Heme/Onc Final Details

Question 1

2 metabolism pathways in RBCs

Answer 1

Glycolysis and HMP shunt

Question 2

Purpose of the HMP shunt in RBCs

Answer 2

Produce NADPH to reduce glutathione

Question 3

Rate limiting step of the HMP shunt in RBCs

Answer 3

Glucose-6-phosephate dehydrogenase

Question 4

Two divisions of leukocytes

Answer 4

Granulocytes (basophils, neutrophils, and eosinophils)

Mononeuclear cells (lypmphocytes and monocytes)

Question 5

Embryo shapped nucleus with “frosted glass” cytoplasm

Answer 5

Monocytes

Question 6

What cytokine from what cell activates macrophages

What other cytokine is produced by these cells

Answer 6

INF-y from TH1 cells

TH1 cells also produce IL-2–> induces proliferation of T cells

Question 7

CD14 and CD40 cell surface markers

Answer 7

Marcophages

“Macro-fourges”

Question 8

Clock face chromatin

What dyscrasia is associated with these cells?

Answer 8

Plasma cells

Multiple Myeloma

Question 9

Which T lymphocytes recognized MHC I and which recognizes MHC II

Answer 9

MHC I–> CD8 cells (because they destroy self cells presenting forein antigen or foriengn cells presenting foreign antigen)

MHC II–> CD4 cells (mediate the responce to antigen presenting cells)

Question 10

Vit K dependant coagulation factors

Answer 10

I, VII, IX, X

Protein C and Protein S

Question 11

Intrinsic coagulation pathway factors and measurement

Answer 11

XII–> XI–> IX–> VIII

VIIIa then activates X–>Xa which is the rate limiting step

Measured with the aPTT

Question 12

Factors and measurement of the extrinsic pathway

Answer 12

VII–>VIIa

VIIa converts X–>Xa which again is the rate limiting step

measured with the PT/INR

Question 13

Common pathway factors

Answer 13

V–>Va by IIa

Va converts II–>IIa (prothrombin–>thrombin)

IIa(thrombin) cleaves fibrinogen to fibrin and crosslinks fibrin mesh

Question 14

What factor inactivates Va and therefore suppresses the common pathway?

In what disease is this an issue?

Answer 14

Protein C cleaves Va–> Factor V Leiden (mutated) cannot be inactivated by protein C

Most common cause of inherited hypercoagulability in whites

Question 15

Heparin inactivates what coag factor?

Answer 15

IIa

Thrombin inhbitor

Question 16

Protein C activated by

Answer 16

thrombomodulin

“modulates platelet plug formation”

Question 17

Two situations in which howell-jolly bodies are seen

Answer 17

Asplenia (autoinfarction or removal)

and

Napthalene poinsoning (moth balls)

Question 18

Pt eating dirt, rocks, excessive ice chips (common)

Answer 18

Pica–> iron deficiency anemia

Question 19

Plummer-vinson syndrome

Answer 19

Iron deficiency anemia

Esophageal webs

Atrophic glossitis

Question 20

Two forms of deletions seen in a-thalassemia

Which is more severe?

Answer 20

Cis and trans

Cis–> problematic because both deletions can be passed down on the one chromosome (Cis occurs in asains)

Trans occurs in africans

Question 21

HbH disease:

Answer 21

3 gene deletion of the alpha chain

Excessive formation of Beta chain tetramers (HbH)

Clinically significan anemia

Question 22

Hb Barts:

Answer 22

y-chain tetramers seen in 4 gene deletion alpha thalassemia

Hydrops fetalis–> incompatible with life

Question 23

Type of thalassemia present in mediterranian populations

Answer 23

B-thalassemia

Question 24

Increased HbA2 and underproduced B-chain

Answer 24

B-thalassemia minor (heterozygote)

Question 25

B-chain absent Increased HbF

Answer 25

B-thalassemia major

Question 26

Tx, complications and classical appearence of B-thalassemia

Answer 26

Transufusions--\> 2' hemochromotosis' Chipmunk facies--\>crew cut skull d/t extrameduallary hematopoeisis

Question 27

What is HbA2

Answer 27

alpha2delta2

Question 28

Enzymes inhibited by lead posioning

Answer 28

Ferrochelatase ALA-dehydratase Inhibits rRNA degrdation in RBD leading to basophilic stipling

Question 29

Signs and Sx of lead poinsoning

Answer 29

Lead lines on gingiva and metaphysis of long bones Encephalopathy and erythrocyte basophilic stipling Abdominal colic and anemia (sideroblastic) Foot and writst drop

Question 30

Tx of lead poisoning in adults? Kids?

Answer 30

Adults: EDTA and dimercaperol Kids: Succimer

Question 31

Major difference between folate and B12 deficiency How do you tell the difference chemically?

Answer 31

**No neuro signs **with **folate deficiencty** Folate deficiency: **elevated homocysteine** and **normal MMA** B12 deficiency: **elevated homocysteine** and **elevated MMA**

Question 32

megaloblastic anemia refractory to b12 and folate

Answer 32

Orotic adiuria Tx: uridine monophosphate

Question 33

free haoptoglobin is decreased in this type of anemia

Answer 33

invascular hymolysis (haptoglobin is binding free heme) --\> PNH, autoimmuno hemolytic anemia, mechanical causes

Question 34

elevated unconjugated bilirubin in thise type of hemolysis

Answer 34

Extravascular (spleen breaking it down) --\>G6PD def, Sickel cell, Hereditary spherocytosis, pyruvate kinase def, Hemeglobin C disease

Question 35

Underlying problem in paroxysmal nacturnal hemoglobinuria

Answer 35

Decay accelerating factor protects again hemolysis by complement (c3 convertase) DAF secured by GPI anchoring protein **Lack of GPI** causes a lack of DAF and completment mediated lysis Occurs at night because mild acidosis activates complement

Question 36

Cell marker indicative of PNH

Answer 36

**Absence of CD55 (DAF) or 59**

Question 37

Hams test

Answer 37

RBC lysis at low pH--\> + PNH

Question 38

Tx for sickle cell

Answer 38

hydroxyurea--\> increases HbF

Question 39

Autoimmune hymolytic anemia is caused by two antibodies

Answer 39

IgG (warm, methyldopa) IgM (cold, m. pnemonia)

Question 40

Painful abdomen, dark urine, polyneuropathy, and psycholigic disturbance

Answer 40

Acute intermittant porphyria

Question 41

enzyme deficient in acute intermittant porphyria

Answer 41

Porphobilinogen deaminase

Question 42

TTP cause and Sx

Answer 42

Defect in ADAMTS13--\> accumilation of vWF multimers FATRN Fever, Anemia, Thrombocytopenia, Renal dysfucntion/failure, Neurologic deficits

Question 43

Fried egg plasma cells

Answer 43

Multiple myeloma

Question 44

Roleaux formation and bence-jones proteinuria (IgG light chains in urine) Mprotein spike

Answer 44

Multiple myeloma

Question 45

Auer rods

Answer 45

AML t(15;17)

Question 46

Tx for AML

Answer 46

All trans retioic acid | (15-17 is vitamin A receptor mutation)

Question 47

Smudge cells

Answer 47

SLL and CLL

Question 48

Trap +

Answer 48

Hairy cell leukemia TRAP (+), trapped in bone marrow, dry tap on bone marrow biopsy

Question 49

Birbeck granules and S100+

Answer 49

Langerhans cell histiocytosis