Page 479 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 23 ■ Myeloproliferative Neoplasms 463

A Summary of Signi cant

Differences Betw een
TABLE 23.6
Polycythemia Vera and Other

Types of Polycythemias

Polycythemia

Vera Other Types

Total blood volume Increased Normal or

decreased

Total leukocytes Increased Normal

Immature red blood Occasional None
cells

Platelets Increased Normal

FIGURE 23.10 A ult wo n with PV showing rke re uc-
LAP stain Increased Normal
tion in ci l rubor er phleboto y. (Reprinte ro Gol DH,

Erythrocyte Decreased Normal Weingeist A. Color Atlas o the Eye in Systemic Disease, B lti ore,

sedimentation rate MD: Lippincott Willi s & Wilkins, 2001, with per ission.)

Serum iron Decreased Normal or

increased re ucing re cell n pl telet proli er tion but t the expense

Erythropoietin Decreased or Normal or o n excess r te o the evelop ent o cute leuke i .

absent increased Chlor bucil lso pro uces n incre se r te o cute leuke-
i . Neither re current ther peutic options.
Blood histamine Increased Normal
An greli e (Agrylin, Bristol-Myers Squibb, New York) is
Unsaturated Increased Normal rel tively new ition to the ther peutic rsen l. An greli e

vitamin B –binding is prost gl n in synthet se inhibitor th t lso inhibits
12
capacity eg k ryocyte pro uction o pl telets n h s little e ect on

Basophil count Increased Normal re cell pro uction. T ere is no e ect on yelopoiesis.

Hyperuricemia Present or absent Normal Inter eron h s lso been use to control rrow overpro-

uction but c uses high risk o u-like sy pto s, y lgi ,
Hyperuricosuria Present or absent Normal
tigue, n ever.

Prognosis and Complications
( proteins C n S n ntithro bin III) h ve occ sion lly

been reporte n coul lso contribute to thro bosis. Te e i n surviv l ti e or untre te sy pto tic p tients
A co p rison o the l bor tory f n ings in PRV n other er i gnosis is 6 to 18 onths. With tre t ent, the e i n

or s o polycythe i is presente in ble 23.6. surviv l is ore th n 10 ye rs.
Cert in prognostic ctors n tre t ent str tegies h ve

Treatment n e ect on surviv l. T e clinic l course o ost p tients
is ch r cterize by low r te o cute leuke i n high
Phlebotomy r te ( pproxi tely 40%) o thro boe bolic co plic -

Pri ry control o PRV is chieve by ther peutic phlebot- tions. Myelof brosis evelops in so e p tients. A high ini-

o y (Fig. 23.10). T e i o phleboto y is to pro uce n ti l he oglobin concentr tion in peripher l bloo n the

iron ef ciency th t then li its re bloo cell pro uction. use o ny yelosuppressive ther py re ssoci te with n

T is y be per or e by the re ov l o units o whole incre se risk o leuke ic tr ns or tion.

bloo or by l rge-volu e erythrocyt pheresis using cell

sep r tor. Cyt pheresis pro uces long-l sting re uction o

re bloo cell volu e ( icrohe tocrit), he oglobin, n NOTE: This is a good time to complete Review Questions

erythrocyte counts s well s the i e i te is ppe r nce related to preceding content.

or re uction o clinic l sy pto s.

T e evolution o PRV is vor bly ltere by ther peutic

phleboto y n che other peutic cytoreduction, which re PRIMARY MYELOFIBROSIS

o en per or e si ult neously.

T e ter yelof brosis enco p sses primary myelo brosis
Chem otherapy (PMF), postessenti l thro bocythe i (E ), n postpoly-

T e inst y o cytore uction o the pl telet count is rug cythe i (PPF-MF). PMF is ch r cterize by syste ic

ther py. Phosphorous 32 (P32) is e ective s tre t ent in bone rrow f brosis n extr e ull ry he topoiesis.