Page 435 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 419

CD38, n chro oso l berr tions 11q−, 13q−, 17p−, n Tese ch r cteristics c n i enti y p tients with e rly-st ge

+12 re use to pre ict surviv l. T e ver ge surviv l ti es ise se who re t high risk or e rly ise se progression.

or untre te p tients re rel te to e ch st ge s ollows: 0, Poor-risk p tients re ch r cterize by v nce clinic l

150 onths; I, 101 onths; II, 71 onths; III, 19 onths; st ge, short ly phocyte oubling ti e, un ut te i u-

n IV, 19 onths. El erly p tients, tre te n untre te , noglobulin he vy gene (IgV ) st tus, istinct geno ic ber-
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survive ro 3 to 5 ye rs on ver ge. T e princip l c use o r tions, ZAP70 n CD38 expression, n elev te seru

e th is usu lly in ection, lthough 25% o CLL p tients ie thy i ine kin se levels.

o c uses unrel te to the isor er bec use o ol er ge. T e

high risk o in ection in p tients with CLL is the result o Clinical Signs and Sym ptom s

ltere hu or l ( ntibo y) i unity c use by suppres- T e typic l p tient with CLL is sy pto tic, n the is-

sion o i unoglobulin synthesis th t le s to hypog - e se is usu lly iscovere t the ti e o routine physic l

globuline i . Autoi une ise se y lso evelop; ex in tion. T e ise se is typic lly suggeste by bnor l

utoi une he olytic ne i evelops in pproxi tely f n ings iscovere on co plete bloo count (CBC) or

one thir o p tients. the ev lu tion o n unrel te illness. Co on sy pto s

Since the intro uction o clinic l st ges, there h s been inclu e l ise, low-gr e ever, n night swe ts. Other

continuous e ort to i enti y new prognostic ctors in CLL sy pto s y be we kness, tigue, norexi , n weight

( ble 22.3). P r eters with e onstr te in epen ent loss. Physic l ex in tion usu lly reve ls cervic l n

prognostic v lue inclu e the supr cl vicul r enop thy. Hep tospleno eg ly is lso re-

quently present.
■ Nu ber o ly phocytes in the peripher l bloo

■ Degree o bone rrow inf ltr tion Laboratory Data

■ Proportion o bnor l ly phoi cells in the peripher l Nor l bone rrow ele ents get crow e out bec use o

bloo the excessive ly phoi pro uction n p cking o the r-

■ Ly phocyte oubling ti e row sp ce by lign nt ly phocytes. T is inf ltr tion by the

■ I unoglobulin he vy ch in v ri ble-region gene ut - leuke ic clone results in ne i , thro bocytopeni , n
tion st tus neutropeni .

■ Cytogenetic bnor lities ssesse by uorescent in situ Although leukocytosis y be observe , it is less pro-
hybri iz tion nounce th n in chronic yelogenous leuke i . ot l

■ Z-ch in– ssoci te protein kin se-70 protein expression
leukocyte counts c n r nge ro 30 to 200 × 10 L. In one
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thir o p tients, the tot l leukocyte count is gre ter th n

100 × 10 /L.
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Factors w ith Prognostic
Absolute ly phocytosis is usu l f n ing. T e Intern -
TABLE 22.3 Signi cance in Chronic tion l Workshop on CLL report requires th t the ly pho-

Lymphocytic Leukemia
cytosis ust be present or t le st 3 onths. In ition,

Factor Low Risk High Risk the Intern tion l Workshop llows or i gnosis o CLL
with lower ly phocyte count in p tients with cytopeni s

Clinical stage or ise se-rel te sy pto s. In the bsence o extr e ul-

9
Binet A B, C l ry tissue involve ent, there ust be ≥5 × 10 /L onoclo-
n l ly phocytes with CLL phenotype in the peripher l
Rai 0 I, II, III, IV
bloo .
Bone marrow Peripher l bloo s e rs (Figs. 22.1 n 22.2) co only

in ltration exhibit up to 80% or 90% s ll ly phocytes. M ny o these

Biopsy Nondiffuse Diffuse pattern cells h ve n over ture look bec use o the hypercon ense

pattern nucle r chro tin p ttern. An occ sion l l rge ly phobl st

Aspirate <80% lymphocytes >80% lymphocytes y be note . S u ge cells re highly ch r cteristic. Both
the gr nulocytes n the pl telets re nor l. It is i port nt
WBC (×10 /L) <50 >50
9
to i erenti te between CLL n other benign or lign nt
Prolymphocytes <10 >10 c uses o ly phocytosis (Box 22.2).

in peripheral Other il to severe i unologic l ys unction typi-

blood (%) f es the ise se. Seru electrophoresis stu ies usu lly show

Lymphocyte <12 mo >12 mo hypog globuline i Genetic stu ies c n inclu e

doubling time icro rr y n lysis (see Fig. 22.3).

β -microglobulin Normal Increased Treatm ent Options
2
CD38 <30% >30%

expression Previously, p tients with CLL were only tre te or p lli tive
re sons, but nu erous new tre t ent options re now v il-
IgV genes Mutated Unmutated ble or the tre t ent o CLL.
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