Page 467 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 23 ■ Myeloproliferative Neoplasms 451

TABLE 23.1 Comparative Peripheral Blood Characteristics of MPNs

CML PM PRV ET

Erythrocytes × 10 /L Decreased Decreased Extremely increased Normal
12

Leukocytes × 10 /L Extremely increased Variable Increased Normal
9
9
Platelets × 10 /L Moderately increased Variable Moderately increased Extremely increased

Teardrop-shaped None Extremely increased None None

erythrocytes

Leukocyte alkaline Decreased Variable Extremely increased Normal/increased

phosphate score

Marrow brosis Variable Very increased None None

Ph chromosome Positive Negative Negative Negative
1

CML, chronic myelogenous leukemia; PM, primary myelo brosis; PRV polycythemia vera; ET, essential thrombocythemia.
,

ctor V. In ny p tients, the levels o d- i er, thro bin- Molecul r ther py or CML h s revolutionize tre t ent

ntithro bin co plex, n pl s in- lph 2-pl s in inhibi- t the olecul r level. Ongoing clinic l tri ls re irecte t

tor co plex re higher th n nor l. olecul r ther py or other MPNs. Cytogenetic n olecu-

P tients with n MPN co only exhibit thro botic phe- l r ch nges er inter eron ther py re pp rent in p tients

no en . T is is believe to be ssoci te with n incre se in with CML, s ni este by ch nge in the Phil elphi

circul ting pl telets ecting the rteri l n venous circul - (Ph ) chro oso e n BCR-ABL gene, respectively.
1
tion. In r re c ses, the initi l ni est tion o MPN c n be D t to te suggest th t inter eron lph y be new

g ngrene o the extre ities. Nu eric l n orphologic l n e ective rug or the tre t ent o MPNs. T e ech -

ch r cteristics o the eg k ryopoiesis i er in e ch c te- nis o ction o inter eron is not co pletely un erstoo .

gory o MPN. A tri o qu lit tive pl telet e ects ( bolishe T is biologic l gent, either lone or in co bin tion with

secon -w ve epinephrine ggreg tion, incre se enosine other ntineopl stic tre t ent, represents the cl ssic ther -

iphosph te [ADP] ggreg tion threshol , n signif c ntly peutic ppro ch or the tre t ent o these isor ers.

re uce enosine triphosph te [A P] secretion uring col-

l gen-in uce ggreg tion) see s to be goo i gnostic

rker o MPN with thro bocytosis. NOTE: This is a good time to review the de nitions of Key

In ition, r chi on te et bolis is requently Terms in the Glossary and ash cards on thePoint. It is a

er nge in p tients with MPN. T e ch nge in thro box- good time to complete Review Questions related to preced-

ne or tion in essenti l thro bocythe i n PRV coul ing content.

be one o the ctors responsible or the i erent inci ences

o thro botic n he orrh gic co plic tions in these is-

e ses. When r chi onic ci et bolis in p tients with n CHRONIC MYELOGENOUS LEUKEMIA

MPN w s ev lu te , the gener tion o thro box ne B2 w s Chronic myelogenous leukemia (CML) is n MPN th t origi-

oun to be signif c ntly re uce n inversely correl te n tes in n bnor l pluripotent bone rrow ste cell n

with the pl telet count in p tients with essenti l thro bo- is consistently ssoci te with the BCR-ABL1 usion gene

cythe i . PRV p tients showe n incre se or tion o loc te in the Phil elphi chro oso e.

this et bolite o r chi onic ci . T e gener tion o pros-

t gl n in E2 n 6-keto-prost gl n in F w s signif c ntly Epidemiology
1α
re uce in p tients with CML.

Ninety-f ve percent o p tients with CML h ve the co on

Prognosis and Treatment type. Other or s, or ex ple, chronic neutrophilic leuke-
i (CNL), re extre ely r re.

Acute leuke ic tr ns or tion in PMF gener lly h s n T is CML is pre o in ntly leuke i o i le- ge

insi ious present tion, contr sting with its brupt onset ults. T e e i n ge t i gnosis is in the f h n sixth

in ost p tients with PRV or essenti l thro bocythe i . ec es o li e. M les h ve slightly gre ter r te o ise se

Approxi tely two thir s o p tients whose illness tr ns- occurrence. T e inci ence o CML in Western countries is

or s into cute leuke i evelop yelogenous leuke i ; esti te to be pproxi tely 2 per 100,000 persons nnu-

the re ining one thir evelop ly phobl stic leuke i . lly n ccounts or 15% o leuke i s in ults ( ll ge

Me i n surviv l ti e ro i gnosis o the cute tr ns or- groups inclu e ). Five to ten percent o p tients h ve his-

tion ver ges only 3 onths. tory o excessive r i tion exposure.