CIS: Non-neoplastic WBC Disorders in Pediatrics

Question 1

How long do RBCs and WBC last?

Answer 1

RBC: 120 days
WBC: not long

Question 2

How do you document a CBC?

Answer 2

make a sideways stick figure with two sets of legs instead of a head at the other end

• WBC, HGB, Platelets, HCT starting at left and going clockwise
• put the differentiation to the side

Question 3

in early iron deficiency anemia, how will the RDW look?

Answer 3

a little elevated

-so look at the differential (the bands, and segs and whatnot)

Question 4

What pneumonic do we use for white blood cell numbers

Answer 4

Never Let Monkeys Eat Bananas

-Neutrophils>Lymphocytes>Monocytes>Eosinophils>Basophils

Question 5

Absolute Neutrophil Count *

Answer 5

ANC= (%neutrophils+%bands)*WBC/100

-remember to move the decimal over 3 places to the right in the WBC because it’s K/uL

Question 6

What is the significant value for the ANC?

*

Answer 6

<500

-the risk of serious infection is high

Question 7

Cyclic Neutropenia*

Answer 7

• cyclic fever, oral ulcers, gingivityis, perodontal disease, recurrent bacterial infections
• defect in stem cell regulatory defect resulting in defective maturations
• sporadic or auto dominant
• CBC 2-3 times weekly for 6-8 weeks to document cycles and nadir, ELA-2 mutation in 80-90%

Question 8

Shwachman-Diamond syndrome*

Answer 8

• triad of neutropenia, exocrine pancreas insufficiency, and skeletal abnormalities (feet, hands)
• may have defects in neutrophil mobility migration and chemotaxis in addition to neutropenia
• Auto recessive
• increased risk for myelodysplastic disease or leukemia
• the only other thing that causes pancreatic insufficiency is cystic fibrosis

Question 9

Causes of Eosinophilia

Answer 9

DNAAACP

• drugs
• neoplasm
• atopic disease
• …. finish this

Question 10

Fanconi anemia*

Answer 10

• bone marrow failure syndrome
• GU and skeletal abnormalities
• increased chromosome fragility

Question 11

Leukocyte adhesion deficiency

Answer 11

• delayed separation of umbilical cord, recurrent and severe bacterial and fungal infections without pus accumulation, poor wound healing, periodontal disease
• neutrophils have diminished adhesion to surfaces and cannot migrate out of blood vessels
• very rare
• inheritance autosomal recessive
• prognosis depends on severity of deficiency

Question 12

Chronic granulomatous disease

Answer 12

• recurrent purulent infections with fungal or bacterial catalse-+ organisms, usually starting in infancy, chronic inflammatory granulomas
• defect in oxidative metabolism, absent generation of superoxide
• diagnosed with nitroblue tetrazolium test

Question 13

When I say infection-associated neutropenia, you say…

Answer 13

parvovirus B19