wbc disorders

Question 1

neutrophilia >7x10*9/l

Answer 1

increased neutrophils
not path.
normal in newborns
transient
no increased bands

Question 2

acute neutrophilia

Answer 2

bact. infec./ toxin
BM response
shift left increase in bands

Question 3

chronic neutrophilia

Answer 3

follows acute
cont. stimulation
bm storage depletion
increase in mitotic pool
shift left: toxic granulation, dohle bodies
increased metamyelocytes and myelocytes

Question 4

leukmoid rxn neutrophilia

Answer 4

extreme rxn to severe infec
necrotic tissue
>25K
many immature cells (bands, meta, myelo)
must distinguish from CML

Question 5

leukemoid rxn vs. CML

Answer 5

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

Question 6

Other causes of neutrophilia

Answer 6

tissue necrosis
burns
rheumatoid arthritis
tumors
steroids
pregnancy

Question 7

Causes of neutropenia <2x109/L/ <1.5x109/L

Answer 7

decreased bm
stem cell disorders
chemo/radiation
myelophthisic anemia
megaloblastic anemai
inherited

Question 8

neutropenia: increased WBC loss

Answer 8

severe infec.
immune loss
hypersplenism
hemodialysis
viral infec

Question 9

neturopenia: pseudoneutropenia

Answer 9

increase mgp
transient
some infec. and hypersensitivity rxns

Question 10

Chronic Granulomatous disease

Answer 10

rare, inherited
ab oxidative metabolism
recurrent infec.
Nitroblue tetrazolium test: detects ab O2 metabolites (+blue)

Question 11

Chediak Higashi anomaly

Answer 11

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

Question 12

neutropenia: agranulocytosis

Answer 12

<0.5 x 10*9/L

agranulocytic= not good for patient

Question 13

May-Hegglin anomaly

Answer 13

rare inherit.
large basophilic inclusions in cytoplasm
maybe thrombocytopenia and giant plts
maybe bleeding
usually asymp

Question 14

Pelger Huet anomaly

Answer 14

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

Question 15

Alder Reilly anomaly

Answer 15

inherited
large purple granules in cyto of wbc
ppt mucopolysaccharides
enzyme deficency
normal cell fx

Question 16

myeloperoxidase deficiency

Answer 16

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)

Question 17

leukocyte adhesion deficiency

Answer 17

rare inhert.
absence of wbc surface adhesion prot. (integrins)
defects in adhesion, chemotaxis, phag., respiratory burst, degran.
frequent infec
high mortality rate

Question 18

systemic lupus erythematosis (SLE)

Answer 18

connective tissue disease
butterfly rash
anti dna acts on wbc (LE factor)
change in nucleus
round homogenous body is ingested by neutrophil (LE cell)

Question 19

lab testing for SLE: clot method

Answer 19

wbc treated to free nuclei
LE factor acts on nuclei
neut. phag. nuclei
smears made and stained
differentiate from tart cell

Question 20

lab testing for SLE: ANA testing

Answer 20

more specific anti nuclear antibody (LE factor)

Question 21

eosinohils

Answer 21

1* tissue cells
very motile
phag.
defense against parasites
anti infam. cells (allergy/asthma)
increased tryptophan= increased eos

Question 22

eosinophilia >450/cumm

Answer 22

parasite infec
allergy
asthma
hypersensitive skin disorder
hematologic malignancy
tryptophan

Question 23

hypereosinophilic syndrome >1500/cumm

Answer 23

tissue infiltration and damage through release of gran.

Charcot Leyden crystals (can damage tissues and heart)

Question 24

eosinopenia

Answer 24

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.

Question 25

basophils

Answer 25

``` limited motility water soluble granules granules contain heparin and histamine modulate inflamm. response receptors for IgE tissue baso= mast cell ```

Question 26

basophilia >150/cumm

Answer 26

immediate hypersensitivity rxn and MPD | malignancy

Question 27

basopenia

Answer 27

difficult to establish | due to inflamm. and immunologic rxns

Question 28

monocytosis >800/cumm

Answer 28

``` inflamm. and malignancies recovery stage of acute infec. and agranulocytosis immune cellular response in TB non bact infec. collagen vascular disorders fungal infec ```

Question 29

monocytopenia <200/cumm

Answer 29

hard to establish | stem cell disorders

Question 30

lipid storage disorders

Answer 30

monocytes/macrophages enzyme necessary to digest phag. material is deficient Gaucher's, Nieman-Pick, Tay-Sachs, Sea Blue Histiocyte

Question 31

Gaucher's disease

Answer 31

``` 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 ```

Question 32

Nieman Pick disease

Answer 32

``` rare inherited jewish pop. deficiency of sphingomyelinase foamy macro. poor physical develop., hepatosplenomegaly fatal by age 3 ```

Question 33

Tay-Sachs disease

Answer 33

``` rare inherited deficiency of b-hexoseamidase accum. of glycolipids and mucopolysaccharides affects cns fatal by age 4 ```

Question 34

Sea Blue Histiocyte syndrome

Answer 34

inherited benign splenomegaly and decreased plts sea blue staining macro in spleen and bm

Question 35

lymphocytosis

Answer 35

relative, absolute, or both kids norm. high lymphs, puberty it decreases usually self limiting due to infec/ inflamm

Question 36

lymphocytosis: infectious lymphocytosis

Answer 36

contagious young kids Adenovirus/ Coxsackie A virus GI, resp., fever, headache, dizzy, painful neck increased leukocytes, large #s of normal lymphs

Question 37

lymphocytosis: whooping cough

Answer 37

Bordatella pertusis increase leukocytes, large #s of normal lymphs may see neutrophilia w/ toxic

Question 38

plasmacytosis

Answer 38

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

Question 39

plasmacytoid lymphs

Answer 39

nucleus off to one side blue cyto halo around nucleus nucleus spoke of a wheel

Question 40

infectious mononucleosis

Answer 40

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

Question 41

infectious mononucleosis lab results

Answer 41

``` leukocytosis 12-25K/cumm >50%lymphs lots of reactive lymphs (t cells) heterogenous population of cells heterophile Ab+ ```

Question 42

cytomegalovirus (CMV)

Answer 42

``` herpes grp virus congenital/ acquried most common viral infec that complictes tissue transplants dangerous to newborns like mono w/out sore throat heterphile negative ```

Question 43

toxoplasmosis

Answer 43

``` protozoan T. gondii ingestion of oocysts from cat feces leukocytosis increased lymphs reactive lymphs- similar to blasts or lymphoma cells heterophile = ```

Question 44

lymphocytopenia

Answer 44

``` inability to mount response to infec. decreased prod./ increased destruction steriods cancer acute inflamm infect SLE chemo/radia malnutrition immune deficiency disorders ```

Question 45

AIDS

Answer 45

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

Question 46

SCIDS (severe combined immune deficiency syndrome)

Answer 46

``` 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 ```

Question 47

Wiskott Aldrich syndrome

Answer 47

sex linked decreased Ab eczema, thrombocytopenia, immune deficiency, death by age 10 bm transplant (if before 5 85% success)/supportive

Question 48

DiGeorge syndrome

Answer 48

``` decreased tcells and lymphoid tiss normal bcells no thymus= hypoparathyroidism heart defects, hypocalcemia, recurrent infec thymic grafts ```

Question 49

Bruton's disease

Answer 49

``` sex linked agammaglobulinemia decreased bcells and plama cells normal tcells resp/skin infec w/ catalase = pyogenic bact gamma globulin injections ```