04a: Heme Flashcards
1
Q
Erythrocyte membrane contains which key transporter?
A
Cl/HCO3 antiporter
2
Q
Reticulocytes are (X) color on Wright-Giemsa stain. What gives them this color?
A
X = blue
Residual rRNA
3
Q
List the types of granules present in platelet as well as the contents of each
A
- Dense granule (ADP, Ca)
2. Alpha granule (vWF, fibrinogen, fibronectin)
4
Q
Petechiae is a sign that platelets are:
A
Either low (thrombocytopenia) or have decreased function
5
Q
vWF receptor:
A
Gp1b
6
Q
Fibrinogen receptor:
A
GpIIb/IIIa
7
Q
Neutrophil specific granules contain:
A
“CLL-LAP specifically for the neutropils”
- Collagenase
- Lactoferrin
- Lysozyme
- LAP (Leukocyte alk phos)
8
Q
Neutrophil azurophilic granules contain:
A
- Proteinases
- Acid phosphatases
- Myeloperoxidase
- Beta-glucoronidase
9
Q
List the key chemotactic agents for neutrophils
A
- IL8
- C5a
- LTB4
Also: kallikrein, platelet-activating factor, and 5-HETE
10
Q
Macrophages are activated by (X). They can present antigen via (Y) receptor.
A
X = IFN-gamma Y = MHC II
11
Q
Septic shock: initiated when (X) part of bacterial LPS binds (Y) receptor on which WBC?
A
X = Lipid A Y = CD14
Macrophages
12
Q
T/F: Basophils mediate allergic reaction
A
True
13
Q
Basophilia is uncommon, but can be a sign of:
A
Myeloproliferative disease (esp CML)
14
Q
Basophil granules contain:
A
Heparin and His
can also synthesize leukotrienes on demand
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)
16
Q
Degranulation of mast cell releases:
A
His, Heparin, tryptase, and eosinophil chemotactic factors
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
18
Q
(X) is used to prevent mast cell degranulation in asthma prophylaxis.
A
X = Cromolyn sodium
Note: not effective for treatment
19
Q
Dendritic cells express (X) receptor(s) on surface.
A
X = MHC II and Fc
20
Q
Tennis-racquet-shaped inclusions are characteristic of (X) skin cells.
A
X = Langerhans (dendritic)
“You need Langer (longer) hands to play tennis”
21
Q
T/F: B cells are born and mature in bone marrow
A
True
22
Q
B cells can function as APC via (X) receptor
A
X = MHC II
23
Q
(X) co-stimulatory signal is necessary for T cell activation.
A
X = CD28
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
25
Hemoglobin electrophoresis: list the various Hgb types (F, A, S, and C) in order of decreasing distance traveled on gel, toward (pos/neg) electrode.
Travel toward positively-charged anode
A (farthest) - Glu (-)
F
S - Glu to Val (neutral)
C - Glu to Lys (+)
"A Fat Santa Claus"
26
(X) activates bradykinin and (Y) inactivates it
```
X = kallikrein
Y = ACE
```
27
T/F: Bradykinin plays role in increasing pain and vasoconstriction.
False - increases pain, vasodilation, and permeability
28
Which cofactor is part of the conversion of pro-thrombin to thrombin by (X)?
X = Factor Xa
| Factor Va
29
Anticoagulants: med with highest efficacy in targeting Factor IIa is (X) and in targeting Factor Xa is (Y).
```
X = heparin (IIa is thrombin)
Y = LMWH
```
30
T/F: Warfarin directly inhibits enzyme gamma-glutamyl carboxylase, which activates factors 2, 7, 9, 10, C, S.
False - warfarin is vit K analog that inhibits vit K epoxide reductase; reduced vit K needed as cofactor for gamma-glutamyl carboxylase
31
Protein C is activated by (X) and then uses (Y) for which function?
```
X = thrombin-thrombomodulin complex (endothelial cells)
Y = Protein S
```
Cleave/inactivate Va and VIIIa factors
32
Endothelial damage and exposure of (X) causes the very initial binding of (Y) in platelet plug formation/primary hemostasis.
```
X = collagen
Y = vWF (from alpha granules of platelets and Wiebel-Palade bodies)
```
33
P2Y12 is a receptor for (X) on (Y) cell in platelet plug formation.
```
X = ADP (released from platelets)
Y = platelet
```
34
P2Y12 inhibition by (X) drug will cause (increase/decrease) in (Y) expression.
X = clopidogrel (or prasugrel, ticlopidine)
Decrease
Y = Gp2b/3a (on platelet surface)
35
A negative ristocetin test that is not correcting by addition of normal plasma to pt plasma is indicative of (X) disease.
X = bernard-soulier (Gp1b R deficiency)
36
In (X) disease, the defect is with the same step/interaction as that which is inhibited by Abciximab.
X = Glanzmann thombasthenia
Deficiency in Gp2b/3a (platelet can't bind fibrinogen)
"GLANZing at those 23 y.o.'s ABs"
37
Acanthocyte, aka (X) cell, is associated with which pathology?
X = spur ("acantho = spiny") RBC
Liver disease, abetalipoproteinemia
38
Difference between basophilic stippling of RBC and sideroblast.
SideroBLAST is in bone marrow (basophilic stippling in peripheral smear)
39
Basophilic stippling of RBCs associated with which pathology?
Lead poisoning, sideroblastic anemia, myelodysplastic syndromes
40
Dacrocyte aka tear-shaped RBC is seen in which pathology?
Bone marrow infiltration (myelofibrosis) - RBC mechanically squeezed out of marrow gives characteristic shape
41
Echinocyte (aka Burr cell) is associated with which pathology?
1. ESRD or liver disease
2. Pyruvate kinase deficiency
3. Traumatic hemolysis like passing through prosthetic valve (esp if seen with helmet cells)
42
Helmet cell, aka (X), is associated with which pathology?
X = schistocyte (fragmented RBC)
1. Mechanical hemolysis
2. MAHA (DIC, TTP/HUS, HELLP)
43
"Target cell" associated with which pathology?
"HALT said the hunter to his target"
1. HbC
2. Asplenia
3. Liver disease
4. Thalassemia
44
(X) bodies seen in RBCs are precipitated Hb
X = heinz (seen in G6PD def)
Hb oxidized (S-S bond) and precipitates
45
Why would a sickle cell disease patient have high MCV?
Folate deficiency (requirement is increased in these patients due to high RBC turnover)
46
List the etiologies for a macrocytic, NON-megaloblastic anemia.
Liver disease, alcoholism, Diamond-Blackfan anemia
47
List the etiologies for a macrocytic, megaloblastic anemia.
Folate and B12 deficiency, orotic aciduria
48
Autoimmune causes of anemia will be (macro/micro/normo)-cytic.
Normocytic (extravascular hemolysis)
49
Corrected reticulocyte count equation:
% reticulocytes* (pt hematocrit/normal hematocrit)
50
Plummer-Vinson triad:
"The plumber WADs in toilet water"
1. Web (esophageal webs)
2. Anemia (Fe def)
3. Dysphagia
51
Hemoglobin Barts is composed of which chains? What's the consequence of this Hb?
4 gamma chains (when there is 4 allele deletion of alpha-globin)
Hydrops fetalis (incompatible with life)
52
HbH disease, with excess HbH which is composed of (X) chains, is a result of:
X = 4 beta chains
3 alpha globin chain deletion
53
"Crew cut" on skull xray is a result of (X) and associated with which disease?
X = marrow expansion
Beta-thalassemia, SCD
54
Hepatosplenomegaly in beta-thalassemia patient is a result of:
Extramedullary (liver, spleen) hematopoiesis (due to high EPO from chronic hemolysis)
55
Lead poisoning: which enzymes inhibitied?
ALA dehydratase and ferrochelatase
56
Lead toxicity: first-line Rx
Dimercaprol and EDTA
| Succimer for chelation for kids
57
Causes of sideroblastic anemia:
1. Genetic (ALA synthase defect)
2. Acquried (myelodysplastic syndrome)
3. Reversible (EtOH most common; isoniazid, lead, vit B6 def, Cu def)
58
B12 deficiency: (low/high) homocysteine and (low/high) methylmalonic acid.
Both high
59
Orotic aciduria is due to defect in (X) that presents with which classical Sx? What's the treatment?
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
Anemia of chronic disease: what's the instigating factor?
Inflammation (which increases hepcidin and decreases iron release from macrophages)
61
Anemia of chronic disease: (low/high) hepcidin, (low/high) transferrin, (low/high) serum Fe, (low/high) ferritin.
High; low, low; high (stored in macrophages)
62
Impaired synthesis of CD55 and CD59 in patient with venous thrombus. Diagnosis? Treatment?
Paroxysmal nocturnal hemoglobinuria
Eculizumab (terminal complement inhibitor)
63
Septic shock/bacteremia in a sickle cell disease patient with functional asplenia.
S. pneumo
| H. flu is #2
64
Direct coombs is used to detect/measure:
Presence of Ig DIRECTLY ON pt RBC membrane (so pt RBCs will agglutinate if anti-Ig Coombs reagent added)
65
Inirect coombs is used to detect/measure:
Presence of Ig in patient SERUM (added RBCs will agglutinate in pt serum if anti-Ig Coombs reagent is added)
66
Primary disturbance regarding Fe balance in pregnancy is (increased/decreased) (X).
Increased
| X = transferrin/TIBC (with unchanged serum iron and ferritin)
67
(X) drugs cause lymphopenia and eosinopenia but neutrophilia. Why?
X = corticosteroids
Decrease activation of neutrophil adhesion molecules (impairs migration out of vasculature to inflamm site)
68
List some genetic causes of lymphopenia.
1. DiGeorge
| 2. SCID
69
If prescriptions not handled carefully, an HIV patient on Zidovudine with concomitant CMV infection runs the risk of which side effect?
Neutropenia (if prescribed Ganciclovir or TMP-SMX antimicrobials)
70
Acute Intermittent Porphyria: (X) inheritance pattern. What are the classic symptoms?
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
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?
Acute intermittent porphyria
High PBG levels in urine are oxidized upon prolonged exposure to light/air
72
Why would patient with (X) defects in heme synthesis avoid alcohol/tobacco?
X = AIP or PCT
Exacerbate Sx due to CYP inducing properties (which increases ALA synthase activity)
73
Heme synthesis: Why is photosensitivity a feature of (AIP/PCT) but not (AIP/PCT)?
PCT; AIP
Accumulation of porphyrinogens in PCT
74
Rx for Acute Intermittent Porphyria
Dextrose/glucose and heme (inhibit ALA synthase)
75
T/F: Warfarin toxicity will cause prolonged PT only.
False - prolonged PT and PTT (def of vit k dependent factors); normal bleeding time
76
Immune thrombocytopenia: Ab against (X). What's the classic marrow biopsy finding and Rx regimen?
X = Gp2b/3a receptor
Biopsy: Lots of megakaryocytes
Rx: steroids, IVIG; splenectomy if refractory disease
77
Pathogenesis of Thrombotic thrombocytopenic purpura (TTP)
ADAMTS-13 (vWF metalloprotease) deficiency causes large vWF multimers that increases platelet adhesion, aggregation, thrombosis
78
Which Sx of TTP are essential for diagnosis?
Thrombocytopenia and MAHA;
Other Sx: neuro, renal, and fever
79
Initial treatment for TTP versus initial treatment for hemolytic uremic syndrome (HUS)
BOTH plasmapharesis initially;
TTP Rx can also involve steroids and rituximab
80
T/F: FFP can be used to reverse both Warfarin and Heparin effects.
False - not heparin (since FFP contains Antithrombin III)
81
Cryoprecipitate contains only (X) proteins.
X = cold-soluble
| Fibrinogen, fibronectin, F VIII, XIII, vWF
82
T/F: Blood transfusion risks include hypercalcemia.
False - hypocalcemia (since citrate is Ca chelator)
83
T/F: Hodgkin's lymphoma has better prognosis than NHL
True
84
Hodgkin lymphoma associated with (X) diseases and NHL with (Y) diseases
```
X = EBV
Y = HIV, autoimmune
```
85
Reed sternberg cells are CD(X) positive (T/B) cells..
X = 15, 30
B-cells
(15x2 owl eyes = 30)
86
Burkitt lymphoma translocation involves:
c-myc (chrom 8) and Ig heavy chain (chrom 14)
87
Burkitt lymphoma high mitotic index characterized by (low/high) (X) fraction.
High
| X = Ki-67 (99%)
88
Which NHLs are associated with EBV?
1. Burkitt
| 2. Primary CNS lymphoma (second most common cause of ring-enhancing lesions w mass effect in HIV pts)
89
Most common cause of NHL in adults
Diffuse large B cell lymphoma
90
Follicular lymphoma associated with which translocation?
t(14;18) = Ig heavy chain (chrom 14) with BCL2 (chrom 18)
91
Painless, waxing and waning lymphadenopathy associated with (X) lymphoma
X = Follicular
92
Mantle cell lymphoma translocation:
t(11;14) - cyclin D (chrom 11) and Ig heavy chain (chrom 14)
93
Which lymphoma presents similarly to toxoplasmosis and must be distinguished from it?
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
Adult T cell lymphoma is caused by (X) and presents with:
X = HTLV (human T lymph virus); IV drug use
Cutaneous lesions, lytic bone lesions/hypercalcemia
95
Mycosis fungoides is a(n) (X) disease that presents with (Y) and may progress to (Z).
```
X = mature T cell neoplasm
Y = skin patches/plaques (with atypical CD4 and neoplastic cells)
Z = Sézary syndrome (T-cell leukemia)
```
96
Key symptoms in multiple myeloma
"CRAB"
1. HyperCalcemia (constipation)
2. Renal failure
3. Anemia
4. Bone lytic lesions/back pain
97
Glassy Ig cast nephropathy is seen in (X) disease. These casts form as a result of:
X = multiple myeloma
High excretion of free light chains aka Bence Jones proteins (exceed absorption capacity; precipitate and form casts)
98
Presence of t(12;21) in (X) leukemia is associated with (better/worse) prognosis.
X = ALL
| Better
99
AML: auer rods stain positive for:
Myeloperoxidase
100
Which leukemias associated with Down syndrome?
AML and ALL
