Page 457 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 441

Clinical Signs and Symptoms Ch r cteristic lly, bloo s ples re escribe s h v-

ing hyperviscosity. Detecting onoclon l g op thies
T e sy pto s o WM re ue to the extent o tu or inf l- usu lly involves seru protein electrophoresis (SPEP) n

tr tion n to elev te IgM levels in the bloo circul tion. i unoelectrophoresis (IEP) to test both seru n urine.

Sy pto s inclu e we kness, tigue ttribut ble to ne i , A ition lly, cryoglobulins c n be etecte in the p tient’s

n blee ing. Bone p in is virtu lly nonexistent. About one seru . Cryoglobulins re proteins th t precipit te or gel

ourth o p tients with WM h ve neurologic l bnor lities. when coole to 0°C n issolve when he te . In ost c ses,

T e inci ence o in ection is twice the nor l r te. P tients onoclon l cryoglobulins re IgM or IgG.

usu lly su er ro chronic ne i n blee ing episo es.

T ro bocytopeni n hyperviscosity y lso contribute Treatment and Prognosis

to the blee ing isor er.

T er py is postpone or sy pto tic p tients, n pro-

Laboratory Data gressive ne i is the ost co on in ic tion or initi tion

o tre t ent. M in ther peutic options inclu e lkyl ting
T e ost consistent e ture o the bone rrow or ly ph gents, nucleosi e n logues, n rituxi b. Novel gents,

no es o WM p tients is the presence o pleo orphic or ex ple, vortezo ib, show pro ise s t rgete ther py

B-line ge cells t i erent st ges o tur tion, such s s ll option in WM.

ly phocytes, ly phopl s cytoi cells ( bun nt b so- Prognostic ctors inclu e the p tient’s ge, β -
2
philic cytopl s but ly phocyte-like nuclei), n pl s icroglobulin level, onoclon l protein level, he oglobin

cells. Bone rrow st cells o WM p tients overexpress concentr tion, n pl telet count. T e reporte e i n sur-

the CD40 lig n (CD154), which is potent in ucer o B-cell viv l o p tients with WM r nges between 5 n 10 ye rs

exp nsion. T e ly phocyte–pl s cells v ry orphologi- ro the ti e o i gnosis.

c lly, r nging ro s ll ly phocytes to obvious pl s

cells (Fig. 22.21). T eir cytopl s is requently r gge n

y cont in perio ic ci -Schi (PAS)-positive teri l NOTE: This is a good time to complete end of the chapter

th t is prob bly i entic l to the circul ting croglobulin. Review Questions.

T e tot l leukocyte count is usu lly nor l, with n bso-

lute ly phocytosis. Mo er te to severe egrees o ne i

re requently observe on peripher l bloo s e rs s well CHAPTER HIGHLIGHTS

s roule ux or tion. T e p tient’s pl s volu e y be

gre tly incre se , n the ESR is incre se . Pl telet counts Leukemias and Lymphomas

re usu lly nor l. Blee ing c use by bnor lities in

pl telet hesiveness n prothro bin ti e y be seen, ■ T e ter ly phoproli er tive isor er inclu es the v ri-

n the v lues o ctor VIII y be low. ous or s o leuke i s n ly pho s th t re o ly -
phoreticul r origin.

■ Ly phoi neopl s cl ssif c tions re b se on the is-

tribution o ise se s leuke i or ly pho . During the

progression o ise se in so e ly pho s, the lign nt

cells y spill into the bloo circul tion. T is spillover

y pro uce leuke ic ph se o the ise se.

■ r nsitions to leuke ic ph se re r re in isor ers such

s Ho gkin ise se but re not unco on in the well-

i erenti te non-Ho gkin ly phocytic ly pho s.

Chronic Lymphocytosis

■ Te WHO Cl ssif c tion o u ours o the H e topoietic

n Ly phoi issues h s enh nce the cl ssif c tion o

ly phoi neopl s s by inclu ing i unophenotypic e -

tures n genetic bnor lities to ef ne i erent isor ers.

■ Ex ples o isor ers o the ture B-cell neopl s

cl ssif c tion inclu e CLL/SLL, B-PLL, n h iry cell

leuke i .

FIGURE 22.21 W l enströ . Bone rrow spir te showing Chronic Lymphocyte Leukemia/ Small
lign nt cells with ly phoi n pl s cytoi orphology. Lymphocytic Lymphoma

(Reprinte ro Greer JP, et l. Wintrobe’s Clinical Hematology,

11th e , Phil elphi , PA: Lippincott Willi s & Wilkins, 2004, ■ Chronic leuke i s re gener lly ch r cterize by the

with per ission.) presence o leukocytosis with n incre se nu ber o