Page 447 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 431

T e higher-gr e ly pho s usu lly pro uce the s e Ig Clinical Signs and Sym ptom s

he vy ch ins n light ch ins s the origin l tu ors. Most co only DLBCL is loc lize to ly ph no es.

P n-B-cell rkers re CD19+ n CD20+ but re usu lly

CD5- n CD10-. Laboratory Characteristics

I unophenotype rkers re CD5+, CD10+, Bcl6, CD30+,
Diffuse Large B-Cell Lym phom a, Not Otherw ise n CD138+.

Speci ed (DLBCL, NOS) Bone rrow involve ent is r re t present tion but c n

DLBCL is heterogeneous group o tu ors co pose o l rge occur l ter in the course o the ise se. Neopl stic cells re

B-ly phoi cells. So e result ro the tr ns or tion ro signif c ntly l rger th n nor l ly phs.

lower-gr e ly pho , such s FL, SLL, or it rises e novo.

Burkitt’s Lymphoma
Classi cation

T ree i erent types o i use LBCLs c n be ef ne b se WHO cl ssif c tion co bines the cute ly phobl stic leu-

on gene expression subgroups: ke i (ALL-3 o the FAB cl ssif c tion) with Burkitt’s ly -
pho . Most but not ll ALL-L3 c ses ppe r to be the
1. Ger in l center, B-cell–like ly pho th t expresses leuke ic ph se o Burkitt’s ly pho . High-gr e ly -

high levels o genes ch r cteristic o ger in l center, pho less co only h s leuke ic ph se.

B-cell–like ly l ger in l center B cells

2. Activ te B-cell–like ly pho , which expresses genes Classi cation and Epidem iology

ch r cteristic o itogenic lly ctiv te bloo B cells

3. New subgroup, type 3 i use LBCL, which h s heteroge- T ree types o Burkitt’s ly pho (BL), high-gr e NHL

neous gene expression th t suggests it inclu es ore th n ly pho , re recognize : A ric n (en e ic), spor ic,

one subtype o ly pho n i uno ef ciency ssoci te . Associ tion with EBV is
100% in A ric n (en e ic) n 20% to 40% in other c tego-

Epidem iology ries. T e DNA o EBV is oun in ost c ses o en e ic n

DLBCL is the ost requent NHL ly pho in North one thir o HIV- ssoci te tu ors.
A eric n Europe n occur in ll ge groups (Fig. 22.12). Burkitt’s ly pho represents pproxi tely one thir o ll

DLBCL ccounts or pproxi tely 40% o new c ses o pe i tric ly pho s occurring outsi e A ric . M ny ult c ses
ly pho . More th n h l o p tients with i use LBCL re ll into the i unoco pro ise c tegory, such s HIV virus.

ol er th n 60 ye rs o ge.
EBV+ LBCL o the el erly is now c lle EBV+ DLBCL Pathogenesis

- NOS in the 2016 WHO revision. It occurs in i uno- Mut tions in tr nscription ctor CF-3 or its neg -
co petent p tients usu lly 50 ye rs ol or ol er. T ey h ve tive regul tor ID3 occur in bout 70% o spor ic n

worse prognosis th n EBV neg tive tu ors. T is subtype i uno ef ciency-rel te BL n 40% o en e ic c ses.
o ly pho is being incre singly recognize in younger CF 3 pro otes surviv l n proli er tion in ly phoi

p tients. cells by ctiv ting the BCR/phosph ti ylinositol 3-kin se

FIGURE 22.12 Ly pho s. A. Non-

Ho gkin ly pho . B. Re ctive enitis in

chil . C. Mixe l rge n s ll ly phocytes

in p tient with Ho gkin ise se. D. L rge
B-cell ly pho . (Reprinte ro Greer JP,

et l. Wintrobe’s Clinical Hematology, 11th

e , Phil elphi , PA: Lippincott Willi s &
Wilkins, 2004, with per ission.)