Pediatric WBC disorders, lymphoma, leukemia Newman Flashcards

1
Q

RBC and WBC life span

A
  • RBC- 120 days

- WBC- hrs up to 21 days

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2
Q

if anemic- then look at?

A
  • MCV

- retic count

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3
Q

WBC numbers

A

Never Let Monkeys Eat Bananas

-Neutrophils > lymphocytes > monocytes > eosinophils > basophils

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4
Q

absolute neutrophil count (ANC)=

A

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

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5
Q

neutropenia- defined as

A

ANC < 1500

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6
Q

severe neutropenia

A

ANC < 500

-risk of serious infection!!!

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7
Q

severe congenital neutropenia

A

(Kostmann syndrome)

-inc risk of leukemia (AML)

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8
Q

cyclic neutropenia

A
  • 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%

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9
Q

Shwachman-Diamond syndrome

A

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

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10
Q

fanconi anemia

A
  • bone marrow failure syndrome

- GU and skeletal abnormalities, inc chromosome fragility

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11
Q

leukocyte adhesion deficiency

A

-delayed separation of umbilical cord

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12
Q

chronic granulomatous dz

A
  • recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy
  • defect in oxidative metabolism
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13
Q

Infection-assoc neutropenia

A
  • many viruses cause neutropenia within the first 2-3 days of illness, lasting up to 1 wk
  • Parvovirus B19!!
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14
Q

Drug-induced neutropenia

A

-cytotoxic agents for tx for malignancies!!

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15
Q

leukocytosis- usually due to

A

reactive process to an infection

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16
Q

most common cancer in adolescents?

A

-lymphoma!!

17
Q

most common malignancy n age group of 15-19 yo

A

-Hodgkin lymphoma

18
Q

pathogenesis of HL

A
  • Reed-Sternberg cell! (large cell with mult or multilobulated nuclei)
  • “orderly fashion”
19
Q

HL- clinical manifestations

A
  • painless, nontender, firm, rubbery lymphadenopathy

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

20
Q

diagnosis of HL

A

-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

21
Q

NHL

A

60% of lymphomas in children and adolescents

22
Q

NHL- can be secondary to?

A

SCID (severe combined immune def)

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

23
Q

NHL- most common subtype

A

-Burkitt lymphoma

24
Q

Burkitt lymphoma- commonly manifests as?

A

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

25
Q

leukemia- results from?

A

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

26
Q

regional collections of nodes

A
  • cervical
  • supra-clavicular
  • axillary
  • inguinal
27
Q

lymphadenopathy- most important component of the work-up

A

-history and physical

28
Q

size of nodes

A
  • > 2 cm is enlarged

- supraclavicular is not normal

29
Q

quality and location of LNs

A
  • lymphadenitis- tender, warm, red, fluctuant
  • benign- soft, mobile, smooth
  • lymphoma- firm and rubbery
  • malignancy or Tb- fixed and matted
  • if generalized- think systemic dz!
30
Q

LDH- marker for>

A

hematologic manifestations

31
Q

testicular mass- must do what?

A

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

32
Q

teratoma

A
  • sacrococcygeal most common
  • benign, well-diff cystic lesions (mature)
  • lesions of indeterminate potential (immature)
  • unequivocally malignant teratomas (mixed with another germ cell tumor component)
33
Q

neuroblastoma- is what?

A
  • tumors of symp ganglia and adrenal medulla
  • derived from primordial neural crest cells
  • most common extracranial solid tumor of childhood!!!
34
Q

neuroblastoma- clinical course

A
  • <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
35
Q

Wilms tumor

A
  • most common primary renal tumor of childhood!!

- age 2-5

36
Q

WAGR syndrome

A
  • wilms tumor, aniridia, genital anomalies, mental retardation
  • 33% of developing wilms tumor
37
Q

Denys-Drash syndrome

A
  • gonadal dysgenesis (male pseudohermaphroditism), early onset nephropathy, mutation in zinc-finger region of WT1
  • 90% chance of developing Wilms tumor
38
Q

Beckwith-Wiedemann syndrome

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