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Flashcards in Shit - Heme Deck (74):
1

Platalets:

- lifespan

- dense granules

- alpha granules

- lifespan 8-10d

- dense = APD and Ca

- alpha = vWF and fibrinogen

2

Neutrophils:

- specific granules:

- azuriphilic granules:

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

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

3

Macrophages and shock

Macro CD14 ----Lipid A of LPS

4

causes of eosinophilia

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

5

Content of eosinophils vs basophils vs mast cells

eos = MBP and histaminase

 

baso = histamine, heparin, late LTs

 

mast = histamine, heparin, tryptase, eosinophil chemotactic factor 

6

Prevention of mast cell degranulation

Cromolyn sodium

7

NK markers

CD16 and CD56

8

Kleihauer-Betke test

%HbF in maternal blood, determines rhogam dosing

9

Extrinsic coagulaton pathway: players and test

VII to VIIa by III

III = thromboplastin = tissue factor; activated by endothelial injury

PT

10

plasminogen to plasmin

tPA (endogenous)

Alteplase, reteplase, streptokinase, tenecteplase

11

Stops plasmin formation:

a2-anti-plasmin (endogenous)

Aminocaproic acid 

12

Factors activated by vit K

II, VII, IX, X, C, S

13

Protein C role

Inactivate Va and VIIIa

 

14

Factor V liden mutation

Factor V that can't be inactivated by protein C --> clotting disease

15

Antithrombin inactivates:

enhanced by:

II, VII, IX, X, XI, XII

*most important = II and Xa

 

enhanced by heparin

16

Platelet activation:

- activated by:

- release:

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

17

Where does vWF come from?

Weibel-palade bodies of endothelium

Alpha-granules of platelets

18

ADP-R inhibitors

Clopidogrel, prasugrel, triclopidine

19

Direct GPIIb/IIIa inhibitors

Abciximab, eptifibatide, tirofiban

20

Ristocetin 

promotes vWF --- GpIb

 

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

21

Direct thrombin inhibitors:

agatroban, dabigatran, bivalirudin

22

Direct Xa inhibitors

Apixaban, rivaroxaban

*fondaparineux

23

Spur cells (acanthocytes)

vs echinocyte (burr cell)

spur = liver disease, abetalipoproteinemia, vit E deficiency

 

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

24

Basophilic stippling vs ringed sideroblasts

basophilic stippling = lead poisoning

 

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

25

bite cells other name:

degmacyte

26

target cells:

HbC, Asplenia, Liver disease, Thalassemias

27

Plumer-Vinson

IDA, esophageal webs, atrophic glossitis

28

alpha-thal

- chrom/problem

- Types

Cr16; deletions

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

trans = african = different

HbBarts = hydrops = 0/4

HbH = 1/4

29

Beta-thal

- chrom/problem

- types

Cr 11, point mutations in spice sites/promoters

Medeterranian

Major = high HbF

Minor = high HbA2

30

Lead roles:

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

Inhibits rRNA degradatoin (basophilic stippling)

Causes sideroblastic anemia

S/S foot/wrist drop, encephalopathy, GI

 

31

Lead poisoning Rx

Dimercaprol

EDTA

Succimer (kids)

Penicillamine

32

Causes of sideroblastic anemia

Hereditary: ALA synthase (1, RLS); XR

Acquired: MDS

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

 

33

Drug causes of folate deficiency

TMP, MTX, phenytoin

34

Orotic aciduria:

cause

labs

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

Non-megaloblastic macrocytic anemia causes

alcoholism

liver disease

hypothyroidism

reticulocytosis

5-FU

36

hepcidin

- MOA

- disease and causes

- Rx

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

Aplastic anemia

- causes

- S/s

Causes:

- radiation.drugs (benzene, alkyl, chloramphenicol)

- B19, HIV, HCV, EBV

-  Fanconi anemia

 

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

38

Hemolytic anemia in a newborn

Pyruvate kinase deficiency

39

PNH: triad and long-term sequale

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

 

Increased incidence of ALL

40

HbS vs HbC mutations

Test for both

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

AHA warm vs cold: coombs, causes, and Abs

Coombs +

 

Warm = IgG = SLE and CLL

Cold = IgM = mycoplasma, mono, malignancy, CLL

42

Pregnancy/OCP effect on iron handling

increases TIBC(transferrin); so decreases %transferin saturation

43

Corticosteroids on blood cell profile

Neutrophilia (prevents extravasation)

Eosinopenia (sequesters them in LNs)

Lymphopenia (apoptosis)

44

Lead poisoning steps affected + location + buildup

2 = ADA dehydratase = cytoplasm = d-ALA

 

7 = ferrocheletase = mitochondria = iron and protoporphyrin 

45

Acute Intermittent Porphyria step blocked and buidup

Exacerbated by:

Rx:

3 = porphobilinogen deaminase = PBG buildup

Exacerbated: 450 inducers, alcohol, starvation

Rx = glucose

46

Porphyria cutanea tarda enzyme and buildup:

Uroporphyrinogen decarboxylase; uroporphyrinogen III buids up

47

Iron poisoning microscopy and major s/s

Membrane lipid peroxidation

GI bleed

48

Desmopresin Rx for:

Hemophilia A (VIII) and vWF deficiency

(endothelial release)

49

Cagualtion factor inhibitor disease

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

Bernard soulier defect

GpIb of platelets (doesnt bind vWF on endothelium)

no agglutination with ristocetin

51

Glanzmann's Thromblasthenia

GpIIb/IIIa deficiency

no platelet crosslingking because cant bind fibrinogen

52

ITP

Rx

Anti-GpIIb/IIIa

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

53

Killer T-cell MOA killing

Perforin and granzyme --> activate CASPASES

54

Features of anaplastic cells

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

55

etoposide/teniposide inhibit:

 

topotecan/irinotecan inhibit:

topo II reannealling their dsDNA breaks

 

topo I

 

*1 can has 2 sides*

56

Rb-P means:

inactivated

cell can progress thru cell cycle (G1 to S)

57

Role of protecin C (and S)

deactivate factors V and VIII by proteolysis

58

59

AT-III deficiency labs, cause

NORMAL PT, PTT, TT

Heparin deos NOT increase PTT

 

AD or acquired (proteinuria)

60

Propthombin 20210A mutation

Point mutation in 3'UTR, which increases protheombin production

61

Fibrinolysis disorders

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

Cryoprecipitate: contents and when its used

Fibrinogen, vWF, VIII, XIII, fibroniectin

 

Fibrinogen or VIII deficiencies

63

CD 15 and CD30+

Reid Sternberg Hodgkins B-cell lymphoma

64

Types of Hodgkins lymphoma + histology

Reid-sternberg owl-eyes

 

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

Lymphocyte risk: good Px

Mixed or Lymphocyte depleted: bad Px

65

Types of burkitts + location

Endemia = africa = jaw

Sporatic = pelvis or abdomen

Immunodeficient

66

painless waxing and waning LAD: Dx

Follicular lymphoma (bcl-2; 14;18)

67

mantle cell TL and CD

11;14, CD5

68

ATCL s/s

lytic bone lesions, hypercalcemia, generalized LAd with HSM

69

mycoses fungioides vs. sezary syndrome

Histo

MF = cutaneous, lymphoma

SS = cutaneous and systemic, leukemia

 

Criberiform nuclei

70

lytic bone lesions in MM: pathophysiology

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

IL-1 activates osteoclasts

71

MM vs MGUS vs WM

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

Myelodysplastic syndrome: disease, causes, sequale

 

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

 

>20% blasts = AML

73

Pseudo-Pelger-Huet anomaly

bilobed PMNs, seen after chemo

74

Sickle cell and MCHC

high via RBC dehydration