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Blood, Vol. 93 No. 3 (February 1), 1999:
pp. 1032-1037
Primary Plasma Cell Leukemia: Clinical, Immunophenotypic, DNA
Ploidy, and Cytogenetic Characteristics
R. García-Sanz,
A. Orfão,
M. González,
M.D. Tabernero,
J. Bladé,
M.J. Moro,
J. Fernández-Calvo,
M.A. Sanz,
J.A. Pérez-Simón,
A. Rasillo, and
J.F. San Miguel
From the Castellano-Leonés Cooperative Group for the Study of
the Monoclonal Gammopathies, Centro de Investigacion del
Cáncer de Salamanca, Institut d'Investigacions Biomédiques
August Pi y Sunyer de Barcelona (IDIBAPS) and the Spanish Cooperative
Group for the Treatment of the Hematological Malignancies (PETHEMA),
and the Department of Hematology, University Hospital of Salamanca,
Salamanca, Spain.
We report on a series of 26 patients diagnosed with primary (de
novo) plasma cell (PC) leukemia (PCL) in whom we analyzed the
clinicobiologic characteristics of the disease together with the
immunophenotype, DNA cell content, proliferative index, and numeric
chromosomal aberrations of the neoplastic PC, and compared them with
664 multiple myeloma (MM) patients at diagnosis. The median age, sex
ratio, and bone lesion extension were similar, but PCL cases displayed
a higher prevalence of clinical stage III, extramedullary involvement,
and Bence Jones cases, with fewer IgA cases than for MM patients. In
addition, according to several prognostic indicators
( 2-microglobulin serum level, proportion of S-phase PCs,
proteinuria, calcium serum level, lactate dehydrogenase [LDH] and
renal function), the incidence of adverse prognostic factors was
significantly higher in PCL versus MM. Immunophenotypic expression was
similar for CD38, CD138, CD2, CD3, CD16, CD10, CD13, and CD15, but PCL
differed from MM in the expression of CD56, CD9 HLA-DR, CD117, and CD20
antigens. Twenty-two PCL cases were diploid and one was hypodiploid,
while most MM cases (57%) showed DNA hyperdiploidy. With the
fluorescent in situ hydridization (FISH) technique, 12 of 13 PCL cases
displayed the numeric aberrations,  13 (86%), ±1 (57%), +18
(43%), and X in women (25%), but they lacked several numeric
aberrations usually found in MM such as +3, +6, +9, +11, and
+15. PCL cases had a lower overall response to therapy than MM cases
(38% v 63%, P = .01332). Among PCL patients, a
trend for a worse response was observed in cases treated with melphalan
and prednisone (MP) versus polychemotherapy. Overall survival was
significantly worse in PCL versus MM patients (8 v 36 months,
P < .0001), but it was significantly better in PCL patients
treated with polychemotherapy versus MP (18 v 3 months, P = .0137). By contrast, MM patients did not show
significant differences in overall survival according to the treatment
used, MP or polychemotherapy. Ten variables seemed to predict survival in PCL patients, but only the 2-microglobulin level and
S-phase PCs retained an independent value in multivariate analysis. In summary, our study illustrates that PCs from PCL display singular phenotypic, DNA cell content, and cytogenetic characteristics that lead
to a different disease evolution versus MM.
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