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STEP 1 > 04a: Heme > Flashcards

04a: Heme Flashcards

1
Q

Erythrocyte membrane contains which key transporter?

A

Cl/HCO3 antiporter

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2
Q

Reticulocytes are (X) color on Wright-Giemsa stain. What gives them this color?

A

X = blue

Residual rRNA

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3
Q

List the types of granules present in platelet as well as the contents of each

A
  1. Dense granule (ADP, Ca)

2. Alpha granule (vWF, fibrinogen, fibronectin)

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4
Q

Petechiae is a sign that platelets are:

A

Either low (thrombocytopenia) or have decreased function

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5
Q

vWF receptor:

A

Gp1b

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6
Q

Fibrinogen receptor:

A

GpIIb/IIIa

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7
Q

Neutrophil specific granules contain:

A

“CLL-LAP specifically for the neutropils”

  1. Collagenase
  2. Lactoferrin
  3. Lysozyme
  4. LAP (Leukocyte alk phos)
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8
Q

Neutrophil azurophilic granules contain:

A
  1. Proteinases
  2. Acid phosphatases
  3. Myeloperoxidase
  4. Beta-glucoronidase
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9
Q

List the key chemotactic agents for neutrophils

A
  1. IL8
  2. C5a
  3. LTB4

Also: kallikrein, platelet-activating factor, and 5-HETE

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10
Q

Macrophages are activated by (X). They can present antigen via (Y) receptor.

A 
X = IFN-gamma
Y = MHC II
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11
Q

Septic shock: initiated when (X) part of bacterial LPS binds (Y) receptor on which WBC?

A 
X = Lipid A
Y = CD14

Macrophages

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12
Q

T/F: Basophils mediate allergic reaction

A

True

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13
Q

Basophilia is uncommon, but can be a sign of:

A

Myeloproliferative disease (esp CML)

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14
Q

Basophil granules contain:

A

Heparin and His

can also synthesize leukotrienes on demand

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15
Q

Mast cells originate from same precursor as (X). Its membrane can bind the (Fc/Fab) portion of Ig(G/A/E/M).

A

X = basophils
Fc
IgE (via weak, non-covalent bonds)

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16
Q

Degranulation of mast cell releases:

A

His, Heparin, tryptase, and eosinophil chemotactic factors

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17
Q

High levels of tryptase, which is released from (X) cell, is sometimes used to support a diagnosis of (Y).

A 
X = mast 
Y = anaphylaxis
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18
Q

(X) is used to prevent mast cell degranulation in asthma prophylaxis.

A

X = Cromolyn sodium

Note: not effective for treatment

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19
Q

Dendritic cells express (X) receptor(s) on surface.

A

X = MHC II and Fc

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20
Q

Tennis-racquet-shaped inclusions are characteristic of (X) skin cells.

A

X = Langerhans (dendritic)

“You need Langer (longer) hands to play tennis”

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21
Q

T/F: B cells are born and mature in bone marrow

A

True

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22
Q

B cells can function as APC via (X) receptor

A

X = MHC II

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23
Q

(X) co-stimulatory signal is necessary for T cell activation.

A

X = CD28

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24
Q

T/F: Plasma cells are almost never found in bone marrow

A

False - stay in marrow and normally don’t circulate in peripheral blood

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25
Q

Hemoglobin electrophoresis: list the various Hgb types (F, A, S, and C) in order of decreasing distance traveled on gel, toward (pos/neg) electrode.

A

Travel toward positively-charged anode

A (farthest) - Glu (-)
F
S - Glu to Val (neutral)
C - Glu to Lys (+)

“A Fat Santa Claus”

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26
Q

(X) activates bradykinin and (Y) inactivates it

A 
X = kallikrein
Y = ACE
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27
Q

T/F: Bradykinin plays role in increasing pain and vasoconstriction.

A

False - increases pain, vasodilation, and permeability

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28
Q

Which cofactor is part of the conversion of pro-thrombin to thrombin by (X)?

A

X = Factor Xa

Factor Va

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29
Q

Anticoagulants: med with highest efficacy in targeting Factor IIa is (X) and in targeting Factor Xa is (Y).

A 
X = heparin (IIa is thrombin)
Y = LMWH
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30
Q

T/F: Warfarin directly inhibits enzyme gamma-glutamyl carboxylase, which activates factors 2, 7, 9, 10, C, S.

A

False - warfarin is vit K analog that inhibits vit K epoxide reductase; reduced vit K needed as cofactor for gamma-glutamyl carboxylase

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31
Q

Protein C is activated by (X) and then uses (Y) for which function?

A 
X = thrombin-thrombomodulin complex (endothelial cells)
Y = Protein S

Cleave/inactivate Va and VIIIa factors

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32
Q

Endothelial damage and exposure of (X) causes the very initial binding of (Y) in platelet plug formation/primary hemostasis.

A 
X = collagen
Y = vWF (from alpha granules of platelets and Wiebel-Palade bodies)
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33
Q

P2Y12 is a receptor for (X) on (Y) cell in platelet plug formation.

A 
X = ADP (released from platelets)
Y = platelet
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34
Q

P2Y12 inhibition by (X) drug will cause (increase/decrease) in (Y) expression.

A

X = clopidogrel (or prasugrel, ticlopidine)
Decrease
Y = Gp2b/3a (on platelet surface)

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35
Q

A negative ristocetin test that is not correcting by addition of normal plasma to pt plasma is indicative of (X) disease.

A

X = bernard-soulier (Gp1b R deficiency)

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36
Q

In (X) disease, the defect is with the same step/interaction as that which is inhibited by Abciximab.

A

X = Glanzmann thombasthenia

Deficiency in Gp2b/3a (platelet can’t bind fibrinogen)

“GLANZing at those 23 y.o.’s ABs”

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37
Q

Acanthocyte, aka (X) cell, is associated with which pathology?

A

X = spur (“acantho = spiny”) RBC

Liver disease, abetalipoproteinemia

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38
Q

Difference between basophilic stippling of RBC and sideroblast.

A

SideroBLAST is in bone marrow (basophilic stippling in peripheral smear)

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39
Q

Basophilic stippling of RBCs associated with which pathology?

A

Lead poisoning, sideroblastic anemia, myelodysplastic syndromes

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40
Q

Dacrocyte aka tear-shaped RBC is seen in which pathology?

A

Bone marrow infiltration (myelofibrosis) - RBC mechanically squeezed out of marrow gives characteristic shape

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41
Q

Echinocyte (aka Burr cell) is associated with which pathology?

A
  1. ESRD or liver disease
  2. Pyruvate kinase deficiency
  3. Traumatic hemolysis like passing through prosthetic valve (esp if seen with helmet cells)
42
Q

Helmet cell, aka (X), is associated with which pathology?

A

X = schistocyte (fragmented RBC)

  1. Mechanical hemolysis
  2. MAHA (DIC, TTP/HUS, HELLP)
43
Q

“Target cell” associated with which pathology?

A

“HALT said the hunter to his target”

  1. HbC
  2. Asplenia
  3. Liver disease
  4. Thalassemia
44
Q

(X) bodies seen in RBCs are precipitated Hb

A

X = heinz (seen in G6PD def)

Hb oxidized (S-S bond) and precipitates

45
Q

Why would a sickle cell disease patient have high MCV?

A

Folate deficiency (requirement is increased in these patients due to high RBC turnover)

46
Q

List the etiologies for a macrocytic, NON-megaloblastic anemia.

A

Liver disease, alcoholism, Diamond-Blackfan anemia

47
Q

List the etiologies for a macrocytic, megaloblastic anemia.

A

Folate and B12 deficiency, orotic aciduria

48
Q

Autoimmune causes of anemia will be (macro/micro/normo)-cytic.

A

Normocytic (extravascular hemolysis)

49
Q

Corrected reticulocyte count equation:

A

% reticulocytes* (pt hematocrit/normal hematocrit)

50
Q

Plummer-Vinson triad:

A

“The plumber WADs in toilet water”

  1. Web (esophageal webs)
  2. Anemia (Fe def)
  3. Dysphagia
51
Q

Hemoglobin Barts is composed of which chains? What’s the consequence of this Hb?

A

4 gamma chains (when there is 4 allele deletion of alpha-globin)

Hydrops fetalis (incompatible with life)

52
Q

HbH disease, with excess HbH which is composed of (X) chains, is a result of:

A

X = 4 beta chains

3 alpha globin chain deletion

53
Q

“Crew cut” on skull xray is a result of (X) and associated with which disease?

A

X = marrow expansion

Beta-thalassemia, SCD

54
Q

Hepatosplenomegaly in beta-thalassemia patient is a result of:

A

Extramedullary (liver, spleen) hematopoiesis (due to high EPO from chronic hemolysis)

55
Q

Lead poisoning: which enzymes inhibitied?

A

ALA dehydratase and ferrochelatase

56
Q

Lead toxicity: first-line Rx

A

Dimercaprol and EDTA

Succimer for chelation for kids

57
Q

Causes of sideroblastic anemia:

A
  1. Genetic (ALA synthase defect)
  2. Acquried (myelodysplastic syndrome)
  3. Reversible (EtOH most common; isoniazid, lead, vit B6 def, Cu def)
58
Q

B12 deficiency: (low/high) homocysteine and (low/high) methylmalonic acid.

A

Both high

59
Q

Orotic aciduria is due to defect in (X) that presents with which classical Sx? What’s the treatment?

A

X = UMP synthase (inability to convert orotic acid to UMP)

Kid failure to thrive/dev delay, megaloblastic anemia that’s refractory to B12/folate; NO hyperammonemia

Rx: UMP

60
Q

Anemia of chronic disease: what’s the instigating factor?

A

Inflammation (which increases hepcidin and decreases iron release from macrophages)

61
Q

Anemia of chronic disease: (low/high) hepcidin, (low/high) transferrin, (low/high) serum Fe, (low/high) ferritin.

A

High; low, low; high (stored in macrophages)

62
Q

Impaired synthesis of CD55 and CD59 in patient with venous thrombus. Diagnosis? Treatment?

A

Paroxysmal nocturnal hemoglobinuria

Eculizumab (terminal complement inhibitor)

63
Q

Septic shock/bacteremia in a sickle cell disease patient with functional asplenia.

A

S. pneumo

H. flu is #2

64
Q

Direct coombs is used to detect/measure:

A

Presence of Ig DIRECTLY ON pt RBC membrane (so pt RBCs will agglutinate if anti-Ig Coombs reagent added)

65
Q

Inirect coombs is used to detect/measure:

A

Presence of Ig in patient SERUM (added RBCs will agglutinate in pt serum if anti-Ig Coombs reagent is added)

66
Q

Primary disturbance regarding Fe balance in pregnancy is (increased/decreased) (X).

A

Increased

X = transferrin/TIBC (with unchanged serum iron and ferritin)

67
Q

(X) drugs cause lymphopenia and eosinopenia but neutrophilia. Why?

A

X = corticosteroids

Decrease activation of neutrophil adhesion molecules (impairs migration out of vasculature to inflamm site)

68
Q

List some genetic causes of lymphopenia.

A
  1. DiGeorge

2. SCID

69
Q

If prescriptions not handled carefully, an HIV patient on Zidovudine with concomitant CMV infection runs the risk of which side effect?

A

Neutropenia (if prescribed Ganciclovir or TMP-SMX antimicrobials)

70
Q

Acute Intermittent Porphyria: (X) inheritance pattern. What are the classic symptoms?

A

X = AD (mutation in PBG deaminase; accumulation of PBG and ALA)

4 P’s for Porphyria!

  1. Painful abdomen
  2. Port-wine colored urine (darkens on prolonged exposure)
  3. Polyneuropathy/Psych (confusion)
  4. P450 inducers (esp EtOH, tobacco) precipitate Sx
71
Q

You run a urinalysis on patient with abdominal pain and confusion. Urine turns a darker, red color after sitting for 24 hours. What’s the diagnosis and mechanism behind this finding?

A

Acute intermittent porphyria

High PBG levels in urine are oxidized upon prolonged exposure to light/air

72
Q

Why would patient with (X) defects in heme synthesis avoid alcohol/tobacco?

A

X = AIP or PCT

Exacerbate Sx due to CYP inducing properties (which increases ALA synthase activity)

73
Q

Heme synthesis: Why is photosensitivity a feature of (AIP/PCT) but not (AIP/PCT)?

A

PCT; AIP

Accumulation of porphyrinogens in PCT

74
Q

Rx for Acute Intermittent Porphyria

A

Dextrose/glucose and heme (inhibit ALA synthase)

75
Q

T/F: Warfarin toxicity will cause prolonged PT only.

A

False - prolonged PT and PTT (def of vit k dependent factors); normal bleeding time

76
Q

Immune thrombocytopenia: Ab against (X). What’s the classic marrow biopsy finding and Rx regimen?

A

X = Gp2b/3a receptor

Biopsy: Lots of megakaryocytes

Rx: steroids, IVIG; splenectomy if refractory disease

77
Q

Pathogenesis of Thrombotic thrombocytopenic purpura (TTP)

A

ADAMTS-13 (vWF metalloprotease) deficiency causes large vWF multimers that increases platelet adhesion, aggregation, thrombosis

78
Q

Which Sx of TTP are essential for diagnosis?

A

Thrombocytopenia and MAHA;

Other Sx: neuro, renal, and fever

79
Q

Initial treatment for TTP versus initial treatment for hemolytic uremic syndrome (HUS)

A

BOTH plasmapharesis initially;

TTP Rx can also involve steroids and rituximab

80
Q

T/F: FFP can be used to reverse both Warfarin and Heparin effects.

A

False - not heparin (since FFP contains Antithrombin III)

81
Q

Cryoprecipitate contains only (X) proteins.

A

X = cold-soluble

Fibrinogen, fibronectin, F VIII, XIII, vWF

82
Q

T/F: Blood transfusion risks include hypercalcemia.

A

False - hypocalcemia (since citrate is Ca chelator)

83
Q

T/F: Hodgkin’s lymphoma has better prognosis than NHL

A

True

84
Q

Hodgkin lymphoma associated with (X) diseases and NHL with (Y) diseases

A 
X = EBV
Y = HIV, autoimmune
85
Q

Reed sternberg cells are CD(X) positive (T/B) cells..

A

X = 15, 30
B-cells

(15x2 owl eyes = 30)

86
Q

Burkitt lymphoma translocation involves:

A

c-myc (chrom 8) and Ig heavy chain (chrom 14)

87
Q

Burkitt lymphoma high mitotic index characterized by (low/high) (X) fraction.

A

High

X = Ki-67 (99%)

88
Q

Which NHLs are associated with EBV?

A
  1. Burkitt

2. Primary CNS lymphoma (second most common cause of ring-enhancing lesions w mass effect in HIV pts)

89
Q

Most common cause of NHL in adults

A

Diffuse large B cell lymphoma

90
Q

Follicular lymphoma associated with which translocation?

A

t(14;18) = Ig heavy chain (chrom 14) with BCL2 (chrom 18)

91
Q

Painless, waxing and waning lymphadenopathy associated with (X) lymphoma

A

X = Follicular

92
Q

Mantle cell lymphoma translocation:

A

t(11;14) - cyclin D (chrom 11) and Ig heavy chain (chrom 14)

93
Q

Which lymphoma presents similarly to toxoplasmosis and must be distinguished from it?

A

Primary CNS lymphoma (a NHL; EBV infection) seen in AIDs and presenting with ring-enhancing lesion (plus mass effect like confusion, memory loss, seizures)

94
Q

Adult T cell lymphoma is caused by (X) and presents with:

A

X = HTLV (human T lymph virus); IV drug use

Cutaneous lesions, lytic bone lesions/hypercalcemia

95
Q

Mycosis fungoides is a(n) (X) disease that presents with (Y) and may progress to (Z).

A 
X = mature T cell neoplasm
Y = skin patches/plaques (with atypical CD4 and neoplastic cells)
Z = Sézary syndrome (T-cell leukemia)
96
Q

Key symptoms in multiple myeloma

A

“CRAB”

  1. HyperCalcemia (constipation)
  2. Renal failure
  3. Anemia
  4. Bone lytic lesions/back pain
97
Q

Glassy Ig cast nephropathy is seen in (X) disease. These casts form as a result of:

A

X = multiple myeloma

High excretion of free light chains aka Bence Jones proteins (exceed absorption capacity; precipitate and form casts)

98
Q

Presence of t(12;21) in (X) leukemia is associated with (better/worse) prognosis.

A

X = ALL

Better

99
Q

AML: auer rods stain positive for:

A

Myeloperoxidase

100
Q

Which leukemias associated with Down syndrome?

A

AML and ALL

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