Page 445 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 429

ly pho , n n pl stic l rge cell ly pho , n one sub- ISFN. I unophenotyping use to conf r presence o

type cl ssif e s precursor NHL: ly phobl stic ly pho . ly pho onoclon l ly pho n conf r the subtype

Aggressive, high-gr e ise se kes up the jority o o ly pho CD10+. Most FL c ses rise bec use o t(14;18)

NHL c ses in chil ren co p re with pre o in nce o involving the BCL-2 gene th t le s to overexpression o

low- n inter e i te-gr e ly pho s in ults. T ere is BCL-2 protein in ly phocytes. BCL-2 protein inhibits in i-

signif c nt overl p between NHL in ults n pe i trics, vi u l cell e th, poptosis, llowing ollicle center cells to

but there re unique ch r cteristics in chil ren. LBCL with ccu ul te n pro uce ly ph enop thy.

IRF4 re rr nge ent th t occurs ost co only in chil ren

n young ults is new provision entity in 2016 WHO Prognosis

revision. T is ly pho is consi ere ore ggressive th n Me i n surviv l is 7 to 9 ye rs, i in olent. Ch r cteristic lly,

other pe i tric types o ly pho . low r te o progression is observe , but it is ore o en

ssoci te with prior or synchronous overt ly pho s th t
Leukemic Phase of Non-Hodgkin require ition l clinic l ssess ent.

Lymphomas

Follicular Lym phom a Variants of FL

In situ ollicul r ly pho (FL) h s been ren e in situ Pe i tric FL is ef nite entity in 2016 WHO cl ssif c tion

ollicul r neopl si (ISFN) with the s e i gnostic criteri but now c lle pe i tric-type FL bec use si il r ly pho-

in the revise 2016 WHO cl ssif c tion. FC is one o the s y occur in ults. It is no l ise se with l rge high

ost co on types o ture B-cell ly phoi neopl s s proli er tive ollicles requently with pro inent bl stoi ol-

in the Unite St tes. licul r center cells r ther th n cl ssic centrobl sts (or centro-
cytes). Bcl2 re rr nge ents ust not be present, but there

Clinical Signs and Sym ptom s y be so e PCL2 protein expression. Pe i tric FL lso

Gener lize ly ph enop thy l cks Bcl6 n MYC re rr nge ents. Ne rly ll c ses re
loc lize n y only require excision.

Laboratory Characteristics GI tr ct FC is nother v ri nt. Duo en l-type FL h s

Most v nce -st ge ise ses (Fig. 22.11) h ve bone r- e tures o loc lize overt low-gr e FL but is istinct ro

row involve ent, n ew c ses lso h ve peripher l bloo other GI tr ct FL n h s ny e tures th t overl p with

involve . In the bone rrow, repl ce ent o he topoietic ISFN s well s so e e tures rese bling n extr no l

precursors by bnor l s ll ly phocytes with bun nt rgin l zone ly pho . T ese p tients h ve n excellent

cle r cytopl s “ rie egg” ppe r nce is observ ble. Cells outco e.

in the peripher l bloo n bone rrow usu lly h ve very

irregul r nucle r outline n eep in ent tion (cle ) o the Mantle Cell Lym phom a

nucle r e br ne (butt cells). In situ MCL is now c lle in situ ntle cell neopl si

Flow cell stu ies e onstr te popul tions o B cells (ISMCN). Distinction o MCL ro other s ll ly phoi

with FL phenotype in bout h l o ll ly ph no es with B-cell neopl s s is i port nt. T is ly pho subtype h s

FIGURE 22.11 Non-Ho gkin ly pho s

o B-cell origin— ollicul r ly pho l bo-

r tory f n ings: cell types, b se on origin o
the lign ncy in the ollicle, s ll cle ve

ollicle center cell (centrocytes), sc nt cyto-

pl s ; l rge ollicle center cell (centrobl sts),

b sophilic cytopl s ; n ollicul r p ttern.
I unophenotype: bcl-2, CD10, CD20,

n sIg positive; CD23 +/−; n CD5 neg -

tive. Fro An erson SC. Anderson’s Atlas o

Hematology, Phil elphi , PA: Wolters Kluwer
He lth/Lippincott Willi s & Wilkins, 2003.