First Aid 392-396 Heme/Onco

Question 1

Which heme disorder is assoc with hypersegmented neutrophils?

Answer 1

Megaloblastic anemia

Question 2

Which three drugs are associated with folate deficiency?

Answer 2

Methotrexate, Trimethoprim, Phenytoin

Question 3

B12 def is associated with inc in what metabolic intermediate?

Answer 3

L-mm CoA, aka methylmalonic acid

Question 4

Where do you see subacute combined degeneration of the spino cerebellar tract?

Answer 4

B12 def - along with lat corticospinal tract, dorsal column dysfunction

Question 5

How do you differentiate Orotic aciduria from Ornithine transcarbamylase def?

Answer 5

There is no hyperammonemia with Orotic aciduria

Question 6

Most common causes of macrocytic anemia that is not megaloblastic?

Answer 6

Alcoholism, lIver disease, hypothyroidism, reticulocytosis

Question 7

Defective enzyme in Orotic aciduria? Substrate?

Answer 7

Orotic Acid –> UMP, done by UMP synthase

Question 8

Short stature, thumb and radial defects?

Answer 8

Diamond Blackfan anemia and Fanconi anemia

Question 9

Two heme diseases with inc HbF?

Answer 9

B thalassemia major, Diamond blackfan anemia

Question 10

dec haptoglobin, inc LDH, target cells seen in ?

Answer 10

intravascular hemolysis

Question 11

Urine test shows Hgb, hemosiderin, and urobilinogen?

Answer 11

Intravacular hemolysis

Question 12

Inc risk of gallstones and BM hyperplasia with what type of hematological disordeR?

Answer 12

extravascular hemolysis

Question 13

Iron, TIBC, ferritin - inc or dec in ACD (anemia of chronic disease)?

Answer 13

Dec FE, TIBC (indicator of transferrin) Inc Ferritin

Question 14

What differentiates Diamond Blackfan from Fanconi Anemia?

Answer 14

Fanconi has cafe au lait spots

Question 15

Is retic # inc or dec with aplastic anemia?

Answer 15

Dec

Question 16

Most common congenital cause of Aplastic anemia?

Answer 16

Fanconi anemia

Question 17

MCV and MCHC in heriditary spherocytosis

?

Answer 17

Inc MCHC (bc no central pallor) and normal to low MCV

Question 18

Dec ATP production is a part of the pathogenesis of what heme disorder?

Answer 18

Pyruvate kinase deficiency - dec ATP will lead to rigid RBCs –> extravasc hemolysis

Question 19

What amino acid mutation in HbC disease?

Answer 19

glutamic acid to lysine in B globin chain

Question 20

CD marker in Paroxysmal nocturnal hemoglobinuria?

Answer 20

CD 55/59

Question 21

Tx for Paroxysmal nocturnal hemoglobinuria?

Answer 21

Eculizumab - complement inhibitor

Question 22

Pathogenesis of PNH?

Answer 22

Impaired synthesis of GPI anchor for decay accelerating factor that protects RBC from complement

Question 23

Why are newborns normally asymptomatic in sickle cell?

Answer 23

They have higher hbF and lower HbS

Question 24

Why is there a crew cut skull in thalassemia major and Sickle cell?

Answer 24

Marrow expansion due to inc erythropoiesis

Question 25

What renal issue in Sickle cell and why?

Answer 25

Renal papillary necrosis - due to dec pO2 in papilla

Question 26

Why give hydroxyurea in Sickle cell?

Answer 26

Inc the amount of HbF

Question 27

Sickle cell osteomyelitis is assoc with which bact?

Answer 27

Salmonella

Question 28

What Ig is seen in Warm AIHA and Cold AIHA?

Answer 28

IgG and IgM

Question 29

Which 3 pathologies are associated with cold agglutination AIHA?

Answer 29

Mycoplasma pneumoniae, Infectious Mono, CLL

Question 30

Drug assoc with Warm AIHA?

Answer 30

methyldopa (alpha)

Question 31

Microangiopathic hemolytic anemia seen in which diseases? (6)

Answer 31

DIC, TTP, HUS, SLE, Malig HTN, HELLP

Question 32

Two diseases associated wtih macroangiopathic anemia?

Answer 32

Prosthetic heart valves and aortic stenosis

Question 33

What is mixed with patients blood in Direct coombs test?

Answer 33

anti Ig antibody

Question 34

What in patients serum will yield a positive result in indirect coombs test?

Answer 34

If serum has anti RBC surface Ig, RBC will agglutinate = pos result

Question 35

What is the difference in Labs between ACD and Hemochromatosis?

Answer 35

Both have low TIBC and and high ferritin, but Hemochromatosis has high Fe levels and also high %transferrin sat'n therefore.

Question 36

Neutropenia cut off point?

Question 37

Eosinophilia cut off point?

Question 38

Which drug causes neutrophilia, inspite of eosinopenia and lymphopenia?

Answer 38

Corticosteroids (dec activation of neutrophil adhesion molecules, dec migration out, stay in blood - However, tend to keep eosinophils in l.n and cause lymphocyte apoptosis )

Question 39

What is a Left shift?

Answer 39

shift to a more immature cell in maturation process - i.e. inc neutrophil precursors, like band cells and metamyelocytes in periph blood. In infection and inflammation, neutrophils are rapidly used up (neutrophils now, macs later) so need to make more. Produce them so fast, end up releasing more immature cells in our hurry.

Question 40

Enzymes def in lead poisoning?

Answer 40

ALA DH, and Ferro chelatase - "Dont eat DAH Fucking Lead!"

Question 41

5 Ps of Acute intermittent porphyrias?

Answer 41

Painful stomach, Port wine colored urine, Polyneuropathy, Psych issues, Precipitated by drugs, etc

Question 42

Tx for AIP? (2) Why?

Answer 42

Glucose and Heme, they inhibit ALA synthase

Question 43

Enzyme def in AIP?

Answer 43

PBG deaminase

Question 44

Which HIV drugs are CI with Acute Intermittent Porphyria?

Answer 44

NNRTIs - Nevirapine, Efavirenz (p450 inducer)

Question 45

Def enzyme in Porphyria cutanea tarda?

Answer 45

UPG decarboxylase

Question 46

Which is the most common Porphyria?

Answer 46

PCTarda

Question 47

Which pathology is assoc with def in ALA synthase?

Answer 47

Sideroblastic anemia - S = synthase and sideroblastic

Question 48

Which porphyria is assoc with photosensitivity?

Answer 48

PCT