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