Page 446 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

430 PART 6 ■ Neoplastic Disorders

low r te o progress ch r cterize by the presence o cyclin T ree types o rgin l zone ly pho h ve been

D1+ cells ost typic lly in the inner ntle zones o ollicles i entif e :

in ly phoi tissues. P tient i gnosis is o en oun inci en- 1. Nodal marginal zone lymphoma, monocytoid B-cell lym-

t lly n h s low r te o progression.
phoma, occurs within the ly ph no es n ccounts or
bout two percent o ll B-cell ly pho s.
Pathophysiology
2. Splenic marginal zone lymphoma occurs ost o en in
MCL cl ssic lly h s been recognize s n ggressive but the spleen n bloo . It h s been ssoci te with Hep titis

incur ble s ll B-cell ly pho th t evelope in line r C. T is or o ly pho kes up bout 1% o ll B-cell

shion ro n ive B cells. wo types o clinic lly in olent ly pho s.

v ri nts th t evelop long two very i erent p thw ys h ve 3. Extranodal marginal zone lymphoma or mucosa-

been recognize . associated lymphoid tissue (MALT). T is is the ost

Cl ssic MCL is usu lly co pose o IGHV-un ut te co on or o rgin l zone ly pho th t occurs

or ini lly ut te B cells th t usu lly express SOX1 n outsi e the ly ph no es, such s the sto ch, s ll intes-

typic lly involves ly ph no es n other extr no l sites. tine, s liv ry gl n , thyroi , eyes, n lungs. MAL ly -

Acquisition o ition l olecul r/cytogenetic bnor li- pho is ivi e into g stric n nong stric. T is or

ties c n le to even ore ggressive bl stoi or pleo or- o ly pho kes up bout 9% o ll B-cell ly pho s.

phic MCL. A secon or o MCL evelops ro IGHV

ut te SOX11-B cells th t le s to leuke ic nonno l MCL Risk Factors

th t usu lly involves the peripher l bloo , bone rrow, n

spleen. T ese c ses re requently clinic lly in olent. A sec- ■ Most MAL ly pho s rise in ucos l sites evoi o

on ry bnor lity, o en involving P53, y occur n org nize ly phoi structures.

le to very ggressive ise se. ■ Risk ctors h ve been i entif e or evelop ent o
MAL . T ese ctors re:

Clinical Signs and Sym ptom s H. pylori (sto ch)
C. jejuni (intestine)
P tients requently present with sy pto s o isse in te C. psittaci (orbit)

ise se involving ultiple ly ph no e groups, bone r- Hep titis C, utoi unity-Sjogren syn ro e (s liv ry

row, peripher l bloo , spleen, liver, n g strointestin l gl n )

tr ct.
Autoi unity-H shi otos’s thyroi itis (thyroi )

B. burgdor eri (skin)
Laboratory Characteristics

Me iu -size ly phoi cells with irregul r nucle r outlines Laboratory Characteristics

erive ro the ollicul r ntle zone re observe . Bone

rrow, peripher l bloo , spleen, n g strointestin l tr ct I unophenotyping o B cells is CD19+, CD20+, n y

re requently involve . be CD43+ but usu lly not the other ntigens expresse by

MCL is ch r cterize by the i unophenotype s ll B-cell ly pho s. Sur ce Ig usu lly IgM is present.

CD19+, CD20+, CD5+, CD23-, FMC-7+, n sIg (strongly Chro oso l tr nsloc tions re t(11;18), t(14;18), n

positive). t(1;14).

Al ost ll c ses re positive or cyclin D1 by i unohis-

toche istry. Cyclin D1 is involve in the regul tion process

o cells ro the G to S ph se o the cell cycle. Overexpression NOTE: This is a good time to complete Review Questions
1
o cyclin D1 in MCL is usu lly the result o t(11;14), which related to the preceding content.

involves the Bcl-1 gene. T e Bcl-1 tr nsloc tion is thought

to le to neopl stic tr ns or tion through the loss o cell

cycle control. Lym phoplasm acytic Lym phom a

T is is n unco on B-cell neopl s co pose o s ll
Prognosis
ly phocytes, pl s cytoi ly phocytes, pl s cells, n
Me i n surviv l is 3 to 4 ye rs. v ri ble nu ber o l rge ly phocytes. T ere c n be bone r-

row involve ent n leuke ic ph se. Ly phopl s cytic

Marginal Zone B-Cell Lym phom a ly pho s (LPLs) re o en ssoci te with high levels o

M rgin l zone ly pho s re group o in olent (slow- IgM p r protein such s Waldenström macroglobulinemia or

growing) NHL B-cell ly pho s. T e occurrence o r- type II cryoglobuline i .

gin l zone ly pho s in ults is pproxi tely 6% to

8% o ll NHLs in the Western he isphere. T ey ccount Laboratory Characteristics

or pproxi tely 12% o ll B-cell ly pho s. T e e i n LPLs y c use lter tions o the ly ph no e rchitecture.

ge or i gnosis is 65 ye rs. T ese ly pho s y tr ns or into l rge cell ly pho s.