Intro to WBCs and WBC disorders

Question 1

relative WBC counts

Answer 1

see notes

Question 2

how to calculate ANC

Answer 2

ANC=WBCx(%neutrophils+%bands)

Question 3

bandemia

Answer 3

increase in bands

Question 4

distribution of neutrophil pools

Answer 4

storage pool = 65% in bone marrow
circulating pool = 5% (in CBC)
marginal pool = 5% (in small blood vessels, along walls)

Question 5

shift neutrophilia

Answer 5

demargination of marginal pool w/out prolif

one possible cause is steroids

Question 6

leukomoid rxn

Answer 6

demargination and marrow proliferation

- increase in both mature & immature forms

Question 7

leukoerythroblastic rxn

Answer 7

premature release of precursor cells
CBC and smear: left shift, early RBC forms (nucleated, reticulocytes), tear drop cells
cause: marrow infiltration, myelofibrosis

Question 8

congenital causes of neutropenia

Answer 8

cyclic neutropenia: autosomal dom
severe congenital neutropenia (SCN): autosomal dom, responds to G-CSF
kostman syndrome: autosomal recessive SCN

Question 9

classifications of neutropenia

Answer 9

ANC 1000-1500 = mild. outpatient management
ANC 500-100 = moderate. increased risk, but still out pt.
ANC <200 = very dangerous

Question 10

congenital disorders of neutrophil function

Answer 10

leukocyte adhesion deficiency
hyperimmunoglobin E syndrome -
Chediak-Higashi syndrome
Chronic granulomatous disease

Question 11

leukocyte adhesion deficiency

Answer 11

prob w/ neutrophil function –> recurrent bacterial infections
neutrophilia w/ NO PUS
tx: stem cell transplant

Question 12

hyperimmunoglobulin E syndrome

Answer 12

problem w/ neutrophil function (chemotaxis)
high IgE
tx supportive w/ abx

Question 13

chediak-higashi syndrome

Answer 13

problem w/ neutrophil function (degranulation)
pyogenic infections
partial albinism
tx: steroids, chemo, stem cell transplant

Question 14

chronic granulomatous disease

Answer 14

problem w/ neutrophil function (bacterial killing–lacks H2O2 and superoxide burst)
pyogenic infections w/ granuloma formations
tx: abx prophylaxis

Question 15

acquired disorders of neutrophil function (3)

Answer 15

1. myelodysplasia/myelodysplastic syndrome
2. alcoholism
3. metabolic disorders

Question 16

causes of eosinophilia

Answer 16

NAACP
N - neoplasm
A- allergies
A - asthma
C - collagen vascular diseases & vasculitis
P - parasitic infection

Question 17

criteria for significant eosinophilia

Answer 17

eosinophil count >1500 for 6 wks

Question 18

primary eosinophilia causes

Answer 18

acute eosinophilic leukemia (related to AML)

chronic eosinophilic leukemia - may be responsive to imatinib/Gleevac

Question 19

basophilia - when do you see it?

Answer 19

rare

seen in CML

Question 20

monocyte function

Answer 20

release granules to kill microorganisms

involved in chronic phase of infection

Question 21

monocytopenia - causes

Answer 21

severe infections
bone marrow failure
hairy cell leukemia

Question 22

smudge cells

Answer 22

think EBV/mono

Question 23

causes of lymphocytosis

Answer 23

non-malignant: acute or chronic infection, drug hypersensitiviy
malignant: CLL, PLL, lymphoma, ALL –> CLONAL

Question 24

light chain restriction

Answer 24

indicates clonality of B cells

Question 25

how to tell if lymphocytosis is caused by T cell over proliferation

Answer 25

PCR or western blot for T cell receptor (no surface markers)