Shit - Heme Flashcards

1
Q

Platalets:

  • lifespan
  • dense granules
  • alpha granules
A
  • lifespan 8-10d
  • dense = APD and Ca
  • alpha = vWF and fibrinogen
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2
Q

Neutrophils:

  • specific granules:
  • azuriphilic granules:
A

specific = ALP, collagenase, lactoferrin, lysozyme* (cell wall breaking)

Azurophilic = proteinase, acid phosphatase, MPO (makes green pus), b-glucuronidase

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3
Q

Macrophages and shock

A

Macro CD14 —-Lipid A of LPS

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4
Q

causes of eosinophilia

A

Il-5, asthma, allergy, neoplasia, parasites, chronic adrenal insufficiency

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5
Q

Content of eosinophils vs basophils vs mast cells

A

eos = MBP and histaminase

baso = histamine, heparin, late LTs

mast = histamine, heparin, tryptase, eosinophil chemotactic factor

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6
Q

Prevention of mast cell degranulation

A

Cromolyn sodium

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7
Q

NK markers

A

CD16 and CD56

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8
Q

Kleihauer-Betke test

A

%HbF in maternal blood, determines rhogam dosing

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9
Q

Extrinsic coagulaton pathway: players and test

A

VII to VIIa by III

III = thromboplastin = tissue factor; activated by endothelial injury

PT

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10
Q

plasminogen to plasmin

A

tPA (endogenous)

Alteplase, reteplase, streptokinase, tenecteplase

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11
Q

Stops plasmin formation:

A

a2-anti-plasmin (endogenous)

Aminocaproic acid

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12
Q

Factors activated by vit K

A

II, VII, IX, X, C, S

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13
Q

Protein C role

A

Inactivate Va and VIIIa

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14
Q

Factor V liden mutation

A

Factor V that can’t be inactivated by protein C –> clotting disease

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15
Q

Antithrombin inactivates:

enhanced by:

A

II, VII, IX, X, XI, XII

*most important = II and Xa

enhanced by heparin

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16
Q

Platelet activation:

  • activated by:
  • release:
A

Activated by platelet GpIb ——- vWF—endotheium

Release ADP, CA and TXA2

ADP ——ADP-R of other platelets –> GPIIb/IIIa espression, which binds fibrinogen causing platelet aggregatoin

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17
Q

Where does vWF come from?

A

Weibel-palade bodies of endothelium

Alpha-granules of platelets

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18
Q

ADP-R inhibitors

A

Clopidogrel, prasugrel, triclopidine

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19
Q

Direct GPIIb/IIIa inhibitors

A

Abciximab, eptifibatide, tirofiban

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20
Q

Ristocetin

A

promotes vWF — GpIb

decreased agglutinatoin in test indicated von Willibran disease or bernard soulier (GpIb deficiency)

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21
Q

Direct thrombin inhibitors:

A

agatroban, dabigatran, bivalirudin

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22
Q

Direct Xa inhibitors

A

Apixaban, rivaroxaban

*fondaparineux

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23
Q

Spur cells (acanthocytes)

vs echinocyte (burr cell)

A

spur = liver disease, abetalipoproteinemia, vit E deficiency

burr = EDTA artifact, uremia, PK deficiency, hypo-Mg, hypo-P, hemolytic anemia, mechanical damage

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24
Q

Basophilic stippling vs ringed sideroblasts

A

basophilic stippling = lead poisoning

ringed sideroblasts = sideroblastic anemia, IRON in the mitochondria becasue d-ala-synthase deficiency MA (also can be from lead poisoning)

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25
Q

bite cells other name:

A

degmacyte

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26
Q

target cells:

A

HbC, Asplenia, Liver disease, Thalassemias

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27
Q

Plumer-Vinson

A

IDA, esophageal webs, atrophic glossitis

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28
Q

alpha-thal

  • chrom/problem
  • Types
A

Cr16; deletions

cis = asian = same strand (worse, creates HbBarts)

trans = african = different

HbBarts = hydrops = 0/4

HbH = 1/4

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29
Q

Beta-thal

  • chrom/problem
  • types
A

Cr 11, point mutations in spice sites/promoters

Medeterranian

Major = high HbF

Minor = high HbA2

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30
Q

Lead roles:

A

Blocks ALA-dehydratase (2) and ferrocheletase (7, last)

Inhibits rRNA degradatoin (basophilic stippling)

Causes sideroblastic anemia

S/S foot/wrist drop, encephalopathy, GI

31
Q

Lead poisoning Rx

A

Dimercaprol

EDTA

Succimer (kids)

Penicillamine

32
Q

Causes of sideroblastic anemia

A

Hereditary: ALA synthase (1, RLS); XR

Acquired: MDS

Reversible: alcohol, lead poisoning, B6 defic., Cu defic., INH

33
Q

Drug causes of folate deficiency

A

TMP, MTX, phenytoin

34
Q

Orotic aciduria:

cause

labs

A

UMP synthase deficiency (AR); builup of orotic acid, no pyrimidine de novo

Megaloblastic anemia refractory to B9 and B12, orotic acid in urine, NO hyperammonemia

35
Q

Non-megaloblastic macrocytic anemia causes

A

alcoholism

liver disease

hypothyroidism

reticulocytosis

5-FU

36
Q

hepcidin

  • MOA
  • disease and causes
  • Rx
A

ARP form liver, increase by inflammation (or high Fe)

blocks ferroportin, preventing intestal cells from BL transport to transferrin, and macrophage release

ACD: SLE, RA, neoplasm, CKD

Rx = EPO

37
Q

Aplastic anemia

  • causes
  • S/s
A

Causes:

  • radiation.drugs (benzene, alkyl, chloramphenicol)
  • B19, HIV, HCV, EBV
  • Fanconi anemia

S/s: fatigue, infections, bleeding (petechiae, purpura)

38
Q

Hemolytic anemia in a newborn

A

Pyruvate kinase deficiency

39
Q

PNH: triad and long-term sequale

A

Triad = Coombs negative HA, pancytopenia (acquired SC defect), venous thrombosis (RBCs relelase pro-coagulant stuff)

Increased incidence of ALL

40
Q

HbS vs HbC mutations

Test for both

A

both Cr.11 pos 6

HbS = valine (/) for glutamate (-); H-phobic, sickles

HbC = lysine (+) for glutamate (-)

*think about electrophoresis bands*

Test = sodium metabisulphite screen (both will sickle)

41
Q

AHA warm vs cold: coombs, causes, and Abs

A

Coombs +

Warm = IgG = SLE and CLL

Cold = IgM = mycoplasma, mono, malignancy, CLL

42
Q

Pregnancy/OCP effect on iron handling

A

increases TIBC(transferrin); so decreases %transferin saturation

43
Q

Corticosteroids on blood cell profile

A

Neutrophilia (prevents extravasation)

Eosinopenia (sequesters them in LNs)

Lymphopenia (apoptosis)

44
Q

Lead poisoning steps affected + location + buildup

A

2 = ADA dehydratase = cytoplasm = d-ALA

7 = ferrocheletase = mitochondria = iron and protoporphyrin

45
Q

Acute Intermittent Porphyria step blocked and buidup

Exacerbated by:

Rx:

A

3 = porphobilinogen deaminase = PBG buildup

Exacerbated: 450 inducers, alcohol, starvation

Rx = glucose

46
Q

Porphyria cutanea tarda enzyme and buildup:

A

Uroporphyrinogen decarboxylase; uroporphyrinogen III buids up

47
Q

Iron poisoning microscopy and major s/s

A

Membrane lipid peroxidation

GI bleed

48
Q

Desmopresin Rx for:

A

Hemophilia A (VIII) and vWF deficiency

(endothelial release)

49
Q

Cagualtion factor inhibitor disease

A

Abs to CFs

MC = VIII; looks like hemophilia A, but when you mix it with test blood PTT doesnt normalize becasue the ABs just attack the fresh cells

50
Q

Bernard soulier defect

A

GpIb of platelets (doesnt bind vWF on endothelium)

no agglutination with ristocetin

51
Q

Glanzmann’s Thromblasthenia

A

GpIIb/IIIa deficiency

no platelet crosslingking because cant bind fibrinogen

52
Q

ITP

Rx

A

Anti-GpIIb/IIIa

Rx = steroids (decrease Ab production via lymphocyte apoptosis), IVIG (gives Abs something else to bind)

53
Q

Killer T-cell MOA killing

A

Perforin and granzyme –> activate CASPASES

54
Q

Features of anaplastic cells

A

loss of polarity, nuclear polymorphism, irregular mitoses, high N:C, giant multinucleated tumour cells

55
Q

etoposide/teniposide inhibit:

topotecan/irinotecan inhibit:

A

topo II reannealling their dsDNA breaks

topo I

*1 can has 2 sides*

56
Q

Rb-P means:

A

inactivated

cell can progress thru cell cycle (G1 to S)

57
Q

Role of protecin C (and S)

A

deactivate factors V and VIII by proteolysis

58
Q
A
59
Q

AT-III deficiency labs, cause

A

NORMAL PT, PTT, TT

Heparin deos NOT increase PTT

AD or acquired (proteinuria)

60
Q

Propthombin 20210A mutation

A

Point mutation in 3’UTR, which increases protheombin production

61
Q

Fibrinolysis disorders

A

radical prostatectomy (urokinase release)

liver cirrhosis –> low a2-anti-plasmin

s/s = DIC but NORMAL platelet count; increased fibrinogen split products (not fibrin)

Rx = aminocaproic acid (inhibits plasminogen)

62
Q

Cryoprecipitate: contents and when its used

A

Fibrinogen, vWF, VIII, XIII, fibroniectin

Fibrinogen or VIII deficiencies

63
Q

CD 15 and CD30+

A

Reid Sternberg Hodgkins B-cell lymphoma

64
Q

Types of Hodgkins lymphoma + histology

A

Reid-sternberg owl-eyes

Nodular sclerosing: M=F, 70%, cervical or mediatinal, lacunae

Lymphocyte risk: good Px

Mixed or Lymphocyte depleted: bad Px

65
Q

Types of burkitts + location

A

Endemia = africa = jaw

Sporatic = pelvis or abdomen

Immunodeficient

66
Q

painless waxing and waning LAD: Dx

A

Follicular lymphoma (bcl-2; 14;18)

67
Q

mantle cell TL and CD

A

11;14, CD5

68
Q

ATCL s/s

A

lytic bone lesions, hypercalcemia, generalized LAd with HSM

69
Q

mycoses fungioides vs. sezary syndrome

Histo

A

MF = cutaneous, lymphoma

SS = cutaneous and systemic, leukemia

Criberiform nuclei

70
Q

lytic bone lesions in MM: pathophysiology

A

Neoplastic B-cells secrete Il-1 and IL-6

IL-1 activates osteoclasts

71
Q

MM vs MGUS vs WM

A

MM = IgG (MC) or IgA M-spike with CRAB s/s

MGUS = IgG or IgA spike with NO s/s (can become MM)

WM = IgM M-spike, hyperviscocity syndromes (blurred vision, raynauds)

72
Q

Myelodysplastic syndrome: disease, causes, sequale

A

de novo mutation or enviro (benzene, radiation, chemo)

>20% blasts = AML

73
Q

Pseudo-Pelger-Huet anomaly

A

bilobed PMNs, seen after chemo

74
Q

Sickle cell and MCHC

A

high via RBC dehydration