Pediatric Leukemia, Lymphoma, & Non-Neoplastic WBC Disorders Flashcards Preview

Heme Lymph Exam II > Pediatric Leukemia, Lymphoma, & Non-Neoplastic WBC Disorders > Flashcards

Flashcards in Pediatric Leukemia, Lymphoma, & Non-Neoplastic WBC Disorders Deck (32)
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1
Q

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

A

Cancer

2
Q

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

A

Leukemias

3
Q

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

A

Lymphoma

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

4
Q

Age distribution and viral associations with Hodgkin Lymphoma

A

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

Associated with EBV, HHV-6, and CMV

5
Q

Pathognomonic feature of HL

A

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

6
Q

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

A

Unexplained fever 39 C

Wt loss >10% total body wt over 6 months

Drenching night sweats

7
Q

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

A

Chest radiography; mediastinal mass

8
Q

______ accounts for 60% of lymphomas in children and adolescents

A

NHL

9
Q

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

A

SCID

Wiscott Aldrich syndrome

10
Q

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?

A

Wiscott Aldrich syndrome

X-linked recessive

11
Q

Describe the 2 major types of Burkitt Lymphoma (NHL)

A

Sporadic type — manifests as abdominal disease

Endemic type — manifests as head and neck disease

12
Q

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

A

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

13
Q

_______ account for the greatest percentage of cases of childhood malignancies

A

Leukemias

14
Q

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

A

ALL

AML

15
Q

How is the absolute neutrophil count (ANC) calculated?

A

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

16
Q

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

A

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

17
Q

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

A

Severe Congenital Neutropenia (Kostmann syndrome)

18
Q

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

A

Autosomal recessive; AML

19
Q

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

A

Cyclic neutropenia

20
Q

Inheritance pattern and risk of malignancy in cyclic neutropenia

A

Sporadic or autosomal dominant

No increased risk of malignancy

21
Q

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

A

Shwachman-Diamond syndrome

22
Q

Inheritance pattern and risk of malignancy with Shwachman-Diamond syndrome

A

Autosomal recessive

Increased risk for myelodysplastic syndrome or leukemia

23
Q

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

A

Fanconi anemia

24
Q

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

A

AML
Brain tumors
Wilms tumor

25
Q

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

A

Leukocyte Adhesion Deficiency

26
Q

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?

A

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

27
Q

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

A

Chediak-Higashi syndrome

28
Q

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

A

Chronic granulomatous disease

29
Q

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

A

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

Good prognosis w/aggresive management of infection

30
Q

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

A

Parvovirus B19

31
Q

Nutritional deficiencies associated with acquired neutropenia

A

Vitamin B12
Folate
Copper
Starvation

32
Q

______ 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

A

Leukocytosis