wbc disorders Flashcards
neutrophilia >7x10*9/l
increased neutrophils not path. normal in newborns transient no increased bands
acute neutrophilia
bact. infec./ toxin
BM response
shift left increase in bands
chronic neutrophilia
follows acute cont. stimulation bm storage depletion increase in mitotic pool shift left: toxic granulation, dohle bodies increased metamyelocytes and myelocytes
leukmoid rxn neutrophilia
extreme rxn to severe infec necrotic tissue >25K many immature cells (bands, meta, myelo) must distinguish from CML
leukemoid rxn vs. CML
Leukemoid: norm rbc & plt, LAP increased, toxic gran., dohle bodies
CML: ab. rbc & plt (decreased rbc, wbc, plt), LAP decreased, no toxic gran., no dohle bodies, increased eos and basos
Other causes of neutrophilia
tissue necrosis burns rheumatoid arthritis tumors steroids pregnancy
Causes of neutropenia <2x109/L/ <1.5x109/L
decreased bm stem cell disorders chemo/radiation myelophthisic anemia megaloblastic anemai inherited
neutropenia: increased WBC loss
severe infec. immune loss hypersplenism hemodialysis viral infec
neturopenia: pseudoneutropenia
increase mgp
transient
some infec. and hypersensitivity rxns
Chronic Granulomatous disease
rare, inherited
ab oxidative metabolism
recurrent infec.
Nitroblue tetrazolium test: detects ab O2 metabolites (+blue)
Chediak Higashi anomaly
rare, inherited
death in infancy due to infec
fusion of 1* and 2* granules= ab lysosomes
locomotion, delayed de-granulation, decreased killing
hypopigmentation
big blue inclusions in neutrophils
neutropenia: agranulocytosis
<0.5 x 10*9/L
agranulocytic= not good for patient
May-Hegglin anomaly
rare inherit. large basophilic inclusions in cytoplasm maybe thrombocytopenia and giant plts maybe bleeding usually asymp
Pelger Huet anomaly
bening inherited ad hyposegs bi lobed segs and increase in bands pince nez cells normal cell fx acquired w/ MPD/MDS must diff. from true left shift
Alder Reilly anomaly
inherited large purple granules in cyto of wbc ppt mucopolysaccharides enzyme deficency normal cell fx
myeloperoxidase deficiency
benign inherited
no increase in infec
causes tech. errror in instruments that use peroxidase to id wbc
use other microbial to kill organisms (diabetic may have increase in C. albicans infec)
leukocyte adhesion deficiency
rare inhert.
absence of wbc surface adhesion prot. (integrins)
defects in adhesion, chemotaxis, phag., respiratory burst, degran.
frequent infec
high mortality rate
systemic lupus erythematosis (SLE)
connective tissue disease butterfly rash anti dna acts on wbc (LE factor) change in nucleus round homogenous body is ingested by neutrophil (LE cell)
lab testing for SLE: clot method
wbc treated to free nuclei LE factor acts on nuclei neut. phag. nuclei smears made and stained differentiate from tart cell
lab testing for SLE: ANA testing
more specific anti nuclear antibody (LE factor)
eosinohils
1* tissue cells very motile phag. defense against parasites anti infam. cells (allergy/asthma) increased tryptophan= increased eos
eosinophilia >450/cumm
parasite infec allergy asthma hypersensitive skin disorder hematologic malignancy tryptophan
hypereosinophilic syndrome >1500/cumm
tissue infiltration and damage through release of gran.
Charcot Leyden crystals (can damage tissues and heart)
eosinopenia
hard to define
no eos associated w/ acute infec/ inflamm.
glucocorticosteroids and epinephrine inhibit eos release from bm and increase margination
acute stress/shock
Cushing’s synd.
basophils
limited motility water soluble granules granules contain heparin and histamine modulate inflamm. response receptors for IgE tissue baso= mast cell
basophilia >150/cumm
immediate hypersensitivity rxn and MPD
malignancy
basopenia
difficult to establish
due to inflamm. and immunologic rxns
monocytosis >800/cumm
inflamm. and malignancies recovery stage of acute infec. and agranulocytosis immune cellular response in TB non bact infec. collagen vascular disorders fungal infec
monocytopenia <200/cumm
hard to establish
stem cell disorders
lipid storage disorders
monocytes/macrophages
enzyme necessary to digest phag. material is deficient
Gaucher’s, Nieman-Pick, Tay-Sachs, Sea Blue Histiocyte
Gaucher’s disease
accum. of lipid in macro. inherited deficiency of glucocerebrosidase pancytopenia splenomegaly and bone pain increase serum phosphate Gaucher cell- tissue paper cell in bm and tissue
Nieman Pick disease
rare inherited jewish pop. deficiency of sphingomyelinase foamy macro. poor physical develop., hepatosplenomegaly fatal by age 3
Tay-Sachs disease
rare inherited deficiency of b-hexoseamidase accum. of glycolipids and mucopolysaccharides affects cns fatal by age 4
Sea Blue Histiocyte syndrome
inherited
benign
splenomegaly and decreased plts
sea blue staining macro in spleen and bm
lymphocytosis
relative, absolute, or both
kids norm. high lymphs, puberty it decreases
usually self limiting due to infec/ inflamm
lymphocytosis: infectious lymphocytosis
contagious
young kids
Adenovirus/ Coxsackie A virus
GI, resp., fever, headache, dizzy, painful neck
increased leukocytes, large #s of normal lymphs
lymphocytosis: whooping cough
Bordatella pertusis
increase leukocytes, large #s of normal lymphs
may see neutrophilia w/ toxic
plasmacytosis
b cells
plasma cells not in PB
intense stimulation of immune system w/ increased gamma globulin
rubeola, plasma cell leukemia, multiple myeloma
Mott cell (russell bodies)
flame cell
plasmacytoid lymphs
nucleus off to one side
blue cyto
halo around nucleus
nucleus spoke of a wheel
infectious mononucleosis
Epstein Barr virus
14-24yrs
EBV infects B cells, Tc cells inhibit proliferation and participate in cellular immunity
lethargy, headach, fever, chills, sore throat, lymphadenopathy, hepatosplenomegaly
infectious mononucleosis lab results
leukocytosis 12-25K/cumm >50%lymphs lots of reactive lymphs (t cells) heterogenous population of cells heterophile Ab+
cytomegalovirus (CMV)
herpes grp virus congenital/ acquried most common viral infec that complictes tissue transplants dangerous to newborns like mono w/out sore throat heterphile negative
toxoplasmosis
protozoan T. gondii ingestion of oocysts from cat feces leukocytosis increased lymphs reactive lymphs- similar to blasts or lymphoma cells heterophile =
lymphocytopenia
inability to mount response to infec. decreased prod./ increased destruction steriods cancer acute inflamm infect SLE chemo/radia malnutrition immune deficiency disorders
AIDS
HIV 1
infect w/ opportunistic organ.
malignancies (Kaposi’s sarcoma of blood vessels)
trans. thru blood/sex
weight loss, night sweats, fever, diarrhea, infec.
pancytopenia, decreased lymps, decreased Th cells (CD4)
no cure= AZT used
SCIDS (severe combined immune deficiency syndrome)
inherited sex linked deficiency of T and B cells decreased lympoid tiss in nodes/bm recurrent infec., rash, failure to thrive, death to sepsis in 2 yrs bm transplant bubble boy
Wiskott Aldrich syndrome
sex linked
decreased Ab
eczema, thrombocytopenia, immune deficiency, death by age 10
bm transplant (if before 5 85% success)/supportive
DiGeorge syndrome
decreased tcells and lymphoid tiss normal bcells no thymus= hypoparathyroidism heart defects, hypocalcemia, recurrent infec thymic grafts
Bruton’s disease
sex linked agammaglobulinemia decreased bcells and plama cells normal tcells resp/skin infec w/ catalase = pyogenic bact gamma globulin injections
