RSL - Haem

Question 1

Anisocytosis

Answer 1

Varying sizes

Question 2

Poikilocytosis

Answer 2

Varying shapes

Question 3

Dense granules (platelets)

Answer 3

ADP, Ca++

Question 4

Alpha granules (platelets)

Answer 4

vWF, Fibrinogen

Question 5

Specific granules (neutrophils)

Answer 5

ALP, collagenase, lysozyme, lactoferrin

Question 6

Azurophilic granules (neutrophls; lysosomes)

Answer 6

Proteinases, acid phosphatase, myeloperoxidase, beta-glucuronidase

Question 7

Neutrophil chemotactic factors

Answer 7

C5a, IL-8, LTB4, Kallikrein, platelet-activating factor

Question 8

Frosted glass cytoplasm

Answer 8

Monocyte

Question 9

Eosinophil products

Answer 9

Major basic protein and histaminase

Question 10

Basophillic granules (Basophils)

Answer 10

Densely packed with histamine and hepararin

Question 11

IgE crosslinking on mast cells –>

Answer 11

Release of contents: Histamine, heparin, eosinphilic chemotactic factors

Question 12

Cromolyn Sodium

Answer 12

Prevents mast cell degranulation

Question 13

Thrombomodulin-thombin complex

Answer 13

–> Activates protein C which binds Protein S –> Factor Va / VIIIa cleavage

Question 14

Factor XIIIa

Answer 14

Stabolizes fibrin meshwork

Question 15

Vitamin K is needed for:

Answer 15

II, VII, IX, X

Question 16

Requires Ca++

Answer 16

V, VIII, VII

Question 17

vWF is from?

Answer 17

Weibel-palade bodies of endothelial cells and alpha granules of platelets

Question 18

Platelet plug formation: 1. injury

Answer 18

endothelial damage –> transient vasoconstriction via neural stimulation reflex and endothelin (released from damaged cell)

Question 19

Platelet plug formation: 2. Exposure

Answer 19

vWF binds collagen

Question 20

Platelet plug formation: 3. Adhesion

Answer 20

Platelets bind vWF via Gp Ib –> conformational change –> dense granule release (ADP/Ca++ –> coagulation), TXA2 –> platelet aggregation

Question 21

Platelet plug formation: 4. Activation

Answer 21

ADP binding receptor –> Gp IIb/IIIa expression

Question 22

Platelet plug formation: 5. Aggregation

Answer 22

Fibrinogen binds Gp IIb/IIIa linking platelets

Question 23

Acanthocyte

Answer 23

Liver disease, abetalipoproteinemia (cholesterol dysregulation): Spiny cell

Question 24

Basophillic stippling

Answer 24

Lead poisoning

Question 25

Degmacyte

Answer 25

Bite cell; G6PD deficiency

Question 26

Target cell

Answer 26

HbC disease, asplenia, Liver disease, thalasemia

Question 27

Heinz bodies

Answer 27

Oxidation of Hb-SH groups to S--S --> Hb precipitation --> phavocytic damage to RBC membrane --> bite cells. Seen in G6PD deficiency, and alpha thalasemia

Question 28

Howell jolly bodies

Answer 28

Basophillic remnants found in RBCs.

Question 29

Iron absorption state

Answer 29

Fe3+

Question 30

Ferroportin

Answer 30

brings Iron into blood -->transferrin

Question 31

Plummer Vinson triad

Answer 31

IDA, Esophageal webs, Atrophic glossitis

Question 32

Cis deletion of thalassemia association

Answer 32

Asian populations

Question 33

Trans deletion of thalassemia association

Answer 33

African populations

Question 34

Alpha vs Beta thal mutations

Answer 34

Alpha: deletion. Beta: splice sites and promoter sequences

Question 35

Lead poisoning (3 mech)

Answer 35

1. Blocks ferrochelatase 2. Blocks ALA dehydratase 3. Blocks rRNA breakdown -->Basophilic stippling

Question 36

Burtons lines on gingevae and lines on metaphysis of long bones on xray

Answer 36

Lead poisoning

Question 37

LEAD sucks

Answer 37

Lead Lines, Encephalopathy and erythrocyte basophyllic stippling, Abdominal pain and Anemia, Drops - wrist and foot; treat with Dimercaprol and EDTA, Succimer for chelation

Question 38

Sideroblastic anemia lab values (Iron, TIBC, Ferritin)

Answer 38

Iron high TIBC Normal/low Ferritin high

Question 39

Sideroblastic anemia treatment

Answer 39

Pyridoxine (B6)

Question 40

Myelophthisic process

Answer 40

Pathological replacement of bone marrow

Question 41

HbC defect

Answer 41

Glutamic acid (-) --> Lysine (+) mutation in Beta globin

Question 42

Diseases / drugs associated with aplastic anemia / crisis

Answer 42

Radiation and drugs: Chloramphenicol, Carbamazepine, Methimazole, NSAIDs, Benzene, Chloramphenicol, Propylthiouracil; Viral agents: Parvovirus B19, EBV, HIV, HCV; Diseases: Beta thal, sickle, paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis, fanconi anemia

Question 43

Paroxysmal nocturnal hemoglobinuria CD?

Answer 43

CD55/59; Decay accelerating factor and MAC inhibitory

Question 44

Sickle cell defect

Answer 44

Glutamic acid --> valine

Question 45

Cold agglutinin seen in?

Answer 45

CLL, Mycoplasma pneumonia, infectious Mononucleosis

Question 46

Lead poisoning Accumulated substance

Answer 46

Protoporphyrin, ALA

Question 47

Heme production

Answer 47

Glycine+Succinyl CoA --> ALA --> Porphobilinogen -->Hydroxymethylbilane --> Uroporphobilinogen --> coproporphobilinogen --> protoporphyrin --> Heme

Question 48

Iron poisoning - mechanism

Answer 48

Peroxidation of lipid membranes

Question 49

Cryoprecipitate

Answer 49

Fibrinogen, VIII, XIII, vWF, FIbronectin

Question 50

Sideroblastic anemia: causes

Answer 50

Genetic Alcohol (reversible) Lead Vit. B6 def. (isoniazid, copper def.)

Question 51

orotic aciduria

Answer 51

UMP synthase def. | Orotic acid --> UMP --> pyrimidines

Question 52

Hepcidin

Answer 52

Binds ferroportin on intestinal cells / macrophages -->decreased Iron release into blood

Question 53

Treat PNH

Answer 53

Eculizumab (terminal compliment inhibitor)

Question 54

Bernard-Soulier syndrome (agglutination?)

Answer 54

Gp1b deficit | No ristoceitin agglutination

Question 55

Glanzmann Thrombasthenia

Answer 55

GpIIb/IIIa loss

Question 56

Von Willebrand disease: inheritance

Answer 56

AD

Question 57

Pentostatin

Answer 57

Hairy cell leukemia