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Flashcards in FA + MTB Deck (91):
1

site of B cells in periphery

1. follicles of Lymph node
2. white pulp of spleen
3. unencapsulated lymphoid tissue

2

fetal erythropoiesis

1. yolk sac: 3-8 weeks
2. liver: 6 weeks - birth
3. spleen: 10-28 weeks
4. Bone marrow: 18 weeks - adult

3

from fastests to slowest Hb (FROM (-) to (+))

A - F - S - C

4

extrinsic pathway (tissue factor pathway) - factors

7 --> 10

5

coagulation intrinsic pathway - factors (contact pathway)

12 --> 11 --> 9 (and then combined)

6

coagulation cascade - combined pathway

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

thrombin actions

- fibrinogen to fibrin monomers
- XIII --> XIIIa
- V --> Va
- VIII --> VIIIa

8

Coagulation steps that require calcium and phospholipids

Around activated 10
Around activated 7

9

Factor Xa inhibitors anticoagulants

1. LMWH (greatest efficacy)
2. Heparin
3. Direct Xa inhibitors (apixaban, rivaroxaba)
4. Fondaparinux

10

Thrombin inhibitors (anticoagulants)

1. Heparin (greatest efficacy)
2. LMWH (delteparin, enoxaparin)
3. Direct thrombin inhibitors (argatroban, bivalirudin, dagigatran)

11

role of vitamin K in procoagulation

oxidized vit K --> reduced K (epoxide reductase)
--> act as a cofactor of γ-glutamyl trasnferase to 2, 7, 9, 10, C, S maturation

12

Pr C to activated Pr C (inactivates ...)

Thrombin- trombomodulin complex (endothelial cells)
inactivates 5+8

13

Heparin enhances the activity of

Antithrombin

14

Principles targets of antithrombin

Thrombin and factor Xa

15

Ristocetin - mechanism of action

Activates vwf to bind GpIb

16

Basophilic stippling RBCs - seen in

1. Lead poisoning
2. sideroblastic anema
3. Myelodisplastic syndromes

17

Acanthocytes RBCs - seen in

1. liver disease
2. abetalipoproteinemia (states of cholesterol dysregulation)

18

Acanthocytes RBCs - also called

spur cell

19

Target RBCs - seen in

1. HbC disease 2. asplenia
3. Liver disease 4. Thalassemia

20

Echinocytes - seen in

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

21

Echinocytes vs acanthocytes according to image

Echinocytes projection are more uniform and smaller

22

Heinz bodies - seen in

1. G6PD deficiency
2. Heinz body - like inclusions in α-thalassemia

23

Heinz bodies - mechanism

Oxidation of HB-SH groups to S-S --> Hb precipitation (Heinz bodies) --> phagocytic damage to RBC membrane --> bite cells

24

Howell-Jolly bodies - seen in

1. 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