270b neutrophil fxn

Question 1

neutrophil - stem cells?

Answer 1

HSC–> common myeloid progenitor (RBCs, platelets, mast cells) –> myeloblast –> neutrophils

Question 2

Neutropenia - number? causes?

Answer 2

Absolute neutrophil count < 1500 cells/mm3

Sepsis/postinfection, drugs (including chemotherapy), aplastic anemia, SLE, radiation

Question 3

Leukocyte adhesion deficiency (LAD 1) - defect? inheritance?

Answer 3

Defect in LFA-1 integrin (CD18) protein on phagocytes –> impaired migration and chemotaxis

autosomal recessive.

Question 4

Leukocyte adhesion deficiency (LAD 1) - presentation?

Answer 4

Recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord (>30 days).

Question 5

Leukocyte adhesion deficiency (LAD 1) - findings?

Answer 5

increased circulating neutrophils.

Absence of neutrophils at infection sites.

Question 6

Chédiak-Higashi syndrome -defect? inheritance?

Answer 6

Defect in lysosomal trafficking regulator gene (LYST). –> Microtubule dysfunction in
phagosome-lysosome fusion

autosomal recessive.

Question 7

Chédiak-Higashi syndrome - presentation?

Answer 7

Recurrent pyogenic infections by staphylococci and streptococci

partial albinism

peripheral neuropathy w/ progressive neurodegeneration

infiltrative lymphohistiocytosis.

Question 8

Chédiak-Higashi syndrome - findings?

Answer 8

Giant granules in neutrophils and platelets.

Pancytopenia.

Mild coagulation defects.

Question 9

Chronic granulomatous disease - defect? inheritance?

Answer 9

Defect of NADPH oxidase –> lower reactive oxygen species (e.g., superoxide) and absent respiratory burst in neutrophils

X-linked recessive.

Question 10

Chronic granulomatous disease - presentation and bacteria?

Answer 10

increased susceptibility to catalase+ organisms (PLACESS):

Pseudomonas
Listeria,
Aspergillus
Candida
E. coli,
S. aureus
Serratia.

Question 11

Chronic granulomatous disease - findings?

Answer 11

Abnormal dihydrorhodamine (flow cytometry) test.

Nitroblue tetrazolium dye reduction test is - (test out of favor)