Page 441 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 425

FIGURE 22.8 Séz ry cells. wo circul ting neopl stic -helper

cells with irregul r nuclei n thin ri o cytopl s re seen.
(Reprinte ro Rubin E, F rber JL. Pathology, 3r e , Phil elphi ,
FIGURE 22.7 Mycosis ungoi es with oc l interstiti l inf ltr tion PA: Lippincott Willi s & Wilkins, 1999, with per ission.)

by s ll ly phocytes. (Reprinte ro C gle P . Color Atlas and

ext o Pulmonary Pathology, Phil elphi , PA: Lippincott Willi s

& Wilkins, 2005, with per ission.) T-Cell Large Granular Lymphocytic
Leukemia (LGL)

-cell LGL h s present tion th t is si il r to CLL but is
Epidem iology
co pose o ture cells. It is i port nt to istinguish

Te nnu l inci ence o C CL in the Unite St tes is esti- ro re ctive ly phocytosis by the presence o n bnor l

te t 7.7 c ses/ illion persons ro 2001 to 2005. phenotype or evi ence o clon lity. In ition, LGL ust

A ults between 40 n 60 ye rs ol re ost requently be istinguishe ro in olent chronic ly phoproli er tion

icte with skin lesions th t progress to the tu or st ge. isor ers o NK cells n ggressive NK-cell leuke i .

Clinical Signs and Sym ptom s Laboratory Features

T e jority o MF p tients present with e rly-st ge ise se In LGL, p tients exhibit ne i , neutropeni , n thro -

or li ite p tches. E ch st ge o MF is ch r cterize by the bocytopeni . A con ition o ne i c n be rel te to bone

egree o skin involve ent by p tches ( -st ge). -st ges rrow inf ltr tion or con ition o pl si o re bloo cell

r nge ro 1 with involve ent o less th n 10% o bo y sur- (RBC) precursors. Con itions o neutropeni n thro -

ce; 2 is gre ter th n 10% o bo y involve ent by p tches bocytopeni c n be ssoci te with i une estruction,

or pl ques. A v nce -st ge MF inclu es p tients with tu ors splenic sequestr tion, or bone rrow inf ltr tion by lig-

( 3) n erythro er ( 4) with or without Séz ry syn ro e n nt cells.

s well s p tients with bloo , no l, bone rrow, or viscer l T e peripher l bloo (Fig. 22.9) e onstr tes o est

ise se. ly phocytosis with ture, clu pe nucle r chro tin.
Neopl stic cells o -LGL re ly phs CD2+, 3+, CD4-,

Laboratory Findings CD5+, CD7+, CD8+, CD16+, CD56 +/-, CD57 +/-, n usu-

Séz ry syn ro e is ef ne s erythro er gre ter th n lly CD56- n CD57+.

80% n leuke ic bloo involve ent gre ter th n 1,000

o circul ting typic l ly phocytes. In peripher l bloo , Adult T-Cell Leukemia/ Lymphoma

the ise se is ch r cterize by the presence o bnor- A ult -cell leuke i /ly pho (A LL) is peripher l

l circul ting ly phocytes, Séz ry cells. A Séz ry cell -cell neopl s c use by hu n -cell ly photropic

(Figs. 22.7 n 22.8) is typic lly the size o s ll ly pho- virus-1 (H LV-1). T ere re our subtypes o the ise se:

cyte n h s rk-st ining, clu pe , nucle r chro tin cute, chronic, ly pho tous, n s ol ering.

p ttern. T e istinctive ol e , groove-like chro tin p t-

tern is escribe s cerebri or . M ture ly phocytes in Epidem iology

Séz ry ispl y phenotype with re ctivity or CD2, CD3, A LL is ost prev lent in southwestern J p n, centr l

CD4, n CD5. A ric , n the C ribbe n b sin.

Prognosis Etiology

C CLs, o which ycosis ungoi es n Séz ry syn ro e A LL is c use by retrovirus spre by c rriers o the virus.

re the ost co only encountere or s, re currently not Tere is less th n 5% ch nce o contr cting A LL. Te vir lly

cur ble in ost p tients. Skin- irecte ther py is extre ely enco e protein, x, represses cell cycle- ctive proteins th t

e ective or p tients with e rly, skin-li ite ise se. results in persistent clon l proli er tion o in ecte cells.