Hematology

Question 1

Composition of:
HbA
HbA2
HbF
Hb Barts
HbH

Answer 1

HbA = 2 alpha, 2 beta
HbA2 = 2 alpha, 2 delta
HbF = 2 alpha, 2 gamma
Hb Barts = 4 gamma
HbH = 4 beta

Question 2

Normal percentages for:
HbA
HbA2
HbF

Answer 2

HbA = 95-98%
HbA2 = 2-3%
HbF:
- Adult = 0.2%
- Newborn = 50-80%
- < 6 mo = < 8%

Question 3

Percent HbS for:
Sickle disease
Sickle trait
Sickle Alpha thal
Sickle Beta thal

Answer 3

Sickle disease (SS) - 80% HbS
Sick trait (SA) - 35-40%
Trait + 1 alpha del - 30-40%
Trait + 2 alpha delt - 25-35%
Trait + beta thal - >40%

Question 4

Hemoglobin in alkaline gel

Answer 4

(Anode)
Hb A
Hb F
Hb S
Hb C
(Cathode)

Question 5

Hemoglobin in acidic gel

Answer 5

(Anode)
Hb C
Hb S
Hb A
Hb F
(Cathode)

Question 6

Von Willebrand Disease - Sizes

Answer 6

1 = All present but decreased
2A = only small present
2B = small and intermediate present
2N = normal (low factor 8)
3 = absence of all

Question 7

Von Willebrand Disease - Mechanisms

Answer 7

1 = general decrease, Aut Dom
2A = assembly issue, so only small present (A = Assembly)
2B = increased platelet affinity - so lose large and keep small and intermediate (B = Binding)
2N = mutation in binding site to factor 8, so only factor destabilized (N = Not VWF)
3 = absence of all, Aut Rec

Question 8

Hemoglobin mutations

Answer 8

HbS - glutamte 6 to valine

HbC - glutamate 6 to lysine

HbE - glutamte 26 to valine

Question 9

HbE - symptoms, demographic

Answer 9

E/E - Mild anemia, microcytosis

A/E - No anemia, microcytosis only

SE Asia

Question 10

Bilirubinemia - Direct/Indirect and Clinical

Gilbert
Crigler-Najjar
Dubin-Johnson
Rotor

Answer 10

Gilbert - indirect (<5); benign, appears during stress

Crigler-Najjar - indirect (>5)

Dubin-Johnson - direct; dark liver

Rotor - direct; benign

Question 11

Alpha globin locus

Answer 11

Zeta x 2 (embryonic)

Alpha x 2 (fetal + adult)

Question 12

Beta globin locus

Answer 12

Epsilon (embryonic)

Gamma (fetal)

Delta (adult)

Beta (adult)

Question 13

HbC - symptoms

Answer 13

Trait (A/C) - asymptomatic, may have atrget cells and rare crystals

Disease (C/C) - splenomegaly, microcytic anemia, RBC crystals

Question 14

Hemoglobin catabolism

Answer 14

Hemoglobin - Heme + globins

Heme - Protoporphyrin, CO, iron
Protoporphyrin to biliverdin then bilirubin

Question 15

Hemophilia A

Answer 15

Factor 8
X linked recessive

Question 16

Hemophilia B

Answer 16

Factor 9
X linked recessive

Question 17

Hemophilia C

Answer 17

Factor 11
Autosomal recessive

Question 18

Myelodysplasia vs MPN vs leukemia

Answer 18

Myelodysplasia = cytopenias

MPN = proliferaiton of mature cells (<20% blasts)

Leukemia = proliferation of immature cells (>20% blasts)

Question 19

Myelodysplasia - marrow characteristics

Answer 19

Hypolobated megas
Erythroid with budding or lobated nuclei

Question 20

Megaloblastic anemia -
Folate vs B12

Answer 20

B12 = elevated homocystine and methylmalonic acid

Folate = only homocysteine

Question 21

Intravascular vs Extravascular hemolysis
- Diseases
- Hb in urine
- Morphology

Answer 21

Intravascular:
- DIC, TTP, HUS, PNH, heart valve
- Free Hb in urine
- Schistocytes

Extravascular
- G6PD, hereditary elliptocytosis, hereditary spherocytosis, Hb disease, thalassemia
- No Hb in urine
- Spherocytes

Question 22

Hereditary Spherocytosis - test

Answer 22

1) Osmotic fragility test

2) EMA test - spherocytes have lower uptake, stomatocytes have higher

Question 23

Stomatocytes
- Description
- Diseases

Answer 23

Stomatocytes
- RBCs with thin slit-like pallor
- Hereditary (Na-K permeability), alcohol/liver disease, Rh null disease

Question 24

G6PD - abnormal cells

Answer 24

Heinz bodies (small round dark inclusion)

Bite cells, blister cells - where spleen removed

Question 25

Target cells - Description - Diseases

Answer 25

Target cells - Target shape - HbC/E/S, liver disease

Question 26

Alpha thalassemia Deletions - CBC, gel

Answer 26

One deletion - silent Normal CBC, Normal gel Two deletions - Thal trait Abnormal CBC, Normal gel Three deletions - HbH disease (4 beta) Abnormal CBC, Fast migrating HbH 20%, HbA 80% Four deletions - Barts (4 gamma) Abnormal CBC, 100% Fast migrating Hb Barts

Question 27

Alpha Thal trait - cis vs trans ethnicities, prognosis

Answer 27

Cis = --/aa, Asian American, worse prognosis Trans = -a/-a, African American, better prognosis

Question 28

Beta thal - gel b thal minor b0 thal major b+ thal major

Answer 28

b thal minor - 94% HbA, 6% HbA2 b0 thal major - 98% HbF, 2% HbA2 b+ thal major - 20% HbA, 80% HbF

Question 29

Howell-Jolly body

Answer 29

Single dense inclusion, made of DNA MDS, post splenectomy, sickle cell

Question 30

Basophilic stippling

Answer 30

Punctate, made of RNA Lead poisoning, MDS, sideroblastic anemia

Question 31

Pappenheimer body

Answer 31

Multple inclusions, made of iron Iron overload and post splenectomy

Question 32

Iron Defic Anemia - Ferritin - TIBC - Transferrin sat - Soluble transferrin receptor

Answer 32

Body scavenging for iron - Normal ferritin - High TIBC - Low Transferrin sat - High soluble transferrin receptor

Question 33

Anemia of chronic disease - Ferritin - TIBC - Transferrin sat - Soluble transferrin receptor

Answer 33

Body keeping iron out of blood - High ferritin - Low TIBC - High transferrin sat - Normal transfferin receptor

Question 34

Sickle cell + alpha vs beta thal Better or worse sickling

Answer 34

Sickle + alpha = better (fewer alpha - competitive binding to normal beta) Sickle + beta = worse (fewer normal beta - worse alpha + sickle)

Question 35

Hemoglobin Lepore - Genetics - Sx - Gel

Answer 35

- Genetics: fusion transcript of delta-beta - Sx: mild microcytic anemi - Gel: Lepore runs at same location as HbS, ~15%

Question 36

When should RhIg be given?

Answer 36

- 28 weeks - After complications - After birth