Page 458 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

442 PART 6 ■ Neoplastic Disorders

ture ly phocytes, ly phocytosis, on peripher l ■ “Low count” MBL h s signif c nt i erences ro CLL

bloo f l . with extre ely li ite , i ny, ch nce o progression. “High

■ M lign nt ly phoproli er tive isor ers re ch r cterize count” MBL nee s to be onitore . It rese bles the phe-
by n ccu ul tion o ly phocytes. notypic n epigenetic / olecul r e tures o R i st ge 0.

■ CLL n SLL re neopl s s co pose o s ll B ly pho-
cytes in the peripher l bloo , bone rrow, spleen, n B-Cell Prolymphocytic Leukemia

ly ph no es, ixe with proly phocytes n p r i u-

nobl sts or ing proli er tion centers in tissue inf ltr tes. ■ B-PLL represents lign ncy o B proly phocytes

In contr st to CLL, SLL is the ter use or nonleuke ic ecting bloo , bone rrow, n spleen.

p tients with the tissue orphology n i unopheno- ■ Proly phocytic leuke i is ch r cterize by l rge nu ber

type o CLL. o s ll ly phocytes with sc nt cytopl s n the i ture

■ CLL is the ost co on or o leuke i in ults in e tures o proly phocytes in the peripher l bloo .
Western countries, but it is very r re in r E stern coun-

tries. CLL n SLL ccount or l ost 7% o NHLs in Hairy Cell Leukemia

biopsies. ■ HCL is n unco on chronic ly phoproli er tive isor-

■ T e e i n ge o onset is 65 ye rs. er o the B-ly phocyte type.

■ More les th n e les (1.5 to 2.1:1) re icte by the ■ T is ture B-cell lign ncy is i gnose b se on clin-
isor er. ic l e tures, orphology, n phenotyping n gener lly

■ CLL h s the highest genetic pre isposition o ll he - tre te with cur tive intent.
tologic neopl s s. A ily pre isposition c n be ocu- ■ HCL is ore co on in les n usu lly ects p tients

ente in 5% to 10% o p tients with CLL. ol er th n 30 ye rs o ge.

■ Cl ssic CLL is usu lly B-cell isor er. It is ch r cterize
by gr u lly progressive ccu ul tion o orphologi- T-Cell and NK-Cell Neoplasms

c lly ture B ly phocytes in the bloo , bone rrow,

n ly ph tic tissues. ■ -PLLs re r re with poor prognosis.

■ More th n 90% o CLL cells re non ivi ing n rreste ■ Distinction ro CLL i gnosis requires ore th n 55%
t G or G in the cell cycle. o circul ting ly phoi cells to h ve the orphology o
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■ Molecul r genetics etection o geno ic re rr nge ents proly phocyte.
y not only ssist with the i gnosis but c n lso provi e ■ Proly phocytic leuke i is ch r cterize by l rge nu -

i port nt prognostic in or tion. ber o s ll ly phocytes with sc nt cytopl s n the

■ Alter tions in CLL ect ev sion o poptosis, sel - i ture e tures o proly phocytes in the peripher l
su ciency in growth, n sti ul tion o ngiogenesis n bloo . Cells o -PLL o en h ve pro inent nucleolus

isse in tion. e iu or s ll in size with convolute nucle r outlines.

■ CLL h s v ri ble clinic l course. Clinic l st ging syste s -PLL in peripher l bloo exhibits s ll to e iu ,
or ssessing prognosis in CLL were evelope in the e rly roun or irregul r nuclei rese bling Séz ry cells.

1980s, b se on e sily obt in ble biologic l n clinic l ■ Séz ry syn ro e is the leuke ic ph se o C CL, ycosis

p r eters. ungoi es.

■ Newer tre t ent che oi unother py w s intro uce ■ In peripher l bloo , the ise se is ch r cterize by the
in 2000. T is str tegy incorpor tes the use o onoclon l presence o bnor l circul ting ly phocytes, Séz ry

ntibo ies to che other py. T is str tegy ppe rs to pro- cells. A Séz ry cell is typic lly the size o s ll ly pho-

vi e or f rst ti e–observe surviv l benef t but is not cyte n h s rk-st ining, clu pe , nucle r chro tin

consi ere cur tive. p ttern. T e istinctive ol e , groove-like chro tin p t-

■ In the uture, p tient-specif c ther peutic rugs y be tern is escribe s cerebri or .
esigne or CLL p tients h rboring bnor lities in ■ -cell l rge gr nul r ly phocytic leuke i (LGL) h s

iRNA expression in their lign nt cells. present tion th t is si il r to CLL but is co pose o

■ Myelo bl tive high- ose che other py, with subsequent ture cells.
utologous or llogeneic SC , is the ost intensive or ■ It is i port nt to istinguish ro re ctive ly phocytosis

o che oi unother py or CLL. by the presence o n bnor l phenotypes or evi ence

o clon lity.

Monoclonal B-Cell Lymphocytosis ■ In ition, LGL ust be istinguishe ro in olent
chronic ly phoproli er tion isor ers o NK cells n

■ MLD is now known to prece e virtu lly ll c ses o ggressive NK-cell leuke i .

CLL/SLL. Although the newly up te Worl He lth ■ A LL is peripher l -cell neopl s c use by hu n -cell

Org niz tion (WHO) will ret in the current criteri or ly photropic virus-1 (H LV-1). T ere re our subtypes o

MLD, it oes e ph size th t the “low count” MLD periph- the ise se: cute, chronic, ly pho tous, n s ol ering.

er l bloo leukocyte count o less th n 0.5 × 10 /L ust be ■ In the peripher l bloo , tu or cells h ve hyperlob te
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istinguishe ro “high count” MBL. nuclei, so eti es with cloverle sh pe.