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Flashcards in RSL - Haem Deck (57):
1

Anisocytosis

Varying sizes

2

Poikilocytosis

Varying shapes

3

Dense granules (platelets)

ADP, Ca++

4

Alpha granules (platelets)

vWF, Fibrinogen

5

Specific granules (neutrophils)

ALP, collagenase, lysozyme, lactoferrin

6

Azurophilic granules (neutrophls; lysosomes)

Proteinases, acid phosphatase, myeloperoxidase, beta-glucuronidase

7

Neutrophil chemotactic factors

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

8

Frosted glass cytoplasm

Monocyte

9

Eosinophil products

Major basic protein and histaminase

10

Basophillic granules (Basophils)

Densely packed with histamine and hepararin

11

IgE crosslinking on mast cells -->

Release of contents: Histamine, heparin, eosinphilic chemotactic factors

12

Cromolyn Sodium

Prevents mast cell degranulation

13

Thrombomodulin-thombin complex

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

14

Factor XIIIa

Stabolizes fibrin meshwork

15

Vitamin K is needed for:

II, VII, IX, X

16

Requires Ca++

V, VIII, VII

17

vWF is from?

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

18

Platelet plug formation: 1. injury

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

19

Platelet plug formation: 2. Exposure

vWF binds collagen

20

Platelet plug formation: 3. Adhesion

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

21

Platelet plug formation: 4. Activation

ADP binding receptor --> Gp IIb/IIIa expression

22

Platelet plug formation: 5. Aggregation

Fibrinogen binds Gp IIb/IIIa linking platelets

23

Acanthocyte

Liver disease, abetalipoproteinemia (cholesterol dysregulation): Spiny cell

24

Basophillic stippling

Lead poisoning

25

Degmacyte

Bite cell; G6PD deficiency

26

Target cell

HbC disease, asplenia, Liver disease, thalasemia

27

Heinz bodies

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

28

Howell jolly bodies

Basophillic remnants found in RBCs.

29

Iron absorption state

Fe3+

30

Ferroportin

brings Iron into blood -->transferrin

31

Plummer Vinson triad

IDA, Esophageal webs, Atrophic glossitis

32

Cis deletion of thalassemia association

Asian populations

33

Trans deletion of thalassemia association

African populations

34

Alpha vs Beta thal mutations

Alpha: deletion. Beta: splice sites and promoter sequences

35

Lead poisoning (3 mech)

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

36

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

Lead poisoning

37

LEAD sucks

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

38

Sideroblastic anemia lab values (Iron, TIBC, Ferritin)

Iron high
TIBC Normal/low
Ferritin high

39

Sideroblastic anemia treatment

Pyridoxine (B6)

40

Myelophthisic process

Pathological replacement of bone marrow

41

HbC defect

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

42

Diseases / drugs associated with aplastic anemia / crisis

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

43

Paroxysmal nocturnal hemoglobinuria CD?

CD55/59; Decay accelerating factor and MAC inhibitory

44

Sickle cell defect

Glutamic acid --> valine

45

Cold agglutinin seen in?

CLL, Mycoplasma pneumonia, infectious Mononucleosis

46

Lead poisoning Accumulated substance

Protoporphyrin, ALA

47

Heme production

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

48

Iron poisoning - mechanism

Peroxidation of lipid membranes

49

Cryoprecipitate

Fibrinogen, VIII, XIII, vWF, FIbronectin

50

Sideroblastic anemia: causes

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

51

orotic aciduria

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

52

Hepcidin

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

53

Treat PNH

Eculizumab (terminal compliment inhibitor)

54

Bernard-Soulier syndrome (agglutination?)

Gp1b deficit
No ristoceitin agglutination

55

Glanzmann Thrombasthenia

GpIIb/IIIa loss

56

Von Willebrand disease: inheritance

AD

57

Pentostatin

Hairy cell leukemia