White Cell Disorders Flashcards
define neutropenia
absolute dec in number of circulating neutrophils so dec ANC (absolute neutrophil count)
what is the lower limit of ANC
1500
neutropenia classifications
mild 1000-1500
moderate 500-1000
severe less than 500
etiology of neutropenia
defects in myelopoiesis, drugs, infections, autoantibodies
what does a neutrophil do
primary cell in immune response to pyogenic (pus) organisms and predominate cell in acute inflammatory infiltrates: so they are the big guns
what does neutropenia inc your susceptibility to
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…
can you have normal WBC and still have neutropenia
yes so look at differential
other names for neutrophils
granulocytes (GRAN%) segmented neutrophils (SEG%)
what are “bands” or banded neutrophils
less mature neutrophils
mature neutrophils are segmented
bands can be listed may or may not be listed separately in differentiated CBC
gene mutations of severe congenital neutropenia (SCN)
autosomal dominant (ELA2 gene) or recessive disorder (HAX1 gene)
characteristics/clinical presentation of severe congenital neutropenia (SCN)
ANC < 200 from birth
recurrent fevers and infections from early infancy
treatment of severe congenital neutropenia (SCN)
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
define cyclic neutropenia
regular, periodic oscillations in ANC
every 21 days or so (period for your neutrophils!)
classically, monocyte count inc with dec ANC
gene mutation of cyclic neutropenia
autosomal dominant inheritance (ELA2 mutation)
diagnosis of cyclic neutropenia
identifying cycle or genetic testing
can cyclic neutropenia present later in life
yes, with Hx of recurrent mouth sores and fever
treatment of cyclic neutropenia
granulocyte colony stimulating factor (G-CSF)
managed by specialist
characteristics of autoimmune neutropenia (AIN)
isolated neutropenia: no other underlying condition causing neutropenia: immune system attacking neutrophils
who normally gets autoimmune neutropenia (AIN)
young kids mostly: self resolving condition
clue it may be autoimmune neutropenia (AIN)
neutropenia is out of proportion to infectious Hx
ie) ANC < 200 but otherwise healthy kid
diagnosis of autoimmune neutropenia (AIN)
anti-neutrophil antibody is helpful but not always diagnostic
followed by specialist to rule out other causes of neutropenia
treatment of autoimmune neutropenia (AIN)
can be self resolving
rarely use G-CSF
follows fever precautions
what are causes of secondary autoimmune neutropenia (AIN)
broader autoimmune disorders (i.e.: Lupus or Sjogren’s)
infection
medications (hydralazine or procainamide)
ethnic neutropenia
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
other causes of neturopenia
acquired or inherited BMF infection drug immune dysfunction neonatal alloimmune neutropenia metabolic disorders nutritional deficiencies (vit B12 or folate) marrow infiltration (tumors)
is severe neutropenia in an ill or febrile patient an emergency
yes
can neutropenia (even severe) be observed initially in an otherwise healthy person
yes
diagnostics for neutropenia
blood cultures
CBC with differential
Hx and physical
what are the other granulocytes besides neutrophils
basophils and eosinophils
results of dec absolute eosinophil count so less than 500
allergy asthma parasitic infections malignancies rheumatologic disorders immunodeficiencies hypereosinophilic syndrome
results of basophilia
hypersensitivity reactions
anaphylaxis
infections
chronic myelogenous leukemia (CML)
agranulocytes (other leukocytes)
lymphocytes and monocytes
what are lymphocytes
T cells B cells NK cells NKT cells primary cell of specific immune recognition memory aspect differentiate between self and non-self
disorders/diseases of lymphocytes
lymphocytosis (infection especially viral and leukemias)
lymphopenia (rheumatologic diseases)
what are monocytes
have multiple functions including phagocytosis and antigen presentation
disorders/diseases of lymphocytes
monocytosis: infections like SBE or TB malignancies rheumatologic diseases SCN
