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Flashcards in 3/10 heme/onc Deck (130)
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1
Q

RBC membrane has what transporter?

A

Membrane contains chloride- HCO3- antiporter, which allows RBCs to export HCO3- and transport CO2 from the periphery to the lungs for elimination.

2
Q

Anisocytosis:

A

varying sizes of RBCs

3
Q

Poikilocytosis:

A

varying shapes

4
Q

platelet

  • dense granules:
  • alpha granules:
A
  • dense granules = ADP, calcium

- a granules = vWF, fibrinogen

5
Q

WBC differential from highest to lowest

-mnemonic:

A
Neutrophils Like Making Everything Better.
Neutrophils (54–62%)
Lymphocytes (25–33%)
Monocytes (3–7%)
Eosinophils (1–3%)
Basophils (0–0.75%)
6
Q

Neutrophils

-granules contain what?

A
  • ALP, collagenase, lysozyme, and lactoferrin.

- azurophilic granules (lysosomes) contain proteinases, acid phosphatase, myeloperoxidase, and β-glucuronidase.

7
Q

Band cells

-what are they?

A

immature neutros

8
Q

Cell surface marker for macrophage?

A

CD14

9
Q

Eosinophil

  • phagocytic?
  • role in type 1 HSR?
A
  • Highly phagocytic for antigen-antibody complexes.

- Produces histaminase and arylsulfatase (helps limit reaction following mast cell degranulation).

10
Q

Causes of eosinophilia =

-mnemonic?

A
NAACP:
Neoplasia 
Asthma
Allergic processes
Connective tissue diseases
Parasites (invasive)
11
Q

Isolated basophilia found in:

A

CML.

12
Q

Mast cell degranulation releases:

A

Histamine, heparin, and eosinophil chemotactic factors.

13
Q

Universal donor of plasma?

-which blood type

A

AB

14
Q

Universal recipient of plasma?

-which blood type?

A

O

15
Q
  • anti-A and anti-B antibodies =
  • anti-Rh=

-what type of Ab and does it cross the placenta?

A
  • IgM (do not cross placenta)

- IgG (cross placenta)

16
Q

Erythryoblastosis fetalis

-Tx:

A

Treatment: Rho(D) IgG for mother during every pregnancy to prevent initial sensitization of Rh- mother to Rh antigen.

17
Q

In basic terms, what is coagulation?

A

Coagulation = soluble fibrinogen converting to insoluble fibrin.
-property of plasma. dont need anything else.

18
Q

Heparin sulfate

-what hooks onto it?

A

-antithrombin 3

19
Q

Antithrombin inhibits activated forms of factors:

A

II, VII, IX, X, XI, XII.

-1972 + 11 & 12

20
Q

thrombomodulin

-binds what? which activates what?

A
  • thrombin

- thrombin activates protein C which inhibits factors 5 & 8.

21
Q

Effects of thrombin on factor 5 & 8?

A

-coag. starts slow, but once thrombin made, thrombin goes back and activates factors 8 and 5 which accelerate the pathway. This speeds up coag.

-Protein C and S inactivate factor 8a and 5a. They’re the
“brakes” to coag pathway.

22
Q

Endothelial cell: Weiber-palody body contains:

A

-vWF in the endothelial cell. These contain vWF & ps-electin.

23
Q

Ticlopidine and clopidogrel:

A
  • inhibit ADP-induced expression of GpIIb/IIIa.

- irreversibly bind ADP receptor.

24
Q

Ristocetin:

A
  • Activates vWF to bind to GpIb.

- Useful for diagnosis: normal platelet aggregation response is not seen in von Willebrand disease.

25
Q

Acanthocyte (spur cell)

-associated w/

A

-Liver disease, abetalipoproteinemia (states of cholesterol dysregulation).

26
Q

Basophilic stippling

-mnemonic

A

BASically, ACiD alcohol is LeThal.

  • Anemia of Chronic Disease
  • alcohol abuse
  • Lead poisoning
  • Thalassemias.
27
Q

Bite cell

-which disease?

A

G6PD deficiency.

28
Q

Elliptocyte

-which disease?

A

Hereditary elliptocytosis.

29
Q

Macro-ovalocyte

-which disease?

A
  • Megaloblastic anemia (also hypersegmented PMNs)

- marrow failure.

30
Q

Mechanical trauma (ie. prosthetic valve) vs. trauma from microthrombi:

A

Look at the platelet count! if its normal, then its a prosthetic, if platelet count is down then its a microthrmbi.

31
Q

Spherocyte

-which disease?

A

-Hereditary spherocytosis, autoimmune hemolysis.

32
Q

Target cell

  • which disease?
  • mnemonic?
A

“HALT,” said the hunter to his target.

-HbC disease, Asplenia, Liver disease, Thalassemia.

33
Q

Heinz bodies

A
  • Oxidation of hemoglobin sulfhydryl groups.
  • Turn into bite cells
  • G6PD def.
34
Q

Corrected reticulocyte count

A

(hct/45)*(retic count).

  • if there are polychromatic cells, then divide by 2
  • 3%+ = good marrow response, 2% and lower = bad marrow response.
35
Q

TIBC inversely related to what?

A

Serum ferritin

36
Q

Law of RBC size

A

RBC always wants to maintain normal concentration of Hb. So if you have low Hb, RBC will be made smaller so that conc. will still be the same!

37
Q

Microcytic anemias

-mnemonic?

A

TAILS

  • Thalassemias
  • ACD
  • Iron def. anemia
  • Lead poisoning
  • Sideroblastic anemia
38
Q

Megaloblastic anemias

-mnemonic?

A

FOBs have nutrition deficiencies.

  • Folate def
  • Orotic aciduria
  • B12 def.
39
Q

Copper deficiency can what type of anemia?

A

microcytic sideroblastic anemia.

40
Q
  • koilonychia & pica

- which disease?

A
  • iron def. anemia
  • koilonychia = spoon shaped nails
  • pica = eating random things
41
Q

How to measure transferrin?

A

TIBC

42
Q

RDW & FEP in iron def anemia

-inc or dec?

A
  • RDW inc. (RBC distribution width)

- FEP inc. (Free erythrocyte protoporphyrin)

43
Q

alpha-thal

  • gene mutations/deletions?
  • cis = asian or african?
  • trans = asian or african?
A
  • gene deletions.
  • cis deletion prevalent in Asian populations.
  • trans deletion prevalent in African populations.
44
Q

Hb Barts

  • what is it?
  • what fetal problem does it cause?
A
  • all 4 alpha-globin genes deleted
  • excess gamma globin forms gamma(4) = Hb Barts
  • causes hydrops fetalis

*incompatible w/life

45
Q

HbH disease

A
  • 3 alpha-globin allele deletion
  • Excess β-globin forms β4 (HbH).
  • very severe anemia.
46
Q

hydrops fetalis:

A

-Extensive compensatory erythropoiesis in the liver disrupts portal blood flow, contributing to dec. albumin synthesis, hypoalbuminemia, and generalized edema (anasarca).

47
Q

How do thalassemias lead to abdominal pain?

A

1) splenic infarction

2) inc hemolysis -> bilirubin gallstones -> RUQ pain.

48
Q

beta-globin genes

-on which chrom?

A

11

49
Q

beta-thal major

-why is there marrow expansion?

A
  • severe anemia due to lack of HbA.
  • tons of EPO released by kidney.
  • marrow hyperplasia and expansion.
50
Q

beta-thal

-mutations or deletions?

A

-point mutations

51
Q

Beta-thal minor

-How do you diagnose?

A

-Dx confirmed by inc. HbA2 (> 3.5%) on electrophoresis.

52
Q

Beta-thal major

  • what causes RBC damage?
  • how can it lead to secondary hemochromatosis?
A
  • no beta chains, so you get alpha tetramers = damages RBC. Spleen will destroy these RBCs w/alpha tetramers.
  • lots of blood transfusions can overload you w/iron.
53
Q

How long after birth does beta-thal major Sxs show up?

A
  • after 5 months

- HbF is protective

54
Q

How does lead poisoning lead to basophilic stippling?

A

Inhibits rRNA degradation, causing RBCs to retain aggregates of rRNA (basophilic stippling).

55
Q

Zn-protoporphyrin

  • seen in what disease?
  • why/how?
A

Lead poisoning

-ferrochelatase knocked off, cant add iron to protoporphyrin, so Zn inserts itself instead, you get Zn-protoporphyrin.

56
Q

Lead poisoning

  • Sxs:
  • mnemonic?
A

LEAD:
-Lead Lines on gingivae (Burton lines) and on metaphyses of long bones D on x-ray.
-Encephalopathy and Erythrocyte basophilic
stippling.
-Abdominal colic and sideroblastic Anemia.
-Drops—wrist and foot drop

57
Q

Lead poisoning

-Tx:

A
  • Dimercaprol and EDTA are 1st line of treatment.

- Succimer for kids.

58
Q

Sideroblastic anemia

  • inheritance?
  • whats the problem?
A
  • X-linked defect in δ-ALA synthase gene.

- also non-inherited causes as well.

59
Q

Cofactor for δ-ALA synthase?

A

B6

60
Q

Sideroblastic anemia

-non-genetic causes:

A

-acquired: myelodysplastic syndromes
-reversible: alcohol is most common, lead, vitamin B6
deficiency, copper deficiency, and isoniazid.

61
Q

Isoniazid can cause which type of anemia?

A

-sideroblastic anemia due to B6 deficiency

62
Q

Sideroblastic anemia

  • serum iron?
  • serum ferritin?
  • TIBC?
A
  • inc iron
  • inc ferritin
  • normal/dec TIBC
63
Q

Sideroblastic anemia

-Tx:

A

pyridoxine (B6)

64
Q

Ferrochelatase

-where is this reaction going down?

A
  • mitochondria.
  • must be, or else why would the iron be accumulating in the mitochondria when this enzyme doesn’t work in lead poisoning or sideroblastic anemia?
65
Q

Ringed sideroblasts seen in which diseases?

A
  • Lead poisoning
  • Sideroblastic anemia
  • iron getting trapped in mitochondria.
66
Q

If a diabetic is under great glycemic control but the HbA1c still really high, what one possible explanation?

A

-Could just mean less HbA1 like w/beta-thalassemia.

67
Q

which anemia can alcohol cause? and how?

A

alcohol can cause macrocytic anemia via toxicity to bone marrow.

68
Q

Folate deficiency

  • homocysteine: inc or dec?
  • methylmalonic acid: inc or dec?
A

-inc. homocysteine: less folate = less B12 taking
methyl group = less passing methyl to homocysteine
= less conversion to methionine = pile up of homocyst.

-methylmalonic acid -> succinyl coa: B12 needed, &
B12 normal here, so methylmalonic acid is normal. This
is good to cancel out B12 def.

69
Q

How does phenytoin lead to megaloblastic anemia?

A

-Folate absorbed in jejunum. Intestinal conjugase (inhib. by phenytoin) req for absorption.

70
Q

How can Crohns disease lead to megaloblastic anemia?

A
  • B12 resorbed in terminal ileum.

- terminal ileum often involved in Crohns disease.

71
Q

PPIs

-what kind of anemia can these lead to?

A

B12 deficiency megaloblastic anemia.

-

72
Q

Most common cause of B12 deficiency?

-Whats test called to Dx this cause?

A
  • pernicious anemia

- Schilling test

73
Q

Pancreatic insufficiency

-can lead to what anemia?

A
  • B12 deficiency megaloblastic anemia

- need pancreatic proteases to cleave R-binder from B12.

74
Q

Orotic aciduria

  • can lead to what anemia?
  • what enzyme is defective?
A

-megaloblastic anemia
-UMP synthase (pyrimidine synth).
-

75
Q

Nonmegaloblastic macrocytic anemias

-causes?

A
  • liver disease; alcoholism; reticulocytosis => inc.Ž  MCV

- drugs (5-FU, zidovudine, hydroxyurea).

76
Q

Normocytic, normochromic anemia

-what are the 2 classes?

A

hemolytic & non-hemolytic

77
Q

Intravascular hemolysis

-haptoglobin, inc or dec?

A

-dec

78
Q

Extravascular hemolysis

  • where is hemolysis taking place?
  • what type of cells will you see in smear?
A
  • Spleen via splenic macrophages

- spherocytes

79
Q

Extravascular hemolysis

-which bilirubin will be inc?

A

-unconjugated bilirubin inc = can cause jaundice.

80
Q

Which 2 vitamins does breast milk not have adequate amounts of?

A
  • vitamin K and D.

- must give vitamin K when baby is born or could get hemorrhagic disease of newborn.

81
Q

henoch schonlein purpura

-Sxs:

A

IgA vasculitis

  • palpable purpura
  • arthralgias
  • GI probs: melena, colicky pain
  • IgA nephropathy
82
Q

Warfarin or heparin

  • which can be used in pregnancy?
  • mnemonic?
A
  • heparin can be used in pregnancy, does NOT cross placenta.

- Do not wage WAR on baby. Keep is HEPpy w/heparin.

83
Q

Tenderness of calf to dorsiflexion = sign of what?

A
  • DVT

- Homan’s sign.

84
Q

LMWH vs unfractinoated heparin

-difference?

A

LMWH has more activity against factor Xa.

85
Q

Sickling test for sickle cell anemia

A

-sodium metabisulfite added should make the cells sickle if you have sickle cell.

86
Q

Sickle cell trait

-whats the only Sx they usually get?

A

hematuria

87
Q

Bleeding from venous puncture sites is highly suggestive of what?

A

DIC

88
Q
spur cells (acanthocytes)
-usually seen in which disease?
A

abetalipoproteinemia

89
Q

Fatigue & heart palpitations (& inc. CO)

-characteristic of which anemias?

A

common to all anemias.

90
Q

Pancytopenia w/o spenomegaly

-DDx:

A
  • severe B12/folate def.
  • aleukemic leukemia
  • myelodysplastic syndrome
91
Q

aplastic anemia

-whats the bone marrow look like?

A

hypocellular filled w/fat cells and fibrotic stroma

92
Q

Warfarin

-prolongs PT or PTT more?

A

-PT

93
Q

Heparin

-prolongs PT or PTT more?

A

-PTT

94
Q

Which pro-coag protein is not made in the liver?

A

vWF

-made in endothelial cells.

95
Q

hypercoag, hemolytic anemia, & pancytopenia

-this triad points toward what disease?

A

paroxysmal nocturnal hematuria

96
Q

DIC

  • fibrinogen levels?
  • PT?
  • factor 5?
  • factor 8?
A
  • fibrinogen = dec.
  • PT = increased
  • 5 & 8 = decreased
97
Q

Whats monitored in a pt to Dx DIC?

A

serum fibrinogen

98
Q

What does a reticulocyte look like?

A

Slightly larger and more blue than mature RBC.

-retains some rRNA which appears blue.

99
Q

Diphyllobothrium latum

-can cause what anemia?

A

B12 def. megaloblastic anemia.

100
Q

ACD

  • serum iron?
  • ferritin
  • TIBC
A
  • iron = low (has to be)
  • ferritin = high
  • TIBC = dec
101
Q

What does hepcidin bind?

A

ferroportin on enterocytes & macrophages

102
Q

What does hepcidin bind?

A

ferroportin on enterocytes & macrophages

-dec. release of iron from macrophages.

103
Q

Aplastic anemia

-which chemicals/drugs can cause it?

A

-benzene, chloramphenicol, alkylating agents, antimetabolites.

104
Q

Aplastic anemia

-which viruses can cause it?

A

-parvovirus B19, EBV, HIV, HCV.

105
Q

Fanconi anemia

  • what is it?
  • which anemia can it cause?
A

DNA repair defect

-aplastic anemia

106
Q

Aplastic anemia

-Tx:

A

-RBC and platelet transfusion, G-CSF, or GM-CSF.

107
Q

aplastic anemia

-is splenomegaly present?

A

NO

108
Q

If you have normal bilirubin levels, what can you rule out?

A

hemolysis

109
Q

Pts w/hemolytic anemia are predisposed what deficiency?

A

Pts w/hemolytic anemia are predisposed to folate deficiency b/c of inc. RBC turnover.
-Iron is effectively recycled & we have a lot of B12 stores.

110
Q

Which proteins can be defective in hereditary spherocytosis?

A

ankyrin, band 3, protein 4.2, spectrin

111
Q

hereditary spherocytosis

  • MCHC: inc or dec?
  • RDW: inc or dec?
  • splenomegaly?
A
  • inc. MCHC, 
  • inc. red cell distribution width
  • yes, splenomegaly.
112
Q
  • osmotic fragility test (+) .

- Eosin-5-maleimide binding test useful for screening.

A

hereditary spherocytosis

113
Q

hereditary spherocytosis

-Tx:

A

splenectomy

114
Q

Most common enzymatic disorder of RBCs?

A

G6PD deficiency

115
Q

G6PD deficiency

  • presentation?
  • possible triggers?
A
  • Back pain, hemoglobinuria a few days after oxidant stress.
  • sulfa drugs, antimalarials, infections, fava beans, TMP-SMX (link btwn UTI & G6PD def.)

*Hb = nephrotoxic & gives you back (kidney) pain.

116
Q

Heinz body

-what is it?

A
  • oxidized Hb

- G6PD deficiency

117
Q

N-MYC

-associated w/which cancers?

A
  • neuroblastoma

- small cell carcinoma of lung

118
Q

Where in the LN do B-cells proliferate & isotype switch?

A

Cortex

-thats where germinal centers are.

119
Q

argatroban, bivalirudin

A
  • directly inhibit thrombin.
  • derivative of hirudin (used by leeches).
  • used instead of herparin in pts w/HIT (heparin induced thrombocytopenia).
120
Q

Whats the most common cause of thrombocytopenia in hospitalized pts?

A

Heparin (esp. unfractionated heparin).

-HIT => paradoxical thrombosis rather than bleeding.

121
Q

cilostazol, dipyridamole

A

PDE 3 inhibitor

  • inc. cAMP in platelets, thus inhibiting platelet aggregation.
  • also vasodilates.
122
Q

Which leukemia has similar presentation to leukamoid reaction?

A

CML

-except in CML the L-ALP (leukocyte alkaline phosphatase) will be low.

123
Q

G6PD def.

-inheritance?

A

X-linked recessive

124
Q

-Most structural abnormalities = what inheritance?

A
  • auto dom

- ie. hereditary spherocytosis

125
Q

-Most enzymes deficiencies = what inheritance?

A

-auto recessive

126
Q

Name an X-linked dominant disease

A

vitamin D resistant rickets

127
Q

what drives extramedullary hematopoiesis?

A

Lots of EPO!

-most often caused by severe chronic hemolytic anemias (ie. beta-thal).

128
Q

“crew cut” skull & chipmunk facies

  • due to what?
  • see in what?
A

marrow expansion/extra medullary hematopoiesis.

-diseases w/chronic anemia like beta-thal.

129
Q

Bleeding after tooth extraction

-sign of what?

A

Hemophilia

-this is NOT a platelet problem, its a coagulation problem.

130
Q

B12 and folate deficiency

-how can these lead to inc risk of thrombosis?

A

-both inc. homocysteine levels.