Pediatric Leukemia, Lymphoma, & Non-Neoplastic WBC Disorders

Question 1

Leading cause of death by disease past infancy among children in US

Answer 1

Cancer

Question 2

Most common type of cancer diagnosed in kids age 0-14 years in US

Answer 2

Leukemias

Question 3

Most common type of cancer diagnosed in 15-19 year olds and the most common malignancy of this age group

Answer 3

Lymphoma

[Most common malignancy in the age group 15-19 is Hodgkin Lymphoma]

Question 4

Age distribution and viral associations with Hodgkin Lymphoma

Answer 4

Bimodal age distribution — peaks at 15-35 y/o and again after 50 y/o

Associated with EBV, HHV-6, and CMV

Question 5

Pathognomonic feature of HL

Answer 5

Reed-sternberg cell — a large cell w/multiple or multilobulated nuclei

Question 6

Clinical manifestations of HL may include B symptoms which are important for both diagnosis and staging — what are B symptoms?

Answer 6

Unexplained fever 39 C

Wt loss >10% total body wt over 6 months

Drenching night sweats

Question 7

Any patient with persistent, unexplained LAD unassociated with an obvious underlying inflammatory or infectious process should undergo _____ _____ to rule out a ______ _____ before undergoing LN biopsy

Answer 7

Chest radiography; mediastinal mass

Question 8

______ accounts for 60% of lymphomas in children and adolescents

Answer 8

NHL

Question 9

A small number of NHL cases are secondary to specific etiologies, including inherited or acquired immune deficiencies. What are 2 examples of immune etiologies?

Answer 9

SCID

Wiscott Aldrich syndrome

Question 10

A male pt presents with NHL, recurrent sino-pulmonary and ear infections, severe atopic dermatitis, and bleeding secondary to significant thrombocytopenia. What is the most likely underlying immune deficiency that led to the development of his NHL? What is its inheritance?

Answer 10

Wiscott Aldrich syndrome

X-linked recessive

Question 11

Describe the 2 major types of Burkitt Lymphoma (NHL)

Answer 11

Sporadic type — manifests as abdominal disease

Endemic type — manifests as head and neck disease

Question 12

T/F: NHL has a poor prognosis in those with localized disease

Answer 12

False — NHL pts with localized dz have 90-100% chance of survival. Advanced disease has a 60-95% chance of survival depending on pathologic subtype, tumor burder at dx (reflected by serum LDH), presence or absence of CNS disease, and specific sites of metastatic spread

Question 13

_______ account for the greatest percentage of cases of childhood malignancies

Answer 13

Leukemias

Question 14

Children with trisomy 21 are at increased risk for what 2 hematologic malignancies?

Answer 14

ALL

AML

Question 15

How is the absolute neutrophil count (ANC) calculated?

Answer 15

[(% neutrophils + % bands) x (WBC)]/(100)

Question 16

If the ANC is less than _______, the risk of serious infection is high

Answer 16

500

Note:
Neutropenia is defined as decrease in ANC to less than 1500/uL

Severe neutropenia = ANC <500/uL

Moderate neutropenia = ANC 500-1000/uL

Mild neutropenia = ANC 1000-1500

Question 17

Congenital disorder of neutrophils that manifests as life-threatening pyogenic infections, often in infancy, due to impaired myeloid differentiation caused by maturational arrest of neutrophil precursors

Answer 17

Severe Congenital Neutropenia (Kostmann syndrome)

Question 18

Severe Congenital Neutropenia (Kostmann syndrome) typically has a _____ _____ inheritance pattern. These patients have an increased risk of developing ____

Answer 18

Autosomal recessive; AML

Question 19

Congenital disorder of neutropenia that manifests as cyclic fever, oral ulcers, gingivitis, periodontal disease, and recurrent bacterial infections due to stem cell regulatory defects resulting in defective neutrophil maturation

Answer 19

Cyclic neutropenia

Question 20

Inheritance pattern and risk of malignancy in cyclic neutropenia

Answer 20

Sporadic or autosomal dominant

No increased risk of malignancy

Question 21

Congenital disorder of neutropenia that manifests as a triad of neutropenia, exocrine pancreas insufficiency, and skeletal abnormalities due to defects in neutrophil mobility, migration, and chemotaxis

Answer 21

Shwachman-Diamond syndrome

Question 22

Inheritance pattern and risk of malignancy with Shwachman-Diamond syndrome

Answer 22

Autosomal recessive

Increased risk for myelodysplastic syndrome or leukemia

Question 23

Autosomal recessive congenital disorder associated with neutropenia that presents in the first 10 years of life, in which all cell lines are affected and is considered the “classic example” of pancytopenia/bone marrow failure/aplastic anemia

Answer 23

Fanconi anemia

Question 24

Autosomal recessive fanconi anemia presents an increased risk for development of what malignancies?

Answer 24

AML
Brain tumors
Wilms tumor

Question 25

Rare autosomal recessive congenital disorder of neutrophil function that presents with delayed separation of the umbilical cord (>3 weeks), recurrent severe bacterial and fungal infections without pus accumulation due to diminished neutrophil adhesion to surfaces

Answer 25

Leukocyte Adhesion Deficiency

Question 26

The triad of severe eczema, recurrent bacterial infections of the skin (usually staph), and recurrent bacterial or fungal pulmonary infections is associated with what congenital disorder of neutrophil function?

Answer 26

Hyper-immunoglobulin E syndrome (hyper-IgE, Job syndrome)

Question 27

Congenital disorder of neutrophil function that presents with partial oculocutaneous albinism, peripheral and cranial neuropathies, neutropenia, and recurrent pyogenic infections

Answer 27

Chediak-Higashi syndrome

Question 28

Acquired disorder of neutrophils that presents with recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy

Answer 28

Chronic granulomatous disease

Question 29

Chronic granulomatous disease affects 1/250,000. What is the defect that causes chronic granulomatous disease and what is the px?

Answer 29

Defect in oxidative metabolism, absent generation of superoxide (which is toxic to microbes)

Good prognosis w/aggresive management of infection

Question 30

Many viruses cause neutropenia within the first 2-3 days of illness, lasting up to 1 week. What is one virus commonly associated with neutropenia as well as “slapped cheek” appearance 1-2 weeks after infection

Answer 30

Parvovirus B19

Question 31

Nutritional deficiencies associated with acquired neutropenia

Answer 31

Vitamin B12
Folate
Copper
Starvation

Question 32

______ is usually a reactive process to an infection which subsides with resolution of the acute event (viral or bacterial infection); Causes include chronic inflammation due to autoimmune disease, rheumatologic disorders, oncologic processes, and seizures

Answer 32

Leukocytosis