pediatric WBC abnormalities Flashcards
White blood cell numbers?
Never Let Monkeys Eat Bananas
Neutrophils>Lymphocytes>monocytes>eosinophils>basophils
How do you calculate ANC? absolute neutrophil count
[(%neutrophils + %bands) x (WBC)]/100
if ANC is below what number, the risk of serious infection is high?
<500
clinical manifestation of Severe congenital neutropenia (Kostmann syndrome)?
life threatening pyogenic infections, often in infancy
What is the defect and bone marrow eval for Kostmann syndrome?
impaired myeloid differentiation so Bone marrow demonstrates myeloid maturation arrest
Kostmann syndrome, 60-80% show what mutations
neutrophil ELA-2
patients with Kostmann Syndrome have increased risk of what
AML
Clinical manifestations of cyclic neutropenia
- cyclic fever
- oral ulcers
- gingivitis
- periodontal disease
- recurrent bacterial infections
What is the time frame for cyclic neutropenia
for 3-7 days every 3 weeks (15-35 days)
mutations for cyclic neutropenia
ELA-2 in 80-90%
Triad of neutropenia, exocrine pancreatic insufficiency, and skeletal abnormalities
Shwachman-Diamond syndrome
Shwachman-Diamond syndrome patients have increased risk of what?
myelodysplastic syndrome or leukemia
Fanconi anemia
congenital disorder associated with neutropenia
- bone marrow failure syndrome
- GU and skeletal abnormalities
- increased chromosome fragility
- Delayed separation of umbilical cord
- recurrent and severe bacterial and fungal infections without pus accumulation
- poor wound healing
- periodontal disease
Leukocyte adhesion deficiency
- Recurrent purulent infection with fungal or bacterial catalase-positive organisms, usually starting in infancy,
- defect in oxidative metabolism
Chronic granulomatous disease
