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Ryan's shit list - Step 1 > RSL - Haem > Flashcards

RSL - Haem Flashcards

1
Q

Anisocytosis

A

Varying sizes

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2
Q

Poikilocytosis

A

Varying shapes

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3
Q

Dense granules (platelets)

A

ADP, Ca++

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4
Q

Alpha granules (platelets)

A

vWF, Fibrinogen

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5
Q

Specific granules (neutrophils)

A

ALP, collagenase, lysozyme, lactoferrin

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6
Q

Azurophilic granules (neutrophls; lysosomes)

A

Proteinases, acid phosphatase, myeloperoxidase, beta-glucuronidase

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7
Q

Neutrophil chemotactic factors

A

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

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8
Q

Frosted glass cytoplasm

A

Monocyte

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9
Q

Eosinophil products

A

Major basic protein and histaminase

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10
Q

Basophillic granules (Basophils)

A

Densely packed with histamine and hepararin

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11
Q

IgE crosslinking on mast cells –>

A

Release of contents: Histamine, heparin, eosinphilic chemotactic factors

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12
Q

Cromolyn Sodium

A

Prevents mast cell degranulation

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13
Q

Thrombomodulin-thombin complex

A

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

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14
Q

Factor XIIIa

A

Stabolizes fibrin meshwork

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15
Q

Vitamin K is needed for:

A

II, VII, IX, X

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16
Q

Requires Ca++

A

V, VIII, VII

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17
Q

vWF is from?

A

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

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18
Q

Platelet plug formation: 1. injury

A

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

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19
Q

Platelet plug formation: 2. Exposure

A

vWF binds collagen

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20
Q

Platelet plug formation: 3. Adhesion

A

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

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21
Q

Platelet plug formation: 4. Activation

A

ADP binding receptor –> Gp IIb/IIIa expression

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22
Q

Platelet plug formation: 5. Aggregation

A

Fibrinogen binds Gp IIb/IIIa linking platelets

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23
Q

Acanthocyte

A

Liver disease, abetalipoproteinemia (cholesterol dysregulation): Spiny cell

24
Q

Basophillic stippling

A

Lead poisoning

25
Q

Degmacyte

A

Bite cell; G6PD deficiency

26
Q

Target cell

A

HbC disease, asplenia, Liver disease, thalasemia

27
Q

Heinz bodies

A

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
Q

Howell jolly bodies

A

Basophillic remnants found in RBCs.

29
Q

Iron absorption state

30
Q

Ferroportin

A

brings Iron into blood –>transferrin

31
Q

Plummer Vinson triad

A

IDA, Esophageal webs, Atrophic glossitis

32
Q

Cis deletion of thalassemia association

A

Asian populations

33
Q

Trans deletion of thalassemia association

A

African populations

34
Q

Alpha vs Beta thal mutations

A

Alpha: deletion. Beta: splice sites and promoter sequences

35
Q

Lead poisoning (3 mech)

A
  1. Blocks ferrochelatase
  2. Blocks ALA dehydratase
  3. Blocks rRNA breakdown –>Basophilic stippling
36
Q

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

A

Lead poisoning

37
Q

LEAD sucks

A

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

38
Q

Sideroblastic anemia lab values (Iron, TIBC, Ferritin)

A

Iron high
TIBC Normal/low
Ferritin high

39
Q

Sideroblastic anemia treatment

A

Pyridoxine (B6)

40
Q

Myelophthisic process

A

Pathological replacement of bone marrow

41
Q

HbC defect

A

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

42
Q

Diseases / drugs associated with aplastic anemia / crisis

A

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
Q

Paroxysmal nocturnal hemoglobinuria CD?

A

CD55/59; Decay accelerating factor and MAC inhibitory

44
Q

Sickle cell defect

A

Glutamic acid –> valine

45
Q

Cold agglutinin seen in?

A

CLL, Mycoplasma pneumonia, infectious Mononucleosis

46
Q

Lead poisoning Accumulated substance

A

Protoporphyrin, ALA

47
Q

Heme production

A

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

48
Q

Iron poisoning - mechanism

A

Peroxidation of lipid membranes

49
Q

Cryoprecipitate

A

Fibrinogen, VIII, XIII, vWF, FIbronectin

50
Q

Sideroblastic anemia: causes

A

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

51
Q

orotic aciduria

A

UMP synthase def.

Orotic acid –> UMP –> pyrimidines

52
Q

Hepcidin

A

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

53
Q

Treat PNH

A

Eculizumab (terminal compliment inhibitor)

54
Q

Bernard-Soulier syndrome (agglutination?)

A

Gp1b deficit

No ristoceitin agglutination

55
Q

Glanzmann Thrombasthenia

A

GpIIb/IIIa loss

56
Q

Von Willebrand disease: inheritance

57
Q

Pentostatin

A

Hairy cell leukemia

Ryan's shit list - Step 1 (27 decks)

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