CIS: Pediatric WBC Neoplasms: Leukemia and Lymphoma

Question 1

What is the most common cancer in adolescents?

Answer 1

Lymphoma

Question 2

two broad categories of lymphoma

Answer 2

hodgkin and non hodgkin

Question 3

Hodgkin lymphoma

Answer 3

malignant process of the lymphoreticular system

• most common malignancy in the age group 15-19 y/o
• Bimodal distribution

Question 4

what is a reed-sternberg cell?

Answer 4

pathognomonic feature of HL

• large cell with multiple or multilobulated nuclei
• hematogenous spread occurs

Question 5

What kind of nodes will we feel in HL?

Answer 5

rubbery and firm

• not red or warm
• don’t hurt
• usually some mediastinal involvement

Question 6

What is important in the staging of HL?

Answer 6

B symptoms

• Unexplained fever (over 39C=103F)
• wt loss >10% of body weight over 6 months
• drenching night sweats

Question 7

Diagnosis of HL?

Answer 7

Any patient with persistent, unexplained LAD unassociated with an obvious underlying inflammatory or infectious process should undergo chest radiography to rule out a mediastinal mass before undergoing lymph node biopsy
-excisional LN biopsy is preferred

Question 8

What do we do after we diagnose HL?

Answer 8

stage it

Question 9

How is the prognosis with HL?

Answer 9

pretty good

Question 10

NHL*

Answer 10

Accounts for 60% of lymphomas in children and adolescents

Question 11

What can predispose us to NHL?

Answer 11

• SCID
• Wiscott-Aldrich syndrome (X-linked recessive): recurrent sinopulmnary infections, eczema, bleeding secondary to thrombocytopenia

Question 12

What is Wiscott-Aldrich syndrome

Answer 12

• recurrent sinopulmonary infection
• eczema
• bleeding (secondary to thrombocytopenia)
• it’s X-linked recessive

Question 13

Which NHL are we supposed to remember

Answer 13

Burkitt Lymphoma

Question 14

What is Burkitt lymphoma?

Answer 14

-commonly manifests as head and neck… can be abdominal with involvement of the bone marrow or CNS

Question 15

What accounts for the greatest percentage of cases of childhood malignancies?

Answer 15

Leukemias

Question 16

Pathogenesis of leukemia

Answer 16

-results from malignant transformation and clonal expansion of hematopoietic cells at an early stage of differentiation that are then unable to undergo further maturation

Question 17

What does ALL have?*

Answer 17

• 4 y/o
• white people
• males>females
• trisomy 21, bloom syndrome, fanconi anemia, ataxia telangiectasia, Shwachman syndrome, neurofibromatosis, twins, siblings at increased risk
• noniherited
• ionizing radiation can cause it
• EBV
• Wiskott-Aldrich, congenital hypogammaglobulinemia, ataxia telangiectasia

Question 18

What does AML have?*

Answer 18

-increased in adolescence
-Equal race
-Equal gender
-trisomy 21 (<3 y/o), bloom syndrome, fanconia anemia, atasia telangiectasia, Kostmann syndrome, NF-1, Diamnod Blackfanc syndrome, Li-Fraumeni syndrom
-Aplast anemia, myelodysplastic syndromes (MDS), PNH
-

Question 19

If he says widened mediastinum, we say…

Answer 19

lymphoma

Question 20

Leukocyte Adhesion deficiency*

Answer 20

• delayed separation of umbilical cord, very rare

- finish this

Question 21

Chronic granulomatous disease*

Answer 21

-recurrent purulent infections with fungal or bacterial catalase-positive organisms, … finish this

Question 22

Infection-associated neutropenia*

Answer 22

• Parvovirus B19

- many viruses will cause neutropenia within the first 2-3 days of illiness, lasting up to 1 week

Question 23

Drug induced neutropenia

Answer 23

cytotoxic agents for treatment of malignancies

Question 24

Leukocytosis

Answer 24

usually a reactive process to an INFECTION which subsides with resolution of the acute event (viral or bacterial infection)