR pathway

EGFR --> RAS --> nucleus

43

panitumumab?

like cetuximab

44

RBC - stomatocytes?

in hereditary stomatocytosis

45

patrier micro-abscess

by T cells infiltration in the skin (Mycoses fungoides)

46

precursor B-ALL vs T-ALL

both TdT
B --> CD10, 19
T --> 2, 3, 4, 5, 7, 8, 1a

47

special characteristic of atypical lymphocytes (eg. EBV)

more cytoplasm

48

biposy diagnostic for MM

more than 30% plasma cells

49

MM mediated osteopenia

IL-1 + IL6 secretion

50

anorexia in cachecia

hypothalamus leads to appetite suppression

51

Platelet function analyzer (PFA-100) test

monitors the time needed for a patient's blood to form a platelet based hemostatic plug in an in vitro environment

52

HER2 is a family member of

EGFR

53

a special characteristic that differentiates dysplasia from Ca

dysplasia is reversible (once invade the basement membrane --> irreeversible ca

54

Osteomyelitis - mcc (and 2MCC) in SCA

1. salmonela
2. S. aureus / E. coli

55

Burkit lymphoma - cells

monomorphic, medium size with round nuclei, multiple nucleoli, vacuolated basophilic cytoplasm

56

t15,17 in AML menas

PML/PAR
PML: promyelocytic leukemia gene
RAR: retinoic acid receptor

57

integrin binds to ECM - specifically

1. fibronectin
2. collagen
3. laminin
(and also binds intracellulary to actin + keratin)

58

caspases - structure + what they cleave

containe cysteine
they are able to cleave aspartic acid residues

59

bleeding problems in renal disease

Uremic platelet dysfunction: uremia --> qualitive platelet disorder --> increased BT with normal platelet count, PT, PTT

60

SC trait - malaria

relative protection --> but when visiting endemic areas should receive prophylaxis

61

Hb electrophoresis pattern and persentages in normal , SC trait, SC

Normal: 99% HbA, 0% S, less than 1% Hb F
SC: 0% HbA, 5-15 F, 85-95 S
SC trait: 50-60 % HbA, less than 2 F, 35-45 S

62

Pleomorphic xanthoastrocytoma

an astrocytoma found in in children and yound adults
--> rarely progress to GBM
histology: reticulin deposits + chronic inflammatory infiltrates

63

cyclophosphamide metabolite causes cystitis - which metabolite

acrolein

64

podophylin - mechanism of action + clinical use

inhibits topoisomerase 2
treat genital wart

65

folate administration in in B12 deficient

1. partially correct anemia
2. worse the neurological symptoms

66

braf related cancer

1. melanoma
hairy cell leukemia

67

aplastic anemia - splenomegaly

no because no progenitors

68

JAK2 mutation in chronic myeloproliferative disoreders

V617F (replaces valine with phenylalanine) --> more sensitive to GF such as erythropoietin + thrmobopoietin)

69

polyceythemia vera can cause peptic ulcer - mechanism

increased histamine secretion (also pruritus)

70

SIS gene - ca

proto-oncogene --> astrocytoma, osteosarcoma

71

TGFA gene - ca

proto-oncogene --> astrocytoma, HCC

72

HER1

proto-oncogene --> scc of lung

73

colon cancer stage

stage A: mucosa (90% survival in 5 years)
stage B: muscular (70-80)
stage C: lymph node (poor)
stage D: distant metstasis (poor)

74

placental abruption mediated DIC - mechanism

tissue factor release in high concentration from the placental trophoblast (placental abruption)

75

placental abruption vs amniotic fluid mediated DIC according to mothers sympoms

amniotic --> PE --> hypotension + cardiogenic shock

76

Breast cancer prognosis in BCL-2 overexpression

better

77

mutated fene in paroxysaml nocturnal hemoglobinuria

phosphatidyloinositol glycan class A (PIGA gene) --> help on GPI synthesis

78

chromosomal rearrangement in non-small cell lung ca

EML4-ALK --> constitutive active tyrosine kinase
Echinoderm microtubule-associated protein like 4, anaplastic lymphoma kinase

79

carbon tetracloride - mechanism of liver damage

CCL4 --> oxidized by P450 --> CCL3 --> lipid peroxidation

80

biopsy - diagnosis of AML

more than 20% of myeloblasts in bone marrow

81

2ry ITP is sometime associated with

HIV or HCV

82

pancreatic role in B12 absorption

cleave R factor from B12 --> allowing B12 to bind to IF

83

ca cell migration steps

1. detachment (decreasing of E-cadherin)
2. Adhesion to basement membtain (increasing of laminin)
3. invasion (MMPs

84

HBV mediated HCC

integration --> HBx protein transcription --> suppression of p53

85

Patietns receiving regular transfusion should undegro routine

chelation therapy

86

tumor that causes pure red aplasia

thymoma, lymphicytic leukemia

87

paraneoplastic of uterine fibrinoids

erithropoietin

88

Pro-carcinoges are converted to carcinogens by

cyt P45O oxidase system (microsomal monoxygenase)

89

leukemoid reaction - number of leukocytes / and histology

more than 50 000
peripheral smear can show Dohle bodies (basophilic) in neutrophils

90

leukemia with 13q-

CLL

91

MTX - enter ther the cell

undergoes polyglutamation --> prevents the movement out of the cell

92

etoposide, tenoposide + ... (similar)

podophyllin (topically to treat genital warts)

93

MCC of thombocytopenia in hospitilized patients

HIT

94

topoisomerase type 1 vs 2 according to action

1. --> single strand --> relieve negative supercoilling
2. --> transient break in both strands --> relieve both positive + negative supercoiling

95

Fludarabine?

deamination resistant purine nucleotide analog --> inhibition of DNA pol, primase, ligase, ribon reductase
--> CLL

96

mensa - mechanism of action

sulfhydryl compound that binds acrolein in the urine (cyclophosphamide is converted to mensa by acrolein)

97

folic acid - MTX v Fluorouracil

reverse toxicity of MTX
increases action of Fluororuacil

98

MDR1?

P-glycoprotein --> transmembrane ATP depended efflux pump protein --> reduces influx and increases efflux of drug --> prevents action of chemotherapeuitic agents
ALSO IN BBB --> prevent penetration of foreign compounds into CNS

99

MM myeloma cells are susceptible to

protease inhibition due to large amount of proteins that they manufacture (SUCH AS BORTEZOMIB, a boronic acid-containing dipeptide) --> APOPTOSIS

100

DVT in pregnancy - treatment

LMWH

101

oral 5-FU

GI ulcerations

102

OKT3?

monoclonal antibody against CD3 --> immunosuppression

103

first cells infected by EBV

pharyngeal B lymphocytes

104

hereditary spherocytosis - mode inheritance

AD

105

embryonic hemoglobins

1. Gower 1 ζ2ε2
2. Portland ζ2γ2
3. Gower 2 α2ε2
(Yolk sac)

106

CLL genetics

BCL2 overexpression

107

Porphyrea cutanea tarda - hereditary or acquired

both, but acquired is MC
due to iron and susceptibility factors (alcohol, smoking, HCV, HIV, halogenated hydrocarbons)

108

SCC - organs with more sickling

organs with high metabolic demands: brain, muscles, placenta

109

what increases the action of ALA synthase

CYP450 inducers

110

Isonizid leading to B6 deficiency

pyridoxine phosphokinase inhibition

111

high oxygen affinity Hb leads to (eg. Hb Chesapeake + Kempsey)

reduced ability to release oxygen within the peripheral tissues --> renal hypoxia --> increased EPO --> erythrocytosis

112

HbS --> why is that bad (chemistry)

contains valine instead of Glutamic --> hydrophobic interaction among Hb molecules --> polymerization + erythrocyte sickling (and weaker 2,3-DPG bidning)
(NO alternation in β structure)

113

pyruvate kinase deficiency - causes of splenomegaly

low ATP --> low transport of ions in RBCs--> deformed erythrocytes --> increased work of splenic parenchyma tp remove them --> Red pulp hyperplasia

114

B12 deficiency is due to complete absence for .... (how long)

4-5 years

115

hemophagocytic lymphohistiocytosis - definition

abnormal activation + proliferation of lymphocytes leading to hemophagocytosis and secretion of preinflammatory cytokines --> immune system overactivation --> attack native cells in bone marrow
it an also causes inflammation of the brain

116

causes and diagnosis of hemophagocytic lymphohistiocytosis

causes: Familiar (AR), 2ry to EBV
diagnosis: bone marrow --> macrophages engulfing RBCs

117

Trousseau syndrome is caused by (and mechanism)

Pancreatic tumor --> tissue factor

118

stomach tumor can cause jaundice?

tumor in lesser stomach --> mass effect --> compress hepatoduodenal ligament --> jaundice

119

DLBCL markers

similar with CLL (including CD5+)

120

IBD - race

Askenazi Jewish