Haematologica 2003 - Supplements

where sheep were raised, whereas no increased risks were found
for those who lived or worked on a farm where cattle, beef, pigs,
or chickens were raised. We observed a modestly increased risk
for pesticides overall (OR=1.3, 95% CI=0.9-1.8), which was
mainly due to exposure to herbicides (OR=1.5, 95% CI=0.7-3.0)
and fungicides (OR=2.3, 95% CI=0.7-7.9). Significantly
increased risks were also observed among pharmacists, dietitians
and therapists (OR=6.1, 95% CI=1.7-22.5), roofers (OR=3.3,
95% CI=1.1-9.8), heating equipment operators (OR=4.7, 95%
CI=1.4-15.8) and hand molders and casters (OR=3.0, 95%
CI=1.0-8.4). In conclusion, our study suggests a modest
increased risk of multiple myeloma for occupational exposure to
pesticides. The observed increased risk among subjects who
lived or worked on a farm where sheep were raised suggests that
certain animal viruses may be involved in myeloma risk.
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Acquired fanconi’s syndrome is an indolent disorder in
the absence of overt multiple myeloma.
C. X. Ma, M.Q. Lacy, J.F. Rompala, A.Dispenzieri, S.V.
Rajkumar, P.R. Greipp, R. Fonseca, R.A. Kyle, M.A. Gertz
Mayo Clinic
Adult acquired Fanconi’s syndrome (FS) is a rare complication of
plasma cell proliferative disorders. It is characterized by an
absorptive defect of the proximal renal tubules. This results in
aminoaciduria, glycosuria, hypophosphatemia, hypokalemia,
hypouricemia, and metabolic acidosis. Methods: We
retrospectively reviewed 32 patients who were diagnosed with
monoclonal gammopathy associated adult acquired FS between
April 1968 and June 2002. The median follow up of survivors
was 56 months (ranges 2 to 238 months). Results: Various forms
of plasma cell disorders, including Multiple Myeloma (MM),
Smoldering Multiple Myeloma, Waldenstrom’s
Macroglobulinemia (WM), and Monoclonal Gammopathy of
Undetermined Significance (MGUS), were identified in these
patients. 91% of these patients were found to have monoclonal
The remaining 9% of patients have monoclonal light chains in
the urine. The median creatinine at diagnosis was 1.9 mg/dl
(ranges 0.9-3.7). Currently, five patients developed ESRD. The
median time to ESRD was 153 months (range 90-238 months).
Pathologically, 47% of the available kidney biopsies
demonstrated cytoplasmic crystals in the proximal tubular
epithelial cells. One patient developed cytoplasmic crystals in the
transplanted kidney 1 year after the transplant for ESRD
secondary to FS. Only one out of the fourteen MGUS patients
subsequently transformed to overt MM. 44% of the patients died
during the period of follow up. The median survival was
significantly different between patients who had overt MM at
diagnosis (43 months) and MGUS (120 months). Only one
patient died from renal failure. Chemotherapy was given to
twenty-one patients for symptomatic MM or progressive renal
failure. Improvement of renal function was found in only a
minority of patients. However, four patients who were treated
with chemotherapy developed secondary leukemia or
myelodysplastic syndrome. The majority of the patients had
elevated alkaline phosphatase levels, reflecting secondary
osteomalacia. Renal osteodystrophy was found in only one
patient who also had ESRD.
102
IgM Myeloma: a rare subtype. Description of 4 cases
Ombretta Annibali, Maria Teresa Petrucci*, Vincenza
Martini*, Anna Levi*, Carolina Fossati, Cristina Tirindelli.
Ematologia University “Campus Bio-Medico”, *Dipartimento di
Biotecnologie Cellulari ed Ematologia University "La Sapienza”,
Rome- Italy.
The distinction between multiple myeloma (MM) and
Waldenström’s macroglobulinemia (WM) usually poses no
diagnostic dilemma. Consistent with a diagnosis of MM is the
presence non IgM monoclonal gammopathy associated to
multiple osteolytic lesions and plasma cell infiltration of the bone
marrow. On the other hand, characteristic of WM is the presence
of an IgM monoclonal gammopathy associated to
lymphadenopathy, hepatosplenomegaly, anemia, and
hyperviscosity syndrome in conjunction with a monoclonal
lymphoplasmacytoid proliferation in both the bone marrow and
peripheral blood is characteristic . Despite that, few cases of IgM
myeloma have been reported , with clinicopathologic features
intermediate to those of MM and WM.. We present 4 patients
with an IgM monoclonal gammopathy in whom morphologic and
clinical features were consistent with the diagnosis of IgM
myeloma .
From July 1973 to April 2002, we observed 3,176 monoclonal
gammopathies of which 316 (9.9%) were of IgM type. At
diagnosis, 187 (59.1%) were MGUS, 93 (29.4%) were WM, 10
(3,1%) were non Hodgkin Lymphoma (NHL) and only 4 (1,3%)
were IgM Myeloma. Of the 186 MGUS, 21 (11%) evolved to
WM and 1 to NHL during the follow-up .
The following Table reports the clinical characteristics of the 4
IgM myeloma:
case
Sex Age Light
Chain
Stage
IgM
gr/dL
(%)BM
plasma
cells
Therapy Response Months
of
survival
1 F 79 k IIA 1.7 31 MP SD 107
2 F 69 k IIIA 2.2 20 RT PD 31
3 M 65 k Smoldering 1 40 none SD 172
4 M 66 λ IIA 2 60 MP SD 15
SD: stable disease, PD: Progressive Disease Survival after
primary diagnosis, MP: melphalan- prednisone; RT: radiotherapy.
None of the 4 patients had diffuse osteolytic lesions. However, in
case 2, who complained lumbal pain and paresthesia, Magnetic
Resonance demonstrated the presence of L2-L5 fractures with
associated pathologic tissue causing spine compression. The
histologic analysis of this tissue showed a diffuse plasma cells
infiltration.After laminectomy, the patient received local
radiotherapy obtaining a reducion of sympthoms but few months
later he died of progressive disease. Of the remaining 3 patients
only case 3 who had a smoldering IgM myeloma is still alive
after 172 months from diagnosis.
In conclusion, IgM myeloma is a rare disease, accounting for
about 1% of all monoclonal IgM and less then 0.5% of MM. The
distinction between the WM and MM rests on the histological
finding of a lymphoplasmacytoid proliferation in WM as opposed
to the predominantly plasma cell rich infiltrate in myeloma. Since
MM and WM differ in prognosis and treatment strategies, the two
disease entities should be distinguished based on clinical criteria
and bone marrow morphology.
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