OncoLog, Volume 56, Number 1, January 2011 Page: 2
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Myeloproliferative Disorders
[Continued from page 1]post-thrombocythemia
myelofibrosis. Also, any of
the three classic myelo-
proliferative disorders can
progress to acute myeloge-
nous leukemia.
Complications from
myeloproliferative
disorders
Patients with essential
thrombocythemia, charc-
terized by excess platelet
production, have a nor-
mal life expectancy in
general, although they
face an increased risk of
blood clots that may af-
fect their quality of life.
Each year, 6,000-7,000
new cases of essential
thrombocythemia are
diagnosed in the United
States.
In patients with poly-
cythemia vera, an abnor-
mal proliferation of all
hematopoietic bone mar-
row elements increases
the total blood volume.
The main complication
this causes for patients
is a high red blood cell
count, which leads to in-
creased clotting similar to
that caused by essential
thrombocythemia. Pa-
tients with polycythem~a
vera have a somewhat
shorter life expectancy than is n
for their age group. Polycythemi
has about the same incidence ra
essential thrombocythemia.
Primary myelofibrosis is less
mon than the other two classic
proliferative disorders. Fach yea
3,000 people in the United Stat
diagnosed with myelofibrosis, in
patients with post-poly:ythemia
post-thrombocythemia myelofib
(who account for up to a quarte
patients with myelofibrosis). In
with myelofibrosis, bore marrow
grow without control, and the b
marrow stromal cells react by se
a number of different proteins t]A ABOVE: Photos of a patient before therapy with an experimen-
tal JAK2 inhibitor show the distended abdomen caused by the
enlarged spleen, a common symptom of myelofibrosis.
V BELOW: Photos taken after 2 months of therapy with a JAK2
inhibitor show a marked reduction in the patient's splenomegaly-
iormal to the formation of fibers in the bone blood ar
ia vera marrow. This scarring prevents the mastocy
ite as bone marrow from being able to pro- cells int
duce enough blood cells. philic ir
com- As the body tries to compensate for potential
myelo- the lack of red blood cells produced by pereosin
r, about the bone marrow, the spleen enlarges is usuall
es are and contributes to the production of and ma
cluding blood cells. Abnormal blood cells can cause eo
and also infiltrate the spleen and other or- part of r
rosis gans (e.g., the liver). As a result, spleen tients w
r of all enlargement occurs in about 80% of and syst
patients patients with myelofibrosis. These pa- allergic
w cells tients' spleens may double or even of these
one triple in size. The liver is enlarged in in their
creting about 40% of myelofibrosis patients. rare," D
hat lead Fatigue is the most common symp- a good1
of eosinophils in the
nd bone marrow) and systemic
tosis (an infiltration of mast
o non-skin tissues). Eosino-
nfiltration of the organs is a
ally deadly characteristic of hy-
ophilic syndrome. Mastocytosis
y confined to the bone marrow
y be indolent or aggressive. Be-
osinophils and mast cells are
normal allergic reactions, pa-
ith hypereosinophilic syndrome
emic mastocytosis experience
reactions and skin rashes. Both
disorders tend to affect patients
40s. "These disorders are very
r. Verstovsek said. "Nobody has
data base of these patients, and2 OncoLog January 20.1
C
tom of myelofibrosis.
Many myelofibrosis pa-
tients also experience de-
creased appetite, weight
loss, and malnutrition as
a result of the enlarged
spleen pressing on the
stomach. These patients
tend to have decreased
performance status and
poor quality of life. As
the disease progresses,
patients may have in-
creasing weakness, pro-
gressive enlargement of
the liver and spleen, liver
failure, portal hyperten-
sion causing bleeding in
the gastrointestinal tract,
pulmonary hypertension,
lung failure, and cardiac
failure. "Most of these
patients die from body
wasting, organ failure,
and similar disease com-
plications within 5-7
years," Dr. Verstovsek
said. About 20% of cases
of myelofibrosis progress
to acute myelogenous
leukemia, he added, and
the average patient sur-
vival after such progres-
sion is only 5 months.
Atypical myeloprolif-
erative disorders include
hypereosinophilic syn-
drome (an excess number
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University of Texas M.D. Anderson Cancer Center. OncoLog, Volume 56, Number 1, January 2011, periodical, January 2011; Houston, Texas. (https://texashistory.unt.edu/ark:/67531/metapth903078/m1/2/: accessed June 12, 2024), University of North Texas Libraries, The Portal to Texas History, https://texashistory.unt.edu; crediting UNT Libraries Government Documents Department.