Page 500 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

484 PART 6 ■ Neoplastic Disorders

NOTE: This is a good time to complete end of chapter
BOX 24.3
Review Questions.

Cytochemical Staining in CMML
CHAPTER HIGHLIGHTS

Alph -n phthyl cet te ester se

Alph -n phthyl butyr te ester se Classi cation

N phthol-ASD-chloro cet te ester se
■ Since the origin l evelop ent o the FAB cl ssif c tion

or yelo yspl stic syn ro es, the WHO h s evelope

newer cl ssif c tion o MDSs n yelo yspl stic syn-
Other Classi cations ro e/ yeloproli er tive neopl s s.

■ MDS is ch r cterize by the si ult neous proli er tion
Atypic l chronic yeloi leuke i ( CML), juvenile yelo- n poptosis o he topoietic cells th t le to nor-

onocytic leuke i (JMML), n MDS/MPNS, uncl ssif ble l or hypercellul r bone rrow n peripher l bloo

(MDS/MPNS-U) re the other, less requent cl ssif c tions in cytopeni s.

the yelo yspl stic syn ro e/ yeloproli er tive c tegory.

1. Atypic l chronic yeloi leuke i ( CML, BCR-ABL1 Pathophysiology

neg tive). T is c tegory exhibits e tures o both yelo- ■ T e MDSs n MDS/MPN re heterogeneous group o

yspl stic n yeloproli er tive isor ers t the ti e o clon l isor ers o the bone rrow.

i gnosis. It is ch r cterize by leukocytosis with jor- ■ An lyses suggest th t the p thogenesis o MDS is ulti-

ity o neutrophils. Multiline ge yspl si is co on. I n step process beginning with the est biliz tion o the ulti-

ssess ent or CSF3R ut tion is positive, chronic neu- potenti l ste cell, c using proli er tion o ivergent clone

trophilic leuke i shoul be consi ere . o genetic lly unst ble pluripotenti l ste cells th t pro uce

2. Juvenile yelo onocytic leuke i . JMML is isor er o orphologic lly v ri ble but clon lly rel te progeny.

chil hoo . It is ch r cterize by the proli er tion o gr n- ■ He topoiesis is yspl stic bec use o ine cient tur -

ulocytic n onocytic line ges. Bl sts n pro ono- tion o slowly exp n ing or so eti es o st ble popu-

cytes ccount or less th n 20% o peripher l bloo cells l tion o bloo cell precursors.

n bone rrow spir tes. Erythroi n eg k ryo-

cytic bnor lities re requently preset. T e BCR-ABL1 Etiology

ut tion is bsent, but ut tions o genes NRAS, KRAS, T e etiology o pri ry MDS is unknown.

P PN-11, CBL, n NF1/MAPK re ch r cteristic. ■

3. Myelo yspl stic syn ro e/ yeloproli er tive neopl s , ■ Secon ry MDSs c n so eti es be irectly rel te to
known gent.
uncl ssif ble. T is neopl s eets the ef nition o T e gre test inci ence o MDS ollows co bine che o-

MDS/MPNS but oes not eet the criteri or CMML or ■ ther py n r i tion ther py.

the other cl ssif c tion in this c tegory.

4. A provision l new c tegory RARS- (re r ctory ne i ■ So e pre isposing ctors or MDS y be genetic.

with ringe si erobl sts n thro bocytosis) h s been Epidemiology

e in 2016. Cytogenetic f n ings inclu e neg tivity or

BCR-ABL n the presence o SF3B1 n JAK2 ut tions. ■ MDS is r re in chil hoo . It occurs inly in ol er in i-
vi u ls n is ore co on in les. T e prev lence o

MDS y be s high s 1:500 in in ivi u ls ol er th n 55

ye rs o ge.

■ It is esti te th t t le st 1,500 to 2,000 c ses o MDS re
i gnose nnu lly in the Unite St tes.

Chromosomal Abnormalities

■ Chro oso l bnor lities h ve been observe in sig-

nif c nt proportion o p tients with MDS. Cytogenetic

i erences exist between pri ry n secon ry MDSs.

■ Chro oso e bnor lities y be onoso ic or triso-

ic in n ture n involve p rti l or tot l chro oso l

lter tions. Most chro oso es ispl y recurrent loss o

FIGURE 24.4 Chronic yelo onocytic leuke i (CCML). chro oso l teri l r ther th n the tr nsloc tions or

(Reprinte ro An erson SC. Anderson’s Atlas o Hematology, inversions.
Phil elphi , PA: Wolters Kluwer He lth/Lippincott Willi s & ■ Surviv l o p tients with MDS is better or those with nor-

Wilkins, Copyright 2003, with per ission.) l chro oso l p tterns.