270b neutrophil fxn Flashcards Preview

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Flashcards in 270b neutrophil fxn Deck (11):
1

neutrophil - stem cells?

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

2

Neutropenia - number? causes?

Absolute neutrophil count < 1500 cells/mm3

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

3

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

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

autosomal recessive.

4

Leukocyte adhesion deficiency (LAD 1) - presentation?

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

5

Leukocyte adhesion deficiency (LAD 1) - findings?

increased circulating neutrophils.

Absence of neutrophils at infection sites.

6

Chédiak-Higashi syndrome -defect? inheritance?

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

autosomal recessive.

7

Chédiak-Higashi syndrome - presentation?

Recurrent pyogenic infections by staphylococci and streptococci

partial albinism

peripheral neuropathy w/ progressive neurodegeneration

infiltrative lymphohistiocytosis.

8

Chédiak-Higashi syndrome - findings?

Giant granules in neutrophils and platelets.

Pancytopenia.

Mild coagulation defects.

9

Chronic granulomatous disease - defect? inheritance?

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

X-linked recessive.

10

Chronic granulomatous disease - presentation and bacteria?

increased susceptibility to catalase+ organisms (PLACESS):

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

11

Chronic granulomatous disease - findings?

Abnormal dihydrorhodamine (flow cytometry) test.

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