13. Disorders of WBCs Flashcards
If the absolute numbers look ok on a blood count, should you stop there? or is there another test?
may need to test FUNCTION of neutrophils as well as numbers, percentages. can have dysfunction even with normal counts
when to worry about dysfunctional neuts even if absolute counts are ok?
- recurrent bacterial infections
- infections of unusual sites (brain,liver)
- infections with unusual pathogens (fungal)
- Chronic gingivitis or aphthous ulcers
if you have a young kid with recurrent infections and an ok absolute count, what should you be thinking of?
congenital disorder of neutrophils
Leukocyte Adhesion Deficiency. defect of what? sx? tx?
- defect of adhesion and rolling.
- recurrent bacterial infections
- neutrophilia, NO PUS
- tx: stem cell transplant
Hyperimmunoglobulin E syndrome: defect? sx? will be high in what? tx?
- defect of chemotaxis.
- sx are chronic dermatitis (eczema), recurrent staph, candidal infections. may also be facial abnormalities
- High IgE levels
- Tx generally supportive with prophy abx
Chediak-Higashi Syndrome: defect? sx? comorbid condition? tx?
- degranulation defect: abnormal granules with impaired delivery of lysosomal enzymes to phagosomes, result in slow killing.
- recurrent pyogenic (pus) infections
- kids also have partial albinism
Chronic Granulomatous Disease: defect? sx? tx?
- defect with bacterial killing. inability to generate neutrophil oxidative burst that produces ROS and bactericidal agent hypochlorous acid
- recurrent pyogenic infectiosn of skin, lungs, formation of granulomas
- tx with prophy abx
if I see eczema, what should I think?
hyperimmunoglobulin E
myelodysplasia/myelodysplastic syndrome: type of disorder? what will neut count be?
- clonal disorder, dysfunction of neuts
- neut count will be decreased OR normal
- other cell lines (plts, RBCs) may be affected
alcoholism: affect on neutrophilis
decreased motility and phagocytosis
metabolic disorders: affect on neut function?
decr adhesion, motility, phagocytosis.
disorder could be diabetes, uremia, malnutrition, autoimmune
what could a dysplastic PMN look like?
might be bi-lobar rather than with 3-4 lobes
eosinophil: what does it do? is it granular? military analogy?
phagocytic cell, primary function is to kill parasites.
has dense red granules.
also involved in hypersensitivity rxns
“special ops”
what problems associated with a low eosinophil count?
low eosinophil count not clinically sig.
eosinophilia: most common cause globally
parasitic infections
eosinophilia: most common cause in dev countries?
atopic allergic disease
Causes of eosinophilia?
N: neoplasm (Hodgkins, Lymphoma, CML, Primary eosinophilic disorders)
A: Allergies
A: asthma
C: collagen vascular diseases and vasculitis
P: parasitic infection
what is the approach to eosinophilia?
more of a history than any test.
atopy, travel, pets, constitutional symptoms (fever, wt loss), drugs/toxins, autoimmune, connective tissue
eosinophilia: tests to run?
CBC, differential
stools for ova, parasites
connective tissue serology (think rheum process)
bone marrow/tissue biopsy
basophil: function? when is basophilia seen?
uncommon cell. disorders are rare
function in hypersensitivity rxns, releases histamine and leukotriends.
basophilia seen with myeloproliferative disorders (Chronic Myelogenous Leukemia: CML)
monocytes: function? when do they get involved? military analogy?
phagocytes, release granules to kill micro-organisms. involved in more chronic phase of infections.
military: reinforcements
what is monocytosis?
too many monocytes
what can cause monocytosis?
- chronic infections
- bone marrow recovery after chemo
- autoimmune and CT disease
- granulomatous disease (sarcoid)
- malignancy
what are some causes of monocytopenia?
uncommon, but:
severe infections/sepsis
bone marrow failure
hairy cell leukemia (agent orange)
