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Flashcards in Heme/Onc Final Details Deck (49):
1

2 metabolism pathways in RBCs

Glycolysis and HMP shunt

2

Purpose of the HMP shunt in RBCs

Produce NADPH to reduce glutathione

3

Rate limiting step of the HMP shunt in RBCs

Glucose-6-phosephate dehydrogenase

4

Two divisions of leukocytes

Granulocytes (basophils, neutrophils, and eosinophils)

Mononeuclear cells (lypmphocytes and monocytes)

5

Embryo shapped nucleus with "frosted glass" cytoplasm

Monocytes

 

A image thumb
6

What cytokine from what cell activates macrophages

What other cytokine is produced by these cells

INF-y from TH1 cells

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

7

CD14 and CD40 cell surface markers

Marcophages

"Macro-fourges"

8

Clock face chromatin

What dyscrasia is associated with these cells?

Plasma cells

Multiple Myeloma

9

Which T lymphocytes recognized MHC I and which recognizes MHC II

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) 

10

Vit K dependant coagulation factors

I, VII, IX, X

Protein C and Protein S

11

Intrinsic coagulation pathway factors and measurement

XII--> XI--> IX--> VIII

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

Measured with the aPTT

 

12

Factors and measurement of the extrinsic pathway

VII-->VIIa

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

measured with the PT/INR

13

Common pathway factors

V-->Va by IIa

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

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

14

What factor inactivates Va and therefore suppresses the common pathway?

In what disease is this an issue?

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

Most common cause of inherited hypercoagulability in whites

15

Heparin inactivates what coag factor?

IIa

Thrombin inhbitor

16

Protein C activated by

thrombomodulin

"modulates platelet plug formation"

17

Two situations in which howell-jolly bodies are seen

Asplenia (autoinfarction or removal)

and

Napthalene poinsoning (moth balls)

18

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

Pica--> iron deficiency anemia

19

Plummer-vinson syndrome

Iron deficiency anemia

Esophageal webs

Atrophic glossitis

A image thumb
20

Two forms of deletions seen in a-thalassemia

Which is more severe?

Cis and trans

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

Trans occurs in africans

21

HbH disease:

3 gene deletion of the alpha chain

Excessive formation of Beta chain tetramers (HbH)

Clinically significan anemia

22

Hb Barts:

y-chain tetramers seen in 4 gene deletion alpha thalassemia

Hydrops fetalis--> incompatible with life

23

Type of thalassemia present in mediterranian populations

B-thalassemia

24

Increased HbA2 and underproduced B-chain

B-thalassemia minor (heterozygote)

25

B-chain absent

Increased HbF

B-thalassemia major

26

Tx, complications and classical appearence of B-thalassemia

Transufusions--> 2' hemochromotosis'

Chipmunk facies-->crew cut skull d/t extrameduallary hematopoeisis

27

What is HbA2

alpha2delta2

28

Enzymes inhibited by lead posioning

Ferrochelatase

ALA-dehydratase

Inhibits rRNA degrdation in RBD leading to basophilic stipling

29

Signs and Sx of lead poinsoning

Lead lines on gingiva and metaphysis of long bones

Encephalopathy and erythrocyte basophilic stipling

Abdominal colic and anemia (sideroblastic)

Foot and writst drop

30

Tx of lead poisoning in adults? Kids?

Adults: EDTA and dimercaperol

Kids: Succimer

31

Major difference between folate and B12 deficiency

How do you tell the difference chemically?

No neuro signs with folate deficiencty

Folate deficiency: elevated homocysteine and normal MMA

B12 deficiency: elevated homocysteine and elevated MMA

32

megaloblastic anemia refractory to b12 and folate

Orotic adiuria

Tx: uridine monophosphate

33

free haoptoglobin is decreased in this type of anemia

invascular hymolysis (haptoglobin is binding free heme)

--> PNH, autoimmuno hemolytic anemia, mechanical causes

34

elevated unconjugated bilirubin in thise type of hemolysis

Extravascular (spleen breaking it down)

-->G6PD def, Sickel cell, Hereditary spherocytosis, pyruvate kinase def, Hemeglobin C disease

35

Underlying problem in paroxysmal nacturnal hemoglobinuria

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

36

Cell marker indicative of PNH

Absence of CD55 (DAF) or 59

37

Hams test

RBC lysis at low pH--> + PNH

38

Tx for sickle cell

hydroxyurea--> increases HbF

39

Autoimmune hymolytic anemia is caused by two antibodies

IgG (warm, methyldopa)

IgM (cold, m. pnemonia)

40

Painful abdomen, dark urine, polyneuropathy, and psycholigic disturbance

Acute intermittant porphyria

41

enzyme deficient in acute intermittant porphyria

Porphobilinogen deaminase

42

TTP cause and Sx

Defect in ADAMTS13--> accumilation of vWF multimers

FATRN

Fever, Anemia, Thrombocytopenia, Renal dysfucntion/failure, Neurologic deficits

43

Fried egg plasma cells

Multiple myeloma

44

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

Mprotein spike

Multiple myeloma

45

Auer rods

AML

t(15;17)

46

Tx for AML

All trans retioic acid

(15-17 is vitamin A receptor mutation)

47

Smudge cells

SLL and CLL

48

Trap +

Hairy cell leukemia

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

49

Birbeck granules and S100+

Langerhans cell histiocytosis