Hem/Onc

Question 1

What is t(4;11) associated with?

Answer 1

ALL

Question 2

What is t(9;22) associated with?

Answer 2

CML

Philadelphia chromosome

Question 3

What is t(8;14) associated with?

Answer 3

Burkitt’s Lymphoma
EBV
Non Hodgkin’s Lymphoma
B cell

Question 4

What are the features of osteosarcoma?

Answer 4

• lesion at proximal or distal end of long bone

- lesion shows a SUNBURST PATTERN

Question 5

What are the features of Ewing sarcoma?

Answer 5

• lesion has a LAMELLATED “ONION SKIN” appearance

Question 6

What are the features of osteoid osteoma?

Answer 6

X-ray will show a central radiolucency with thickened bone around it.

Question 7

When does ABO incompatibility usually occur?

Answer 7

In a mother with type O blood during first (or subsequent) pregnancy. She will have anti-A and anti-B antibodies that can cause hemolytic anemia in a baby with type A or type B blood.

Question 8

What are the features of erythema infectiosum?

Answer 8

• Parvovirus B19
• Slapped cheek
• Rash on extremities will appear later (lacy rash, macular, morbilliform)
• Diagnose with IgM titers
• Can cause aplastic crisis
• Intrauterine infection can cause hydrosphere fetalis

Question 9

What are the features of paroxysmal nocturnal hemoglobinuria (PNH)?

Answer 9

• Dark colored urine in the mornings
• Complement mediated hemolysis
• Coombs negative auto hemolytic anemia
• Diagnosis: flow cytometry for CD59, DF assay, Ham’s test

Question 10

What are the encapsulated organisms?

Answer 10

"Some Nasty Killers Have Some Capsule Protection."
S = Streptococcus pheumoniae
N = Neiserria meningitidis
K = Klebsiella pneumoniae 
H = Haemophilus influenzae 
S = Salmonella typhi
C = Cryptococcus neoformans
P = Pseudomonas aeruginosa

Question 11

What are the four features of iron deficiency anemia?

Answer 11

• Low transferrin saturation
• High RDW
• Low reticulocyte count
• Ferritin level < 15

Question 12

What is transferrin?

Answer 12

Transports iron.

Saturation level will be low if there is no iron available to transport.

Question 13

How do you calculate the Mentzer Index?

Answer 13

MCV/RBC

>12 = iron deficiency anemia 
<11 = thalassemia

Question 14

What is seen on the blood smear for beta thalassemia major?

Answer 14

Microcytosis
Target cells
Basophilic stippling

Question 15

How do you differentiate lead toxicity from iron deficiency anemia?

Answer 15

RBCs will have basophilic stippling.

Question 16

At what lead level is chelation therapy indicated?

Answer 16

Level > 45

Chelate with EDTA, dimercaprol, or d-penicillamine.

Question 17

What is pernicious anemia?

Answer 17

The lack of intrinsic factor, a protein produced by the stomach to help absorb vitamin B 12.

Question 18

What are the symptoms of vitamin B 12 deficiency?

Answer 18

Macrocytic anemia
Neurologic issues (paresthesias, ataxia, decreased reflexes)
In pregnant women, spina bifida

Question 19

What are the features of Fanconi anemia?

Answer 19

MACROCYTIC ANEMIA/ APLASTIC ANEMIA - everything else is small (small head, short stature)

F = Facial (microcephaly)
A = Abnormal testes and kidneys (hypogonadism, renal abnormalities)
N = No thumbs/radius
C = Cafe au lait spots
O = Ocular abnormality
N = Neutropenia
I = Increased risk of AML

Question 20

What are the features of diamond Blackfan anemia?

Answer 20

MACROCYTIC ANEMIA

• Pure red cell aplasia
• Present around 3 months of age
• Low reticulocyte count
• Physical: triphalangeal thumb, craniofacial abnormalities

Question 21

What is maternal immune thrombocytopenic Purpura?

Answer 21

Thrombocytopenia in neonates that lasts for weeks.

Mother forms antibodies against fetal platelet antigens inherited from the father.

aka, neonatal alloimmune thrombocytopenia (NAIT)

Question 22

What are the vitamin K dependent coagulation factors?

Answer 22

2, 7, 9, 10

Question 23

What are the features of von Willebrand disease?

Answer 23

“Factor eight on a plate to go on a date with von Willebrand.”
- Factor 8 and platelets need vWF to function.

• Autosomal dominant
• Elevated PTT
• Prolonged bleeding time

Question 24

How is von Willebrand deficiency treated?

Answer 24

• Minor bleed: give DDAVP (to increase plasma factor 8 and vWF
• Severe bleed: Factor 8 concentrate (Humate P); cryoprecipitate (has factor 8 and vWF
• Aminocaproic acid: for mucosal bleeding (helps to inhibit fibrinolysis)

Question 25

What are the lab features of DIC?

Answer 25

Thrombocytopenia
Low fibrinogen
Elevated fibrinogen split products (D-dimer)
Elevated thrombin time 
Elevated PT 
Elevated PTT

Question 26

How is DIC treated?

Answer 26

• Thrombocytopenia: platelets
• Anemia: pRBCs
• Low fibrinogen: cryoprecipitate (has fibrinogen, factor 8, vWF)
• Low clotting factors: FFP (has most of the clotting factors)

Question 27

What is an osteochondroma?

Answer 27

• Common, benign bony tumor
• Usually located near growth plates
• Has a cartilaginous cap

Question 28

Which causes more severe anemia: alpha or beta thalassemia?

Answer 28

Beta thalassemia

Question 29

What are the 3 types of beta thalassemia?

Answer 29

• Beta thalassemia trait: mild microcytosis
• Beta thalassemia intermedia: obvious microcytosis; occasional blood tx after illness
• Beta thalassemia major: Cooley’s anemia; frequent tx

Question 30

What are the clinical features of Beta thalassemia major?

Answer 30

• Frequent tx
• Hemochromatosis
• Smear: microcytes, target cells, basophillic stippling
• Requires chelation (tx with desferoximine)