Embed Size (px)
Citation preview
COMMENTARY
When is myeloma, myeloma?
MORIE A. GERTZ
Mayo Clinic, Department of Hematology, Rochester, MN 55905, USA
In this issue of Leukemia and Lymphoma, Dimopou-
los and colleagues [1] report on a unique subset of
patients with multiple myeloma. This subset is not
the patients under the age of 40, which accounts for
2% of all patients with this disease. Rather, the
intriguing populations that they report are those
patients who appear to have multiple myeloma but
do not have 10% plasma cells in the bone marrow.
Experienced clinicians recognize that for the pur-
poses of clinical trials, multiple myeloma requires the
presence of 410% plasma cells in the bone marrow.
Experts nonetheless realize that patients can present
with multiple myeloma, large monoclonal proteins,
bony destructive lesions seen on plain radiographs,
but without a sufficient number of plasma cells to
‘‘diagnose’’ multiple myeloma. How are we to deal
with these patients, and what is their natural history?
Kyle et al. [2], reporting on 1027 patients with
multiple myeloma, identify 4% who have 510%
plasma cells in the bone marrow. This paper verifies
that patients can have myeloma but not fulfill the
standard diagnostic criteria as published by the
International Working Group, which requires
410% plasma cells in the bone marrow [3].
Patients with 510% plasma cells have presented in
one of two ways. In the first, patients present with a
solitary plasmacytoma, and subsequently, over a
period of years, go on to develop multiple other lytic
lesions with ‘‘relatively’’ normal bone marrows
containing low numbers of plasma cells in the bone
marrow. It is worth noting that eight of the ten
patients described in [1] had fewer than 5% plasma
cells in their bone marrow. As one might guess, these
patients do well, and as noted by Dimopoulos et al.
[1], none of these patients had a hemoglobin 510
and all had normal serum creatinine which would fit
with low tumor mass. These patients excrete very
modest amounts of light chains, which would not
be expected to produce toxicity to the kidneys. In
the second presentation, these patients appear to
have traditional multiple myeloma but simply do not
have sufficient plasma cells to fit our arbitrary cutoff
of 10%. In the current manuscript [1], a number of
important observations were made including a
prolonged survival, which may or may not be solely
related to these patients being international stage
system I [4]. Larger numbers would need to be
reported to demonstrate an impact of the percentage
bone marrow plasma cells independent of stage. This
paper reports durable responses and prolonged
survivals following high-dose therapy and stem cell
transplant, although this trend has not achieved
statistical significance due to small numbers.
Whether this entity should be referred to as
macrofocal multiple myeloma, multiple myeloma
with 510% plasma cells, or ‘‘multiple solitary plas-
macytomas’’ has yet to be determined by any
consensus panels. The take-home message goes well
beyond the fact that there are patients under the age of
40 with multiple myeloma and emphasizes that
the practice of medicine continues ‘‘without rules’’.
A clinician must keep in mind that published criteria
for diagnosis for a whole host of diseases are
consensus based and are not absolute, although may
be required for the rigorous study of the disorder [3].
In patients with the typical clinical appearance of
multiple myeloma including lytic bone lesions, 510%
marrow plasma cells and excluding amyloidosis,
this unique entity labeled by Dimopoulos et al.
[1] as ‘‘macrofocal multiple myeloma’’ must be kept
in mind since these patients appear to have better
outcomes when exposed to the treatment that we
now accept as standard of therapy for multiple
myeloma in its classic form.
Correspondence: Morie A. Gertz, Mayo Clinic, Department of Hematology, 200 First Street, SW, Rochester, MN 55905, USA. Tel: 1 507 284 4102.
E-mail: [email protected]
Leukemia & Lymphoma, August 2006; 47(8): 1452 – 1453
ISSN 1042-8194 print/ISSN 1029-2403 online � 2006 Informa UK Ltd.
DOI: 10.1080/10428190600667622
Leu
k L
ymph
oma
Dow
nloa
ded
from
info
rmah
ealth
care
.com
by
Uni
vers
ity o
f U
lste
r at
Jor
dans
tow
n on
10/
27/1
4Fo
r pe
rson
al u
se o
nly.
References
1. Dimopoulos MA, Pouli A, Anagnostopoulos A, Repoussis P,
Symeonidis A, Terpos E, et al. Macrofocal multiple myeloma in
young patients: A distinct entity with favorable prognosis. Leuk
Lymphoma 2006;47:1553 – 1556.
2. Kyle RA, Gertz MA, Witzig TE, Lust JA, Lacy MQ,
Dispenzieri A, et al. Review of 1027 patients with newly
diagnosed multiple myeloma. Mayo Clin Proc 2003;78:21 – 33.
3. Durie BG, Kyle RA, Belch A, Bensinger W, Blade J,
Boccadoro M, et al. Myeloma management guidelines: a
consensus report from the Scientific Advisors of the Interna-
tional Myeloma Foundation. Hematol J 2003;4:379 – 398.
4. Greipp PR, San Miguel J, Durie BG, Crowley JJ, Barlogie B,
Blade J, et al. International staging system for multiple
myeloma. J Clin Oncol 2005;23:3412 – 3420.
When is myeloma, myeloma? 1453
Leu
k L
ymph
oma
Dow
nloa
ded
from
info
rmah
ealth
care
.com
by
Uni
vers
ity o
f U
lste
r at
Jor
dans
tow
n on
10/
27/1
4Fo
r pe
rson
al u
se o
nly.
