Pediatric WBC disorders, lymphoma, leukemia Newman

Question 1

RBC and WBC life span

Answer 1

• RBC- 120 days

- WBC- hrs up to 21 days

Question 2

if anemic- then look at?

Answer 2

• MCV

- retic count

Question 3

WBC numbers

Answer 3

Never Let Monkeys Eat Bananas

-Neutrophils > lymphocytes > monocytes > eosinophils > basophils

Question 4

absolute neutrophil count (ANC)=

Answer 4

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

Question 5

neutropenia- defined as

Answer 5

ANC < 1500

Question 6

severe neutropenia

Answer 6

ANC < 500

-risk of serious infection!!!

Question 7

severe congenital neutropenia

Answer 7

(Kostmann syndrome)

-inc risk of leukemia (AML)

Question 8

cyclic neutropenia

Answer 8

• neutropenia (<200) for 3-7 days every 3 wls

- CBC 2-3 times weekly for 6-8 wks to document cycles and nadir, ELA-2 mutation in 80-90%

Question 9

Shwachman-Diamond syndrome

Answer 9

-triad of neutropenia, exocrine pancreas insufficiency, skeletal abnormalities (feet, hands)

Question 10

fanconi anemia

Answer 10

• bone marrow failure syndrome

- GU and skeletal abnormalities, inc chromosome fragility

Question 11

leukocyte adhesion deficiency

Answer 11

-delayed separation of umbilical cord

Question 12

chronic granulomatous dz

Answer 12

• recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy
• defect in oxidative metabolism

Question 13

Infection-assoc neutropenia

Answer 13

• many viruses cause neutropenia within the first 2-3 days of illness, lasting up to 1 wk
• Parvovirus B19!!

Question 14

Drug-induced neutropenia

Answer 14

-cytotoxic agents for tx for malignancies!!

Question 15

leukocytosis- usually due to

Answer 15

reactive process to an infection

Question 16

most common cancer in adolescents?

Answer 16

-lymphoma!!

Question 17

most common malignancy n age group of 15-19 yo

Answer 17

-Hodgkin lymphoma

Question 18

pathogenesis of HL

Answer 18

• Reed-Sternberg cell! (large cell with mult or multilobulated nuclei)
• “orderly fashion”

Question 19

HL- clinical manifestations

Answer 19

• painless, nontender, firm, rubbery lymphadenopathy

- B symptoms (systemic sx important in staging)- fever (39C), wt loss >10% over 6 months, drenching night sweats

Question 20

diagnosis of HL

Answer 20

-any pt with persistent, unexplained lymphadenopathy unassoc with an obvious underlying infl or infectious process should undergo CHEST RADIOGRAPHY to rule out a mediastinal mass before undergoing LN biopsy

Question 21

NHL

Answer 21

60% of lymphomas in children and adolescents

Question 22

NHL- can be secondary to?

Answer 22

SCID (severe combined immune def)

-Wiscott-Aldrich syndrome (X-linked recessive)- recurrent sinopulm infections, eczema, bleeding (thrombocytopenia)

Question 23

NHL- most common subtype

Answer 23

-Burkitt lymphoma

Question 24

Burkitt lymphoma- commonly manifests as?

Answer 24

-abd (sporadic type) or head and neck dz (endemic type with involvement of BM or CNS

Question 25

leukemia- results from?

Answer 25

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

Question 26

regional collections of nodes

Answer 26

- cervical - supra-clavicular - axillary - inguinal

Question 27

lymphadenopathy- most important component of the work-up

Answer 27

-history and physical

Question 28

size of nodes

Answer 28

- >2 cm is enlarged | - supraclavicular is not normal

Question 29

quality and location of LNs

Answer 29

- lymphadenitis- tender, warm, red, fluctuant - benign- soft, mobile, smooth - lymphoma- firm and rubbery - malignancy or Tb- fixed and matted * if generalized- think systemic dz!

Question 30

LDH- marker for>

Answer 30

hematologic manifestations

Question 31

testicular mass- must do what?

Answer 31

w/o hx of trauma/injury- CHEST RADIOGRAPH is indicated

Question 32

teratoma

Answer 32

- sacrococcygeal most common - benign, well-diff cystic lesions (mature) - lesions of indeterminate potential (immature) - unequivocally malignant teratomas (mixed with another germ cell tumor component)

Question 33

neuroblastoma- is what?

Answer 33

- tumors of symp ganglia and adrenal medulla - derived from primordial neural crest cells - most common extracranial solid tumor of childhood!!!

Question 34

neuroblastoma- clinical course

Answer 34

- <2 yo- fever, large abd mass, weight loss, disseminated dz may present with mult cutaneous metastases (blueberry muffin baby) - older children- may not be dx until metastasis produces sx's- bone pain, resp sx, GI, periorbital region - 90% produce catecholamines!!!- elevated urine VMA and HVA

Question 35

Wilms tumor

Answer 35

- most common primary renal tumor of childhood!! | - age 2-5

Question 36

WAGR syndrome

Answer 36

- wilms tumor, aniridia, genital anomalies, mental retardation - 33% of developing wilms tumor

Question 37

Denys-Drash syndrome

Answer 37

- gonadal dysgenesis (male pseudohermaphroditism), early onset nephropathy, mutation in zinc-finger region of WT1 - 90% chance of developing Wilms tumor

Question 38

Beckwith-Wiedemann syndrome

Answer 38

- enlargement of body organs - macroglossia - hemihypertrophy - omphalocele - abnormal large cells in adrenal cortex