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Blood, 1 December 2001, Vol. 98, No. 12, pp. 3249-3255
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Diagnostic and clinical relevance of the number of circulating
CD34+ cells in myelofibrosis with myeloid
metaplasia
Giovanni Barosi,
Gianluca Viarengo,
Alessandro Pecci,
Vittorio Rosti,
Giovanna Piaggio,
Monia Marchetti, and
Francesco Frassoni on behalf of the Investigators of
the Italian Registry of Myelofibrosis
with Myeloid Metaplasia
From the Laboratory of Medical Informatics, the Unit of
Clinical Immunology and Immunohematology, the Transfusion Service, and
the Department of Internal Medicine and Clinical Oncology, Istituto di
Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo,
Pavia, Italy; and the Division of Hematology, Ospedale San Martino,
Genoa, Italy.
The absolute content of CD34+ cells in the peripheral
blood of 84 patients with myelofibrosis with myeloid metaplasia (MMM) and 23 patients with other Philadelphia-negative (Ph )
chronic myeloproliferative disorders (CMDs) was investigated. In MMM, the median absolute number of circulating CD34+
cells was consistently high (91.6 × 106/L; range,
0-2460 × 106/L). Receiver operating
characteristic curve analysis showed that 15 × 106/L as a decision criterion for CD34+
cells produced an almost complete discrimination between MMM patients
out of therapy and other Ph CMDs (positive predictive
value, 98.4%; negative predictive value, 85.0%). MMM patients with
higher numbers of CD34+ cells had a significantly longer
disease duration (P = .019) and higher spleen volume
index (P = .014), liver volume (P = .000), percentage of circulating immature myeloid cells
(P = .020), and percentage of myeloid blasts
(P = .000). When CD34+ cells were correlated
with the use of Dupriez risk stratification, CD34+ cells
increased significantly from low-risk (median,
68.1 × 106/L) to intermediate-risk (median,
112.8 × 106/L) and high-risk patients (median
666.1 × 106/L) (F = 4.95;
P = .009). When CD34+ cells were correlated
with a severity score on the basis of both myeloproliferative and
myelodepletive characteristics of the disease, only the
myeloproliferation index was significantly associated with
CD34+ cell level (F = 5.7;
P = .000). Overall survival and interval to blast
transformation from the time of CD34+ cell analysis were
significantly shorter in patients with more than
300 × 106/L CD34+ cells
(P = .005 and .0005, respectively). In conclusion, the absolute number of CD34+ circulating cells allows MMM to be
distinguished from other Ph CMDs; it is strongly
associated with the extent of myeloproliferation and predicts evolution
toward blast transformation.
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