heme

Question 1

PMN chemotaxis

Answer 1

IL-8
LTB4
C5a
kallikrein
PAF

Question 2

eosinophil products

Answer 2

histaminASES

MBP

Question 3

Sphereocytes

Answer 3

ANY type of hemolytic anemia
- hereditary sphereocytosis
- AHA (cold or warm)
Also burns and old samples

Question 4

basophilic stippling is:

Answer 4

rRNA - lead prevents its degradation

Question 5

Overlapping metal Rxs

Answer 5

Dimercaprol and succimer

for lead, mercury, gold, arsenic

Question 6

causes of sideroblastic anemia

Answer 6

–| ala synthase

XR, lead, B6 deficiency (or INH), alcohol, copper deifciency

Question 7

kid with failure to thrive, developmental delays and B12/folate resistant megaloblastic anemia

Answer 7

Orotic aciduria
AR, no UMP synthase .: rx = UMP

NO hyperammonemia (OTC deficiency)

Question 8

ACD

Answer 8

increased hepcidin —| ferroportin (BL enterocytes + macrophages)

Question 9

aplastic anemia causes

*NO HSM!

Answer 9

drugs - benzene, chloramphenicol, chemo
radiation
viral - B19, EBV, HIV, HCV
Fanconi ANEMIA
idiopathic (immune or 1' SC defect); may follow acute hepatitis

Question 10

back pain with hemoglobinuria

Answer 10

G6PD

Hb is nephrotoxic

Question 11

hemolytic anemia in the newborn

Answer 11

pyruvate kinase deficiency

Question 12

PNH triad and labs and rx

Answer 12

Triad:

• coombs -ve H.A.
• pancytopenia (high up SC defect, can get AML)
• venous thrombosis

Lab = no CD55/59 on RBC flow

Rx = eculizumab (C5 inhibitor)

Question 13

transferrin levels in hemochromatosis

Answer 13

DECREASED (to try to lower it)

Question 14

batteries and ammunition exposure

Answer 14

lead poisoning

Question 15

AIP enzyme and s/s

Answer 15

porphobilinogen deaminase

5Ps (pain, port-wine, polyneuropathy, psychological, ppt drugs = 450 inducers, alcohol, starvation)

Question 16

PCT enzyme

Answer 16

uroprophyrinogen decarboxylase

Question 17

immune thrombocytopenia:
Abs
Ppt factor
rx

Answer 17

IgG Abs to GpIIb/IIIa
ppt by viral infection, SLE, HIV, HBC
Rx = steroid and IVIG for spleen to eat those

Question 18

TTP MOA and pentad:

Answer 18

MOA: ADAMTS problem; can’t chop up vWF so big multipliers super-activate platelets and you get clots

pentad: thrombocytopenia, microangiopathic H.A., neurologial, renal, fever

Question 19

prothrombin gene mutation

Answer 19

3’UTR point mutation causing INCREASED prothrombin production

Question 20

cyroppt

Answer 20

fibrinogen
vWF
VIII
XIII
Fibronectin

Question 21

high WBCs, low LAP

Answer 21

CML

Question 22

lymphoma with strong EBV associations

Answer 22

Hogkins (owl eyes, CD15 and 30)

non-hodgkins - endemic burkits t(8;14)

Question 23

Functions of the genes in cancer translocations:

• 8;14
• 14;18
• 11;14
• 9;22
• 15;17

Answer 23

• 8;14: 8 = c-myc = TC activator = burkitts
• 14;18: 18 = bcl-2 = blocks apoptosis = follicular
• 11;14: 11 = cyclin D1 = cell cycle regulator = mantle cell
• 9;22: 22 = abl = tyrosine kinase (cyto + nuc) = CML
• 15;17: 15 = RAR = myelocyte differentiation = PAML

Question 24

microsomal monooxygenase =

Answer 24

p450

Question 25

hematology drug causeing neutropenia presenting as fever and mouth ulcers

Answer 25

Ticlopidine (ADP-R blocker) use only if allergic to asprin + clopidogrel

Question 26

Features of anaplastic cells

Answer 26

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

Question 27

Rb-P means:

Answer 27

inactivated cell can progress thru cell cycle (G1 to S)

Question 28

AT-III deficiency labs, cause

Answer 28

NORMAL PT, PTT, TT Heparin deos NOT increase PTT AD or acquired (proteinuria)

Question 29

hodgkins subtypes

Answer 29

Nodular sclerosing: M=F, 70%, cervical or mediatinal, lacunae Lymphocyte risk: good Px Mixed or Lymphocyte depleted: bad Px

Question 30

painless waxing and waning LAD

Answer 30

follicular (NHL); 14;18 bcl-2

Question 31

lytic bone lesions, hypercalcemia, skin rash, generalized LAD with HSM IVDA, japan/caribbean/west africa

Answer 31

adult T-cell lymphoma (NHL); HTLV-1

Question 32

mycoses fungioides vs. sezary syndrome [T-cell NHLs] Histo

Answer 32

``` MF = cutaneous patches/plaques ("pautrier microabscesses"), LYMPHOMA SS = cutaneous and systemic, LEUKEMIA ``` Criberiform nuclei in atypical CD4+ cells

Question 33

MM vs MGUS vs WM

Answer 33

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)

Question 34

Pseudo-Pelger-Huet anomaly

Answer 34

bilobed PMNs, seen after chemo

Question 35

B-ALL: markers T-ALL: markers

Answer 35

B-ALL = tdt, CD10, CD19, CD20 T-ALL = tdt, CD2,3,4,5,7,8

Question 36

t(12;21)

Answer 36

good prognosis of ALL

Question 37

CD5+ B-cell cancers

Answer 37

NHL mantle cell | CLL/SLL

Question 38

smudge cells and AHA

Answer 38

CLL/SLL

Question 39

Cancer ages

Answer 39

ALL - 60 Hairy - adults (b-cell) AML - >65 (alk chemo, radiation, myeloprolif disorders, also downs); DIC CML - 45-85 (low LAP; blast crisis = ALL/AML)

Question 40

kid with skin rash, lytic bone lesions, recurrent otitis media with mastoid mass

Answer 40

Lanterns cell histiocytosis S-100 and CD1a Birbeck granules types: names = malignant; 2 = 3yrs (with DI and exophthalmos)

Question 41

ectopic EPO

Answer 41

RCC HCC hydroneophrosis

Question 42

HIT

Answer 42

IgG--------heparin-PF4

Question 43

AE ticlopidine

Answer 43

(ADP-R blocker) | neutropenia - fever and mouth ulcers

Question 44

DOC intermittent claudication

Answer 44

cilostazole/dipyridamole - PDE3 inhibitor .: increase cAMP in platelets .: prevents aggregation - direct vasodilation AE = headache, flush