FA + MTB Flashcards
1
Q
site of B cells in periphery
A
- follicles of Lymph node
- white pulp of spleen
- unencapsulated lymphoid tissue
2
Q
fetal erythropoiesis
A
- yolk sac: 3-8 weeks
- liver: 6 weeks - birth
- spleen: 10-28 weeks
- Bone marrow: 18 weeks - adult
3
Q
from fastests to slowest Hb (FROM (-) to (+))
A
A - F - S - C
4
Q
extrinsic pathway (tissue factor pathway) - factors
A
7 –> 10
5
Q
coagulation intrinsic pathway - factors (contact pathway)
A
12 –> 11 –> 9 (and then combined)
6
Q
coagulation cascade - combined pathway
A
X –> Xa
Xa + Va –> prothrombin to thrombin
thrombin –> fibrinogen to fibrin and XIII to XIIIa
–> aggegation of fibrin + Ca2+ + XIIIa –> firin mesh stabilizes platelet plug
7
Q
thrombin actions
A
- fibrinogen to fibrin monomers
- XIII –> XIIIa
- V –> Va
- VIII –> VIIIa
8
Q
Coagulation steps that require calcium and phospholipids
A
Around activated 10
Around activated 7
9
Q
Factor Xa inhibitors anticoagulants
A
- LMWH (greatest efficacy)
- Heparin
- Direct Xa inhibitors (apixaban, rivaroxaba)
- Fondaparinux
10
Q
Thrombin inhibitors (anticoagulants)
A
- Heparin (greatest efficacy)
- LMWH (delteparin, enoxaparin)
- Direct thrombin inhibitors (argatroban, bivalirudin, dagigatran)
11
Q
role of vitamin K in procoagulation
A
oxidized vit K –> reduced K (epoxide reductase)
–> act as a cofactor of γ-glutamyl trasnferase to 2, 7, 9, 10, C, S maturation
12
Q
Pr C to activated Pr C (inactivates …)
A
Thrombin- trombomodulin complex (endothelial cells)
inactivates 5+8
13
Q
Heparin enhances the activity of
A
Antithrombin
14
Q
Principles targets of antithrombin
A
Thrombin and factor Xa
15
Q
Ristocetin - mechanism of action
A
Activates vwf to bind GpIb
16
Q
Basophilic stippling RBCs - seen in
A
- Lead poisoning
- sideroblastic anema
- Myelodisplastic syndromes
17
Q
Acanthocytes RBCs - seen in
A
- liver disease
2. abetalipoproteinemia (states of cholesterol dysregulation)
18
Q
Acanthocytes RBCs - also called
A
spur cell
19
Q
Target RBCs - seen in
A
- HbC disease 2. asplenia
3. Liver disease 4. Thalassemia
20
Q
Echinocytes - seen in
A
- end-stage renal disease
- liver disease
- pyruvate kinase
21
Q
Echinocytes vs acanthocytes according to image
A
Echinocytes projection are more uniform and smaller
22
Q
Heinz bodies - seen in
A
- G6PD deficiency
2. Heinz body - like inclusions in α-thalassemia
23
Q
Heinz bodies - mechanism
A
Oxidation of HB-SH groups to S-S –> Hb precipitation (Heinz bodies) –> phagocytic damage to RBC membrane –> bite cells
24
Q
Howell-Jolly bodies - seen in
A
- asplenia
2. functional hyposplenia
25
ringed sideroblasts cells vs basophilc cells regarding area
basoph: peripheral smear
siderob: BM
26
Non-Megaloblastic macrocytic anemias
1. Hypothyroidism
2. Liver disease
3. Alcoholism
4. Reticulocytosis
5. Diamond-Blackdan anemia
27
Nonhemolytic normocytic anemias
1. Anemia of chronic disease (early)
2. Iron deficiency (early)
3. Chronic kidney disease
4. Aplastic anemia
28
Extrinsic Hemolytic normocytic anemias
1. Autoimmune
2. Macroangiopathic
3. Microangiopathic
4. Infections
29
Intrinsic Hemolytic normocytic anemias
1. RBC membrane defects (hereditary spherocytosis)
2. RBC enzymes deficiency (G6PD, pyruvate kinase)
3. HBC defects
4. Paroxysmal nocturnal hemlglobinuria
5. Sickle cell anemia
30
another cause of megalobastic anemia
fanconi
31
Iron deficiency iron labs
- Decreased iron
- Decreased ferritin
- Increase TIBC
- microcytosis and hypochromasia (central pallor)
- INCREASED ERYTHRO PROTOPORPHYRIN
32
Plummer-Vinson triad
1. Iron deficiency anemia
2. Esophageal web
3. dysphagia
(also atrophic glossitis)
33
a Thalassemia: cis vs trans thalassemia
Cis: Asia, -/- a/a (same chromosome)
Trans: Africa, a/- a/- (separate chormosomes)
34
3 allele a deletion
very little α
| HbH (4β)
35
4 allele a deltion
```
Hb bart (4γ)
Hydrops fetalis
```
36
Major β-thalassemia major - presentation (besides anemia) and mechanism (when starts)
1. marrow expansion --> skeleta deformities (Crew cut on skull x ray) + chipmunk facies
2. extramedullary hematopoiesis --> hepatosplenomegaly
Until 6 months is asymptomatic (HbF)
37
Major β-thalassemia major - definition/lab/treatment
homozygote --> β chain is absent
severe microcytic, hypochromic anemia with target cells and increased anisopoikilocytosis, high HbF
treatment: blood transfusion (2ry hemochromatosis)
38
HbS/β-thalassemia heterozygote
Mild to moderate sickle cell anemia depending on amount of β-globin production
39
Microcytic anemias
1. Iron deficiency (late)
2. Thalassemias
3. Anemia of chronic disease (late)
4. Lead poisoning
5. Sideroblastic anemia
40
Lead inhibits (causing)
Ferrochelatase and ALA dehydratase --> decreases heme and increase protoporphyrin
rRNA degradation --> rRNA aggregation --> basophilic stippling
41
Lead poisoning symptoms and signs
1. Burton lines
2. Metaphysis lines on x ray
3. Encephalopathy
4. Erythrocyte basophilic stippling
5. Siderblastic anemia
6. ABDOMINAL COLIC
7. Drops of wrist and foot
8. KIDNEY DISEASE
42
Lead poisoning-first line treatment
Sideroblastic anemia types of causes (and causes)
43
Sideroblastic anemia types of causes (and causes)
1. Genetic --> X-linked defect in δ-ALA synthase gene
2. acquired --> Myelodysplastic syndrome
3. reversible --> Alcohol (MC), Lead, Vit B6 deficiency, Isoniazid, Copper deficiency, chloramphenicol)
44
sideroblastic anemia - treatment
B6, cofactor for δ-ALA synthase
45
Findings of megaloblastic anemia
1. RBC macrocytosis 2. Hypersegmented neutrophils 3. Glossitis
46
Folate deficiency vs b12 deficiency according to methylmalonic acid, homocystein, and neurologic symptoms
1. Methylmalonic acid (increased only in B12)
2. Neurologic symptoms (only in B12)
3. Homocysteine (increased in Both)
47
Neurologic symptoms of B12 deficiency (and why)
Sabacute combined degeneration due to its involvement in fatty acid pathays and myelin synthesis:
1. Spinocerebellar tract
2. Lateral corticospinal tract
3. Dorsal column dysfunction
48
Orotic aciduria symptoms
1. Failure to thrive
2. Developmental delay
3. Megaloblastic anemia refractory to folate and B12
(NO HYPERAMMONIA)
49
Orotic aciduria treatment/diagnosis
UMP (to bypass)
| diagnosis: Orotic acid in urine
50
Anemia of chronic disease iron status / type of anemia
Decreased iron
Decreased TIBC
Increased ferritin
--> Normocytic, but can become microcytic
51
Hereditary spherocytosis - due to defect in
proteins interacting with RBC membrane skeleton and plasma membrane
1. Ankyrin
2. Band 3
3. Protein 4.2
4. Spectrin
52
Hereditary spherocytosis labs
1. Osmotic fragility test (+)
2. Normal to decreased MCV with abudance of cells
3. Increased RDW
4. Increased MCHC (no central pallor)
5. round RBCs with less surface area
53
Hereditary spherocytosis treatment/intravascular vs extravascular/susceptible for
- Splenectomy (causes splenomegaly)
- extravascular hemolysis
- susceptible for B19
54
G6PD deficiency - hemolytic anemia following
1. Sulfa drugs
2. Antimalarias
3. Infections
4. Fava beans
55
Pyruvate deficiency anemia pathophysiology
AR. Decreased ATP--> rigid RBCs --> extravascular hemolysis
56
HBC pathophysiology
Lysine instead of glutamic acid in β globin
57
Starting event of paroxysmal nocturnal hemoglobinuria / mechanism
Acquired mutation in hematopoietic stem cell --> impaired synsthesis of for GPI anchor for decay-accelerating factor that protects RBCs from complement --> increased C-mediated intravascular RBCs lysis
58
paroxysmal nocturnal hemoglobinuria increases the incidence of
ACUTE LEUKEMIAS
59
paroxysmal nocturnal hemoglobinuria triad
1. Coombs hemolytic anemia
2. Pancytopenia
3. Venous thrombosis
60
paroxysmal nocturnal hemoglobinuria flow cytometry
CD 55/59 ( - )RBCs
61
paroxysmal nocturnal hemoglobinuria treatment
Eculizumab (terminal complement inhibitor)
62
Sickle cell anemia mutation / intravascular vs extravascular hemolysis
Valine instead of Glutamic acid
| intravascular + extravascular
63
Sickle cell anemia diagnosis and treatment
Diagnosis: Hb electrophoresis
Treatment: hydroxyurea, hydration
64
Pregnancy (ocp use) - iron status
Increases the Transferin/TIBC
| Decrease the transferin saturation
65
Autoimmune hemolytic anemia types
Warm (IgG)
| Cold (IgM and complement)
66
Causes of Warm agglutinin
1. SLE
2. CLL
3. Drugs (methyldopa)
4. Idiopathic
- -> chronic anemia
67
Causes of Cold agglutinin
1. CLL
2. Mycoplasma pneumonia
3. Infectious mononucleosis
4. Idiopathic
- -> acute anemia
68
Diamond-Blackfan anemia - description
Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells
increased % of HbF (but low total Hb)
69
Diamond-Blackfan anemia - presentation
1. short stature
2. craniofacial abnormalities
3. upper extremity malformations (triphalangeal thumbs) in up to 50% of cases
70
HbC - homozygotes - blood smear
hemoglobin crystals within RBCs and target cells
71
RBC agglutination with cold exposure - presentation
acute anemia
| painful, blue fingers and toes with cold exposure
72
extravascular hemolytic anemia - findings
1. Spherocytes in peripheral smear
2. high LDH
3. no hemoglobinuria/hemosiderinuria
4. increased UCB (--> jaundice)
5. urobilinogen in urine
73
intravascular hemolytic anemia - findings
1. low haptoglobin
2. high LDH
3. schistocytes
4. high reticulocytes
5. hemoglobinuria
6. hemosiderinuria
7. urobilinogen in urine
8. increased UCB
74
Fanconi anemia - mechanism of action
DNA repair defect causing bone marrow failure
75
Fanconi anemia - presentation
1. Aplastic anemia
2. Increased incidence of tumor/leukemia
3. Cafe au lait spots
4. Thumb/radial defects
76
iron studies in iron deficiency anemia
- serum iron: low
- TIBC: high
- Ferritin: low
- iron/TIBC sat: low
77
iron studies in hemochromatosis
- serum iron: high
- TIBC: low
- Ferritin: high
- iron/TIBC sat: high
78
iron studies in Chronic disease
- serum iron: low
- TIBC: low
- Ferritin: high
- iron/TIBC sat: normal
79
iron studies in pregnancy/OCP
- serum iron: normal
- TIBC: increased
- Ferritin: normal
- iron/TIBC sat: decreased
80
neutropenia - cell count
less than 1500 cells/mm3
81
lymphopenia - cell count
lymphocytes less than 1500 (3000 in children)
82
eosinopenia - cell count / causes
less than 30 cells/mm3
Cushing syndrome
corticosteroinds
83
Lead poisoning - enzyme affected (and where)
1. Ferrochelatase (mit)
| 2. ALA dehydratase (cytoplasm)
84
Lead poisoning - way of exposure and neurological symptoms (children and adults)
Children --> exposure to lead paint --> mental deterioration
Adults --> environmental exposure (batteries ammunition) --> headache, memory loss, demyelination
85
Acute intermittent porhyria symptoms
mnemonic - 5 Ps
1. Painful abdomen
2. Port wine - colored urine
3. Polyneuropathy
4. Psychological disturbances
5. Precipitated by drugs (P450 inducers) Alcohol, starvation
86
Acute intermittent porhyria affected enzyme
Porphobilinogen deaminase
87
Acute intermittent porhyria is precipitated by
1. Drugs (cytochromic P-450 inducers)
2. Alcohol
3. Starvation
88
Acute intermittent porhyria treatment
Glucose and heme (inhibit ALA synthase)
89
Porphyria cutanea tarda affected enzyme
Uroporphyrinogen decarboxylase
90
Porphyria cutanea tarda - presenting symptoms / assocaited with
Blistering cutaneous photosensitivity
HCV
exacerbated with alcohol
91
MC porphyria
Porphyria cutanea tarda
