Other Flashcards
1
Q
Protein C function
A
Cleaves and inactivates Va and VIIIa
2
Q
Vwf is founded
A
- a granules of platelets
2. Weidel palade bodies of endothelial cells
3
Q
Inside endothelial cells
A
- Vwf and VIII
- Thrombloplastin
- Thrombomodulin
- tPA
- PGI2
4
Q
Schistocytes are also called
A
Helmet cells
5
Q
Teardrop cells also are also called
A
Dacrocytes
6
Q
Heinz bodies
A
Hb precipitation
7
Q
β-thalassemia treatment side effect
A
Secondary hemochromatosis (from blood transfusions)
8
Q
Anemias-parvovirus
A
- β-Thalassemia
- Aplastic
- Sickle cell
- Hereditary spherocytosis
9
Q
When does the major β-thalassemia become symptomatic
A
6 months (because of HbF)
10
Q
Sideroblastic anemia treatment
A
Pyridoxine (B6)
11
Q
Follate deficiency drugs
A
- Methotrexate
- Phenytoin
- Trimethoprim
12
Q
Intravascular hemolysis findings
A
- Decreased haptoglobin 2. Increased LDH 3. Schistocytes. 4. Increased reticulocytes in peripheral blood smear 5. Hemoglobinuria
- Hemosiderinuria 7. Urobilinogen in urine
13
Q
Extravascular hemolysis findings
A
- Spherocytes 2. Increased LDH 3. No hemoglobinuria/hemosiderinuria 4. Increased unconjucated bilirubin
14
Q
Aplastic anemia-drugs
A
- Benzene
- Chloramphenicol
- Alkylating agents
- Antimetabolites
15
Q
Increased MCHC anemia
A
Hereditary spherocytosis
16
Q
Paroxismal noctural hemoglobinuria - impaired synthesis of
A
GPI-anchor for decay accelerating factor
17
Q
Hemophillia treatment
A
A–> desmopressin and factor 8 concentrate
B–> factor 9 concentrate
C–> factor 11 concentrate
18
Q
TTP symptoms
A
- Neurological
- Renal
- Fever
- Thrombocytopenia
- Microangiopathic hemolytic anemia
19
Q
Packed RBCs dosage effect
A
Increased Hb and O2 carrying capacity
20
Q
Platelets dosage effect
A
Increases 5000
21
Q
Neutropenia causes
A
- Postinfection/sepsis
- SLE
- Drugs (Chemotherapy)
- Radiation
- Aplastic anemia
22
Q
Eosinopenia
A
Corticosteroids
23
Q
Lymphopenia
A
- Scid
- SLE
- Corticosteroids
- POSTOPERATIVE
- DiGeorge
- Radiation
- Sepsis
24
Q
LAP
A
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
