Heme & Onc

Question 1

Hematopoiesis sites

Answer 1

Yolk sac 1* until 6 (10 weeks)
Liver 1* 6-22 weeks
BM 22+ (starts 8-19 weeks)

Question 2

Thalassemia

Answer 2

Quantitative defect of globin chain synthesis

Question 3

Alpha thalassemia

Answer 3

One gene absent - silent carrier
2 absent - alpha thal trait
3 absent - Hb H (4 beta)
None - hydrops

Question 4

Beta thalassemia

Answer 4

Trait

Disease - zero have increased Hb F and A2 but severe anemia ; plus have some Hb A and moderate anemia

Question 5

Sickle cell disease

Answer 5

Qualitative abnormality of beta globin chain gene

Valine for glutamic acid at position 6

Question 6

Fetal maternal hemorrhage calculation for Rhogam dose (kleihauer betke)

Answer 6

% Hb F = #fetal cells/maternal cells x100
1% = 50mL
The number of vials of rhogam is 1mL fetal blood/15
1 vial= 360mcg

Question 7

Apt test

Answer 7

Done on fetal sample and detects maternal blood

Question 8

Diamond Blackfan

Answer 8

Congenital hypoplastic anemia
Or congenital erythroid aplasia
Macrocytic anemia with reticulocytopenia
LBW, short stature, abn facies, triphalangeal thumbs, abn cardiac and renal systems
Congenital anomalies in ~40% of patients

Need chronic transfusions, steroids and BMT

Question 9

Constitutional aplastic anemia

Answer 9

Fanconi anemia
Ch instability with breaks common
Triphalangeal thumbs, radial hypoplasia, short stature, hyperpigmentation, renal anomalies

Test ch with mitomycin c - increased ch breaks

Treat androgens and hematopoietic growth factors

Question 10

Acquired erythrocyte underproduction

Answer 10

Erythroid aplasia (parvo)
Aplastic anemia
Infectious
Treatment with growth factors  
BMT
Iron, folic acid, vit b12 deficiency 
Lead toxicity

Question 11

Indirect vs direct Coombs

Answer 11

Direct performed on infants blood - ab noted on pt RBC

Indirect performed on mothers blood - detects ab in serum

Question 12

Polycythemia blood volume exchange

Answer 12

Blood volume = (observed - desired Hct)* infant blood volume / observed Hct

Question 13

Sick
Bleeding with
Dec PLT inc PT inc PTT

Answer 13

DIC

Question 14

Sick
Bleeding with
Dec PLT nl PT nl PTT

Answer 14

Platelet consumption

Question 15

Sick
Bleeding with
Nl PLT inc PT inc PTT

Answer 15

Liver disease

Question 16

Sick
Bleeding with
Nl PLT nl PT nl PTT

Answer 16

Compromised vascular integrity

Question 17

Healthy
Bleeding with
Dec PLT nl PT nl PTT

Answer 17

ITP, occult infection, thrombosis, BM hypoplasia or infiltration

Question 18

Healthy
Bleeding with
Nl PLT nl PT nl PTT

Answer 18

Vit K deficiency

Question 19

Healthy
Bleeding with
Nl PLT nl PT inc PTT

Answer 19

Hereditary clotting factor deficiencies

Question 20

Healthy
Bleeding with
Nl PLT nl PT nl PTT

Answer 20

Local bleeding, plt abn, factor 13 deficiency, VWD

Question 21

Langerhan cell histiocytosis

Answer 21

Fever
Pancytopenia
Multiorgan infiltration

Question 22

Hemophagic lymphohistiocytosis (hlh)

Answer 22

Familial - AR, fever, pancytopenia, HSM, adenopathy, fatal

Virus associated - fever, pancytopenia, HSM, CMV EBV HAV adenovirus

Question 23

Solid tumors

Answer 23

Teratoma
Neuroblastoma
Pheochromocytoma
Wilms
Hepatoblastoma
Retinoblastoma
Rhabdomyosarcoma

Question 24

Neuroblastoma

Answer 24

Second most common solid tumor in neonatal period
Prognosis depends on stage, age, n-myc amplification
Stage IV-S = <1 yo, localized primary tumor with mets limited to live skin or BM (favorable)