Leukocyte Disorders

Question 1

What disease is characterized by neutropenia, giant granules abnormally released, abnormal microtubule assembly, and a CHS gene auto recessively inherited?

Answer 1

Chediak-Higashi Syndrome

Question 1

Defects in ____ have an assoc. increased risk for Neisseria bacteria (meningitis, arthritis, sepsis).

Answer 1

C5-C9

Question 2

What is Myeloperoxidase Deficiency?

Answer 2

deficiency of myeloperoxidase; mild defect in killing bacteria but major defect in killing candida; a post-translational defect in protein processing; auto recessive

Question 2

Deficiencies of factors 1q, 4, and 2 are assoc. with an increased risk of ____ and other autoimmune and inflammatory vascular diseases.

Answer 2

SLE

Question 2

Deficiency of ____ causes insufficient opsonization of bacteria and recurrent infections.

Answer 2

C3

Question 4

What disease is characterized by neutrophilia, defective oxidase enzymes and no toxic O2 metabolites produced, deficiency of b558 and -phox gene product, and an auto-recessive inheritance?

Answer 4

chronic granulomatous disease (CGD)

Question 5

For severe neutropenia, treat with _____.

Answer 5

G-CSF

Question 7

What is chronic granulomatous disease (CGD)?

Answer 7

neutrophilia; defective oxidase enzymes and no toxic O2 metabolites produced; deficiency of b558 and -phox gene product; auto-recessive inheritance.

Question 8

Deficiency of C3 causes insufficient ______ and recurrent infections.

Answer 8

opsonization of bacteria

Question 10

What is leukocyte adhesion deficiency I?

Answer 10

neutrophilia; decreased adhesion to endothelium; auto recessive deficiency of CD18

Question 11

Defects in C5-C9 have an assoc. increased risk for ____(meningitis, arthritis, sepsis).

Answer 11

Neisseria bacteria

Question 12

Deficiencies of factors ___, ____, and ____ are assoc. with an increased risk of SLE and other autoimmune and inflammatory vascular diseases.

Answer 12

1q, 4, and 2

Question 13

What disease is characterized by a deficiency of myeloperoxidase, mild defect in killing bacteria but major defect in killing candida, a post-translational defect in protein processing, and is auto recessive?

Answer 13

Myeloperoxidase deficiency

Question 14

What disorder is characterized by neutrophilia, decreased adhesion to endothelium, and an auto recessive deficiency of CD18?

Answer 14

leukocyte adhesion deficiency I

Question 15

What is Chediak-Higashi Syndrome?

Answer 15

neutropenia; giant granules abnormally released; abnormal microtubule assembly; CHS gene auto recessive