White Cell Disorders

Question 1

define neutropenia

Answer 1

absolute dec in number of circulating neutrophils so dec ANC (absolute neutrophil count)

Question 2

what is the lower limit of ANC

Answer 2

1500

Question 3

neutropenia classifications

Answer 3

mild 1000-1500
moderate 500-1000
severe less than 500

Question 4

etiology of neutropenia

Answer 4

defects in myelopoiesis, drugs, infections, autoantibodies

Question 5

what does a neutrophil do

Answer 5

primary cell in immune response to pyogenic (pus) organisms and predominate cell in acute inflammatory infiltrates: so they are the big guns

Question 6

what does neutropenia inc your susceptibility to

Answer 6

bacterial and fungal infections
skin and oral cavity most commonly affected
sepsis is a common complication (morbidity and mortality)
signs and symptoms may be altered with neutropenic patient: infection may not look normal because don’t have and WBCs to fight it so no pus forming no inflammation maybe…

Question 7

can you have normal WBC and still have neutropenia

Answer 7

yes so look at differential

Question 8

other names for neutrophils

Answer 8

granulocytes (GRAN%)
segmented neutrophils (SEG%)

Question 9

what are “bands” or banded neutrophils

Answer 9

less mature neutrophils
mature neutrophils are segmented
bands can be listed may or may not be listed separately in differentiated CBC

Question 10

gene mutations of severe congenital neutropenia (SCN)

Answer 10

autosomal dominant (ELA2 gene) or
recessive disorder (HAX1 gene)

Question 11

characteristics/clinical presentation of severe congenital neutropenia (SCN)

Answer 11

ANC < 200 from birth

recurrent fevers and infections from early infancy

Question 12

treatment of severe congenital neutropenia (SCN)

Answer 12

as soon as possible: start granulocyte colony stimulating factor (G-CSF): given as a shot
fever precautions
appropriate oral/hand hygiene
annual surveillance of marrow
managed by specialist

Question 13

define cyclic neutropenia

Answer 13

regular, periodic oscillations in ANC
every 21 days or so (period for your neutrophils!)
classically, monocyte count inc with dec ANC

Question 14

gene mutation of cyclic neutropenia

Answer 14

autosomal dominant inheritance (ELA2 mutation)

Question 15

diagnosis of cyclic neutropenia

Answer 15

identifying cycle or genetic testing

Question 16

can cyclic neutropenia present later in life

Answer 16

yes, with Hx of recurrent mouth sores and fever

Question 17

treatment of cyclic neutropenia

Answer 17

granulocyte colony stimulating factor (G-CSF)

managed by specialist

Question 18

characteristics of autoimmune neutropenia (AIN)

Answer 18

isolated neutropenia: no other underlying condition causing neutropenia: immune system attacking neutrophils

Question 19

who normally gets autoimmune neutropenia (AIN)

Answer 19

young kids mostly: self resolving condition

Question 20

clue it may be autoimmune neutropenia (AIN)

Answer 20

neutropenia is out of proportion to infectious Hx

ie) ANC < 200 but otherwise healthy kid

Question 21

diagnosis of autoimmune neutropenia (AIN)

Answer 21

anti-neutrophil antibody is helpful but not always diagnostic
followed by specialist to rule out other causes of neutropenia

Question 22

treatment of autoimmune neutropenia (AIN)

Answer 22

can be self resolving
rarely use G-CSF
follows fever precautions

Question 23

what are causes of secondary autoimmune neutropenia (AIN)

Answer 23

broader autoimmune disorders (i.e.: Lupus or Sjogren’s)
infection
medications (hydralazine or procainamide)

Question 24

ethnic neutropenia

Answer 24

benign mild-moderate neutropenia
mostly in Africans, Jewish, and Arab popln
otherwise normal leukocytes/bone marrow
NO Hx or RISK of recurrent infections
does NOT require specialist care

Question 25

other causes of neturopenia

Answer 25

``` acquired or inherited BMF infection drug immune dysfunction neonatal alloimmune neutropenia metabolic disorders nutritional deficiencies (vit B12 or folate) marrow infiltration (tumors) ```

Question 26

is severe neutropenia in an ill or febrile patient an emergency

Answer 26

yes

Question 27

can neutropenia (even severe) be observed initially in an otherwise healthy person

Answer 27

yes

Question 28

diagnostics for neutropenia

Answer 28

blood cultures CBC with differential Hx and physical

Question 29

what are the other granulocytes besides neutrophils

Answer 29

basophils and eosinophils

Question 30

results of dec absolute eosinophil count so less than 500

Answer 30

``` allergy asthma parasitic infections malignancies rheumatologic disorders immunodeficiencies hypereosinophilic syndrome ```

Question 31

results of basophilia

Answer 31

hypersensitivity reactions anaphylaxis infections chronic myelogenous leukemia (CML)

Question 32

agranulocytes (other leukocytes)

Answer 32

lymphocytes and monocytes

Question 33

what are lymphocytes

Answer 33

``` T cells B cells NK cells NKT cells primary cell of specific immune recognition memory aspect differentiate between self and non-self ```

Question 34

disorders/diseases of lymphocytes

Answer 34

lymphocytosis (infection especially viral and leukemias) | lymphopenia (rheumatologic diseases)

Question 35

what are monocytes

Answer 35

have multiple functions including phagocytosis and antigen presentation

Question 36

disorders/diseases of lymphocytes

Answer 36

``` monocytosis: infections like SBE or TB malignancies rheumatologic diseases SCN ```