Page 439 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 22 ■ Lymphoid and Plasma Cell Neoplasms 423

FIGURE 22.4 H iry cell leuke i . A. Peripher l

bloo . B. Bone rrow (H&E st in). C. Spleen
(H&E st in). (Reprinte ro H n in RI, et l.

Blood Principles and Practice o Hematology, 2n e ,

Phil elphi , PA: Lippincott Willi s & Wilkins,
2003, with per ission.)

Treatm ent and Prognosis T-CELL AND NK-CELL NEOPLASMS

T e clinic l course o HCL is ore benign th n ny or s

o leuke i . P tients requently live ore th n ec e er M ture -cell neopl s s re erive ro ture or post-

i gnosis. T e gre test risk o e th is ro in ection. thy ic cells. Bec use NK cells re closely rel te n

In ost c ses, HCL is controll ble with tr ition l che o- sh re so e i unophenotypic l n unction l properties

ther py, cl ribine, chlorin te purine nucleosi e n log. with cells, these two cl sses o neopl s s re consi ere

In s ll clinic l tri l, 70% o HCL p tients whose ise ses together ( bles 22.4 n 22.5).

were resist nt to convention l che other py experience

re ission when tre te with nti-CD22 reco bin nt i u- T-Cell Prolymphocytic Leukemia

notoxin (BL22). Recently, it w s e onstr te th t short

or l course o ve ur enib, n inhibitor o b-R serine- -cell proly phocytic leuke i s ( -PLLs) re r re with

threonine kin se with V600E ut tion (BRAF V600E), w s poor prognosis. Distinction ro CLL i gnosis requires

highly e ective in p tients with rel pse or re r ctory HCL. ore th n 55% o circul ting ly phoi cells to h ve the
orphology o proly phocyte.

Hairy Cell Leukem ia Variant
Epidem iology
A v ri nt or o HCL, h iry cell leuke i v ri nt (vHCL),

w s iscovere in 1980. In ition, uch r rer v ri nts, -cell PLL is r re n ccounts or less th n 2% o ly phoi

J p nese v ri nt n bl stic v ri nt, exist. vHCL is ore leuke i s. T is or o leuke i occurs in ol er ults with

ggressive type o HCL n h s i erent orphologic l e i n ge in the 60s ( ge 61 or cell). M les h ve

ch r cteristics th n typic l HCL. higher inci ence o these leuke i s.

vHCL cells re s ller th n the typic l HCL cell with

centr l roun nucleus, pro inent nucleoli, l rger nucle r- Clinical Signs and Sym ptom s

to-cytopl s ic r tio, n b sophilic cytopl s with occ - -cell PLL p tients exhibit sy pto tic spleno eg ly n

sion l cytopl s ic projections. vHCL y present with ly phocytosis. -PLL p tients ore requently experience

orphologic e tures inter e i te between h iry cells n ly ph enop thy, hep to eg ly, n skin lesions th n B-PLL.

proly phocytes.

Di erenti l i gnosis o vHCL ro typic l HCL c n be Laboratory Findings

e by cytoche ic l st ining ( RAP) n i unopheno- -cell n B-PLLs c n be istinguishe ro e ch other n

typing using ow cyto etry. vHCL h s poor prognosis. ro other n B cells leuke i s by ev lu tion o bloo