Haematologica 2003 - Supplements

081
Incidence of solid tumors in cohort of patients with
Monoclonal Gammopathies of Undetermined
Significance along twenty years.
Montañés MA, Franco-García E1, Martos C2, García-
Carpintero G2, Recaséns V, Gómez-López L2, Giralt M,
Rubio-Félix D, Giraldo P.
Miguel Servet University Hospital, Military Hospital1, Aragon
Health Service2. Zaragoza. Spain.
Background: Monoclonal gammopathy of undetermined
significance (MGUS) is a disorder frequently related to
immunological disfunctions of B-cell lines and aging, as well as
several types of cancer, with an estimated incidence of 3% in
people over 70s. Purpose of study: to determine the incidence of
neoplastic disease in MGUS patients and to compare with cancer
incidence in general population during a period of 20 years.
Patients & Methods: Since 1967 a cohort of 1,906 patients
diagnosed of MGUS in the Hematology Department of Miguel
Servet University Hospital was followed-up to 2002 (population
at risk: 533,946 inh). A longitudinal, retrospective and descriptive
study has been performed. Data source: clinical reports and
population Cancer Registry of Zaragoza. Variables: demographic
data (age, gender), date of MGUS diagnosis, immunochemical
subtype, date of cancer diagnosis, subtype and location of cancer.
Cohort was stratified according to age and sex. Cancer diagnosis
was considered as previous, concomitant or after MGUS
diagnosis. Results: The incidence rate of MGUS in our
population is 13.4 SD 1.6 cases/105 inh/y, as previously reported;
estimated cancer incidence in our area was 255.2 SD 32.3
cases/105 inh/y for the period 1978-1998. During this period 639
MGUS developed cancer (33.1%), 684 neoplastic disorders were
detected (range 1-3), in 35 cases (5.5%) two different neoplastic
disorders were diagnosis and in 4 cases (0.5%) three different
tumors were. In 25 cases a non-hematological and a
hematological neoplasia were observed. In 180 cases (27.3%)
both MGUS and neoplasia were simultaneous, in 293 cases
(42.8%) cancer diagnosis preceded MGUS detection, and in the
remaining 211 (30.9%) neoplasia appears along the follow-up.
The risk of cancer in patients with MGUS was 4.87 and 1.34
when only non hematological tumor were considered. Mean age:
65.5 SD 13.6 years. Gender: 384 M/255 F. MGUS subtype: IgG
433 (67.7%). IgA 58 (9.1%), IgM 142 (22.3%) and light chain
disease 6 (0.9%); of these 30 were biclonal (4.7%) and 3 were
triclonal (0.5%). Median time from MGUS to cancer was 60.1
SD 23.2 months (range 0-152). Cancer types associated to MGUS
were: 12 MM, 294 lymphoproliferative disorders (NHL, CLL,
HD, LAL), 5 LANL, 39 CMPD (PV, CML, MF, ET), 21 MDS
and 294 non hematological cancers (skin 14.9%, digestive tract
12.6%, larynx and area 8.2%, urinary tract 7.8%, lung 7.1%,
genital tract 4.7%, breast 3.4%, prostate 3.1%, thyroid gland
2.4%, CNS 1.4%, liver or gall bladder 1.1% and others 9.2%).
Incidence rate x 105 inh / year
gastric
M / F
colorectal
M / F
58.0/
45.6
167.5/
lung
M / F
breast
M / F
bladder
M / F
prostate
M
General
population
28.8 /
19.1
92.6 /
-
- /
92.0
53.2 / - 76.5
MGUS 78.8 /
187.2 - / 147.8 / 51.8
population 55.4 110.8 / 13.8 124.6 55.4
Comments: a higher incidence of cancer was observed among
population having MGUS, compared to general population. This
fact must be considered as not incidental, suggesting a strong
relationship between MGUS and neoplasia.
082
SYSTEMIC MANIFESTATIONS ASSOCIATED WITH
MONOCLONAL GAMMOPAPHY
B.Grosbois, E.Laurat, M.Sebillot, C.Cazalets, J.Bracq,
B.Cador, P.Jego
Department of Internal Medicine, CHU Hopital Sud, Rennes
FRANCE
In a retrospective cohort of 580 monoclonal gammopathy (MG)
from a single institution we studied the frequency and type of
systemic manifestations non-fortuitously associated with MG.
After exclusion of amyloïdosis and cryoglobulinemia we found
30 MG patients (5,2 %) presenting with systemic manifestations
(26 with one single and 2 with double). The distribution
according to the type of manifestations was 14 neurologic (12
peripheral neuropathy, 2 motoneurone disease), 8 dermatologic (2
urticaria, 2 facial oedema, 1 vascular purpura, 1 necrobiotic
xauthogranuloma, 1 pyodema gangrenosum and 1 buccal aphtous
associated with splenic aseptic abscesses), 6 rheumatologic (seronegative
polyarthritis), 4 hematologic (1 auto immune hemelytic
anemia, 1 erythroblastopenia, 1 acquired factor V deficiency, 1
acquired factor VIII deficiency). In 26 patients systemic
manifestations revealed MG. Immunochemical type MG were
IGM(20),IGG(9) and 1 IgA (1). MG were classified as 17
Waldenström’s Macroglobulinemia, 9 MGUS and 4 Multiple
Myeloma. Sixteen patients received specific treatment of MG
(polychemotherapy, chlorambucil) with improvement in 13 cases.
Seven patients received steroid therapy with immunosuppressive
agents (cyclophosphamide, azathioprime) and improvement was
observed in 6 cases.
We conclude that associated sytemic manifestations, although
rare (5 %), often reveal MG. These manifestations are related to
auto-antibody activity of MG The most frequent type of MG is
IgM related to Waldenströms’s Macroglobulinemia.. Specific
treatment can frequently improve these manifestations.
083
THE INCIDENCE OF SUBCLINICAL BONE DISEASE IN
PATIENTS WITH MGUS OR SMOULDERING MYELOMA.
J. Sanders, B. Crawford, J. Gibson, L. Kerr, P.J. Ho, H.
Iland, G. Young, D.E. Joshua.
Institute of Haematology and Department of Endocrinology, Royal
Prince Alfred Hospital, Sydney, Australia
This two-part study was designed to assess the incidence of
subclinical bone disease in patients with monoclonal
gammopathy of unknown significance (MGUS) or untreated
smouldering myeloma using markers of bone turnover, resorption
and bone density measurements. In patients in whom
abnormalities are identified we plan to assess the effects of a
single dose of zoledronic acid (Zometa©).
Patients attending our myeloma clinic were screened using the
following criteria.
Age between 50 and 80 years, premenopausal women excluded.
Diagnosis of MGUS or smouldering myeloma and the
demonstration of a stable paraprotein, IgA, IgG or light chain
disease without evidence of lytic lesions.
No previous chemotherapy or treatment for myeloma (including
bisphosphonates).
No use of glucocorticoids, calcitriol, estrogen or androgen
hormone therapy, calcium or vitamin D supplementation in the 3
months prior to screening.
Normal liver and kidney function.
Assessment involved baseline measurement of:
Serum bone density specific alkaline phosphatase (serum ostase)
S124