Page 481 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 23 ■ Myeloproliferative Neoplasms 465

resulting ro thro bocytopeni , bnor l pl telet unc-

tion, or both. About one thir o p tients ni est purpur .

More th n 40% o p tients h ve osteosclerosis with cco -

p nying bone p in, l ise, n leukocytosis. A il egree

o j un ice, b o in l ullness, yspepsi , or weight loss

y be ni este in so e p tients. Port l hypertension

y be evi ent.

In r re c se, one p tient presente with bre st ss.

Excision biopsy o the ss reve le extr e ull ry he -

topoiesis, s i histop thologic l ex in tion o the liver

n the spleen. T is type o present tion e onstr tes the

co ple ent ry ch r cter o both i gnostic o lities n

the rese bl nce to ly pho o the bre st, lthough the FIGURE 23.11 ypic l peripher l bloo s e r o chronic pri-
ry yelof brosis with leukoerythrobl stosis n te r rop re
fn ings re too nonspecif c to rule out bre st c rcino . bloo cells. (Reprinte ro McCl tchey KD. Clinical Laboratory

Knowle ge o the clinic l history n histop thology is nec- Medicine, 2n e , Phil elphi , PA: Lippincott Willi s & Wilkins,

ess ry to ke the proper i gnosis. Another r re present - 2002, with per ission.)

tion occurre in p tient with cut neous extr e ull ry

he topoiesis. T e skin lesions ppe re s ultiple p p- Erythrocytes

ules n no ules on the trunk. Histologic l ex in tion Mil ne i c use by ine ective erythropoiesis (Fig. 23.11),

o lesion showe ll three co ponents o the he topoi- ecre se re bloo cell surviv l, n overt he olysis y

etic tissue, th t is, yeloi , erythroi , n eg k ryocytic occur. Polychro tophili n n elev te reticulocyte count

series.
in the bsence o erythropoietic stress provi e n i port nt

clue to i gnosis bec use they signi y bre k own in r-
Cellular Alterations row ultr structure.

He tologic l f n ings (Box 23.3) re v ri ble n non-

uni or , but bloo orphology provi es the best clues Leukocytes

to i gnosis. T e leukoerythrobl stic picture o te r rop- In pproxi tely 50% o p tients, the tot l leukocyte (WBC)

sh pe erythrocytes, nucle te erythrocytes, n i ture count is incre se . Most p tients h ve tot l WBC counts less
9
yeloi cells is cl ssic or yelof brosis. Leukocytosis, il th n 30 × 10 /L, but the tot l WBC count c n be s high s
9
ne i , thro bocytosis, n p nhyperpl si in the rrow 100 × 10 /L. A high WBC count (neutrophili ) n i ture

re ch r cteristic in the e rly st ges. Extr e ull ry he - gr nulocytes on peripher l bloo s e rs inclu ing bl sts

topoiesis, peripher l cytopeni s (i.e., ne i , leukopeni , c n cre te picture th t c n be con use with leuke i .

or thro bocytopeni ), n yelof brosis, with or without

osteosclerosis, re ect the ch nges seen in the l ter st ges. Platelets

r nsitions ong the i erent types o MPNs n ter in - T e concentr tion o pl telets is v ri ble, but gi nt yspl s-

tion in cute leuke i or rrow ilure re co on. tic pl telets n r g ents o eg k ryocytes c n be seen.
T ro bocytosis gr u lly progresses to thro bocytopeni .

As yelof brosis progresses, the entire orphologic l picture

BOX 23-3 o yelophthisis (inf ltr tive yelop thy) un ol s: te r rop-
sh pe erythrocytes, nucle te erythrocytes, e rly gr nulo-

cytic or s, biz rre pl telets, n eg k ryocyte r g ents.

Diagnosis of Primary Myelo brosis *

Bone Marrow

MAJOR CRITERIA T e bone rrow is hypocellul r n beco es f brotic with

1. Meg k ryocytic proli er tion with bnor l orphol- n ssoci te ecre se in he topoiesis. Bone rrow spi-
ogy, usu lly cco p nie by reticulin n /or coll gen r tion is unsuccess ul in ne rly 90% o p tients bec use

fbrosis reticulin n coll gen f brosis lock in the rrow content,
2. Not eeting the criteri or other MPNs c using ry t p. A bone rrow biopsy shows f brosis, gen-

3. Evi ence o JAK2V617F or other rel te ut tions er lly with incre se nu bers o eg k ryocytes.

MINOR CRITERIA

1. Leukoerythrobl stosis Prognosis

2. Ane i Te e i n surviv l ti e r nges ro 4.3 to 5.0 ye rs. In

3. Incre se seru l ctic ehy rogen se (LDH) levels p tients with PMF, he oglobin concentr tion, pl telet

4. Spleno eg ly count, n the presence o osteo yelosclerosis h ve been

*Di gnosis requires eeting ll three jor n two inor criteri . i entif e s ctors with prognostic signif c nce. P tients

with he oglobin concentr tion less th n 10 g/ L h ve