Pediatric WBC disorders, lymphoma, leukemia Newman Flashcards
RBC and WBC life span
- RBC- 120 days
- WBC- hrs up to 21 days
if anemic- then look at?
- MCV
- retic count
WBC numbers
Never Let Monkeys Eat Bananas
-Neutrophils > lymphocytes > monocytes > eosinophils > basophils
absolute neutrophil count (ANC)=
[(%neutrophils + %bands) x (WBC)] / 100
neutropenia- defined as
ANC < 1500
severe neutropenia
ANC < 500
-risk of serious infection!!!
severe congenital neutropenia
(Kostmann syndrome)
-inc risk of leukemia (AML)
cyclic neutropenia
- 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%
Shwachman-Diamond syndrome
-triad of neutropenia, exocrine pancreas insufficiency, skeletal abnormalities (feet, hands)
fanconi anemia
- bone marrow failure syndrome
- GU and skeletal abnormalities, inc chromosome fragility
leukocyte adhesion deficiency
-delayed separation of umbilical cord
chronic granulomatous dz
- recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy
- defect in oxidative metabolism
Infection-assoc neutropenia
- many viruses cause neutropenia within the first 2-3 days of illness, lasting up to 1 wk
- Parvovirus B19!!
Drug-induced neutropenia
-cytotoxic agents for tx for malignancies!!
leukocytosis- usually due to
reactive process to an infection
most common cancer in adolescents?
-lymphoma!!
most common malignancy n age group of 15-19 yo
-Hodgkin lymphoma
pathogenesis of HL
- Reed-Sternberg cell! (large cell with mult or multilobulated nuclei)
- “orderly fashion”
HL- clinical manifestations
- painless, nontender, firm, rubbery lymphadenopathy
- B symptoms (systemic sx important in staging)- fever (39C), wt loss >10% over 6 months, drenching night sweats
diagnosis of HL
-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
NHL
60% of lymphomas in children and adolescents
NHL- can be secondary to?
SCID (severe combined immune def)
-Wiscott-Aldrich syndrome (X-linked recessive)- recurrent sinopulm infections, eczema, bleeding (thrombocytopenia)
NHL- most common subtype
-Burkitt lymphoma
Burkitt lymphoma- commonly manifests as?
-abd (sporadic type) or head and neck dz (endemic type with involvement of BM or CNS
leukemia- results from?
-malignant transformation and clonal expansion of hematopoietic cells at an early stage of differentiation that are then unable to undergo further maturation
regional collections of nodes
- cervical
- supra-clavicular
- axillary
- inguinal
lymphadenopathy- most important component of the work-up
-history and physical
size of nodes
- > 2 cm is enlarged
- supraclavicular is not normal
quality and location of LNs
- lymphadenitis- tender, warm, red, fluctuant
- benign- soft, mobile, smooth
- lymphoma- firm and rubbery
- malignancy or Tb- fixed and matted
- if generalized- think systemic dz!
LDH- marker for>
hematologic manifestations
testicular mass- must do what?
w/o hx of trauma/injury- CHEST RADIOGRAPH is indicated
teratoma
- sacrococcygeal most common
- benign, well-diff cystic lesions (mature)
- lesions of indeterminate potential (immature)
- unequivocally malignant teratomas (mixed with another germ cell tumor component)
neuroblastoma- is what?
- tumors of symp ganglia and adrenal medulla
- derived from primordial neural crest cells
- most common extracranial solid tumor of childhood!!!
neuroblastoma- clinical course
- <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
Wilms tumor
- most common primary renal tumor of childhood!!
- age 2-5
WAGR syndrome
- wilms tumor, aniridia, genital anomalies, mental retardation
- 33% of developing wilms tumor
Denys-Drash syndrome
- gonadal dysgenesis (male pseudohermaphroditism), early onset nephropathy, mutation in zinc-finger region of WT1
- 90% chance of developing Wilms tumor
Beckwith-Wiedemann syndrome
- enlargement of body organs
- macroglossia
- hemihypertrophy
- omphalocele
- abnormal large cells in adrenal cortex
