Heme

Question 1

Increased Hct means?

Answer 1

Polycythemia/ Erythrocytosis

Question 2

What do Dense nd alpha granules contain in platelets?

Answer 2

dense= Ca + ADP
alpha= Fibrinogen + vWF

Question 3

Blood cell differential?

Answer 3

PMNs= 54-62%
Lymphs= 25-33%
Monocytes= 3-7%
Eos= 1-3%
Basophils= 0-1%

Question 4

What cell contains Azurophilic granules?

Answer 4

PMNs= Lysosomes

Question 5

Large cell with Kidney shaped nucleus and frosted glass cytoplasm?

Answer 5

Monocyte

Question 6

What cell contains Histaminase and arylsulfatase to help limit rxn following mast cell degranulation?

Answer 6

Eosinophils

Question 7

What do basophils contain?

Answer 7

Heparin
Histamine
LTD4

Question 8

What is CD28?

Answer 8

Co-stimulatory signal required for Tcell activation

Question 9

What coagulation factors are in the COMMON pathway?

Answer 9

X
V
II (Thrombin)
I (Fibrinogen)
XIII (cross links fibrin)

Question 10

What is the function of Protein C/S?

Answer 10

Inactivate Factors V + VIII

Question 11

What is the function of Ca & ADP released by platelets?

Answer 11

Ca=> required for coagulation pathways

ADP=> induces GPIIb/IIIa (fibrinogen) receptor expression

Question 12

Def in GpIb receptor?

Answer 12

Bernard-Soulier==> NO vWF adhesion

Question 13

Def in GpIIb/IIIa receptor?

Answer 13

Glanzmanns thombasthenia ==> binds Fibrinogen

Receptor blocked by Abciximab*

Question 14

Abciximab function like what disease?

Answer 14

Glanzmann’s thrombosthenia

Question 15

What blocks ADP receptor?

Answer 15

Clopidogrel

ticlopidine

Question 16

What is inside Weibel Palade bodies of endothelial cells?

Answer 16

vWF
PGI2
tPA
thromboplastin

Question 17

Ringed sideroblasts?

Answer 17

Excess IRON inside Mitochondria

Question 18

Heinz bodies vs howell jolly bodies?

Answer 18

Heinze=> Oxidation of iron leads to denatured Hb

Howell=> Nuclear remnant (asplenics)

Question 19

Mothball (naphthalene) ingestion or asplenic blood smear shows?

Answer 19

Howell jolly bodies

Question 20

Triad of Fe def + esophageal webs + Atrophic glossitis?

Answer 20

Plummer vinson syndrome

Question 21

HbH vs Hb Barts?

Answer 21

Alpha thalasemia
Barts ==> NO alpha chains = 4 Gamma chain= Hydrops
H ==> 3alpha deletion = 4 Beta chains

Question 22

What causes Beta thalasemia?

Answer 22

Point mutation @ splice site or Promoter sequence

Question 23

Pt with Increased HbA2 vs HbF (“chipmunk facies”)?

Answer 23

HbA2==> beta thal Minor

HbF/chipmunk ==> beta thal Major

Question 24

Lead inhibits?

Answer 24

Ferrochelatase + ALA dehydratase

rRNA degradation

Question 25

Hereditary Siderblastic anemia vs INH similarities?

Answer 25

``` Hereditary = ALA synthase def INH = B6 (pyridoxine) = cofactor for ALA synthase ```

Question 26

Child with megaloblastic anemia not corrected with B12 + Folate?

Answer 26

Orotic aciduria==> Rx with Uridine monophosphate

Question 27

Newborn with hemolytic anemia, thorny RBCs, and enzyme defect?

Answer 27

Pyruvate kinase defect==> dec ATP = rigid RBCs

Question 28

What is Eculizumab?

Answer 28

inhibits Complement cascade== Rx for PNH (CD55/59 def)

Question 29

SS mutation?

Answer 29

Glutamic acid--> Valine @ p6 | >60%= sickling

Question 30

Cryoprecipitate vs FFP?

Answer 30

Cryo==> contains Fibrinogen + VIII (given for selective def) | FFP ==> All coag factors (Warfarin OD, DIC)

Question 31

Risks of repeated Transfusions?

Answer 31

Iron Overload Hypocalcemia (citrate chelates Ca) Hyperkalemia (lysed RBC release IC K+)

Question 32

Why is leukocyte Alkaline phosphatase important?

Answer 32

PMNs that are active have ALP Left shift in Inflammation= Increased ALP CML left shift = Dec ALP

Question 33

Binucleate cell that is CD30+ & CD15+?

Answer 33

Reed Stenrberg cell= Hodgkins

Question 34

Post transfusion pt gets fever, back pain, and hypotension?

Answer 34

Hemolytic transfusion rxn = T2HS

Question 35

t(12;21), TdT+, CALLA+?

Answer 35

ALL

Question 36

smudge cells on smear + autoimmune warm hemolytic anemia?

Answer 36

CLL

Question 37

pt with Basophilia, neutrophilia, increased metamyelocytes. Low ALP?

Answer 37

CML | **Can have blast crisis .vs. [CLL which CANNOT]

Question 38

What are auer rods?

Answer 38

Azurophilic granules in APML t(15;17)

Question 39

Child with lytic bone lesions, Rash, and biopsy shows S100+ CD1 cells with Birbeck granules?

Answer 39

Langerhans histiocytosis

Question 40

What mutation causes Myelofibroproliferative disorders?

Answer 40

JAK2 mutations (receptor-associated TK)