White blood Cell Disorders

Question 0

4 congenital neutrophil disorders

Answer 0

leukocyte adhesion deficiency
hyperimmunoglobin E syndrome
chediak-higashi syndrome
chronic granulomatous disease

Question 1

when to worry about dysfunction

Answer 1

recurrent bacterial infections
infections of unusal sites
infections with unusual pathogens
chronic gingivitis or aphthous ulcers

Question 2

acquired neutrophil disorders

Answer 2

myelodysplastic syndrome
alcoholism
metabolic disorders

Question 3

leukocyte adhesion deficiecny

Answer 3

adhesion and rolling (chemotactic) def; recurrent bacterial, no pus
treatment is stem cell transplant

Question 4

hyperimmunoglobulin e syndrome

Answer 4

chemotaxic defect
chronic dermatitis, recurrent staph and lung infections
high Ige levels
treatment- supportive with prophylactic antibiotics

Question 5

Chediak-Higashi syndrome

Answer 5

degranulation defect
recurrent pyogenic infections
children have partial albinism; treat with steroids, chemo, and stem cell transplant

Question 6

chronic granulomatous disease

Answer 6

bacterial killing defect- inability to generate neutrophil oxidative burst
recurrent infections of skin and lungs with granulomas
treatment- antibiotic prophylaxis

Question 7

primary job of eosinophils

Answer 7

kill parasites

Question 8

siginificant eosinophilia

Answer 8

absolute eosinophil count >1500 cells/ml for > 6 weeks

Question 9

severe eosinophilia

Answer 9

> 5000 cells/ ul

Question 10

most common cause eosinophilia worldwide

Answer 10

parasitic infections

Question 11

most common cause of eosinophilia in developed countries

Answer 11

atopic allergic diseases

Question 12

causes of eosinophilia

Answer 12

N-neoplasm (hodgkins, lymphoma, CML, primary eosinophilic disorders)
allergies (drugs, environmental allergens
asthma
Collagen vascular diseases & vasculitis
Parsitic infections

Question 13

acute eosinophilic leukemia

Answer 13

rare variant of acute myeloid leukemia

Question 14

chronic eosinophilic leukemia

Answer 14

some have FIP1L1/PDGFRA mutation that responds to tyrosine kinase inhibitor treatment with imatinib

Question 15

diagnostic appraoch for eosinophilia

Answer 15

complete H&P
CBC
serial stools
connective tissue serological studies such as ANA
bone marrow or tissue biopsy

Question 16

basophils

Answer 16

uncommon cell that functions in hypersensitivity reactions

releases histamine and leukotrienes

Question 17

basophilia=

Answer 17

CML

Question 18

monocytes

Answer 18

phagocytes that release granules to kill microrganisms

precursors to macrophages, histocytes, dendritic cells

Question 19

causes of monocytosis

Answer 19

chronic infections, bone marrow recovery after chemo, autoimmune/ct disorders, granulomatous disorders, malignancy

Question 20

causes monocytopenia

Answer 20

uncommon but seen with severe infections
bone marrow failure states
hairy cell leukemia

Question 21

first thing to do to establish if it is a primary lymphoproliferative disorder

Answer 21

establish clonality

Question 22

establish clonality of B cells

Answer 22

flow cytometry for cell surface markers; they will be light chain restricted ratio will be k:l with either K or L high

Question 23

cell surface marker all B cells have

Answer 23

cd 20

Question 24

clonality in T cells

Answer 24

PCR or westernblot

Question 25

causes of lymphocytopenia

Answer 25

congenital (wiskott-aldrich syndrome)
infectious
medication
hematologic malignancies

Question 26

pancytopenia

Answer 26

all three cell lines down