Haematologica 2003 - Supplements

intervals 5.71 - 36.09 months) versus 54.93 months ( 52.05 -
57.82, p = 0.000 ). These findings confirm that ASCT may
produce prolonged survival in PCL. However, as for conventional
therapy, outcomes are significantly inferior to those in multiple
myeloma despite the fact that any group of PCL patients
achieving autograft are biologically pre-selected through early
morbidity and mortality during initial chemotherapy. Further
study of novel treatment strategies is required to produce progress
in the treatment of this aggressive disease.
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Autologous transplantation in primary plasma cell
leukaemia: an EBMT study comparing presenting
features and outcome with common-type myeloma
M. Drake*, T.C.M.Morris*, T. Löppönen**, B. Bjorkstrand**,
G. Gahrton**, J. Apperley+.
*Haematology Department, Belfast City Hospital, Northern Ireland;
** Huddinge University Hospital, Stockholm, Sweden; +Royal Post
Graduate Medical School, Hammersmith Hospital, London
Primary plasma cell leukaemia ( PCL ) is a rare disorder,
characterised by plasma cells circulating in the peripheral blood
at absolute numbers > 2.0 x 109/l or greater than 20% of
peripheral leucocytes. The disorder is frequently advanced at
presentation and is often rapidly progressive with a median
survival of 8-12 months with conventional chemotherapy,
significantly shorter than for multiple myeloma. High dose
therapy and autologous haemopoietic transplantation ( ASCT )
has been reported to produce prolonged survival but as with
conventional therapy, may produce inferior outcome relative to
that seen in multiple myeloma, as reported in our previous study
of 56 patients with PCL . Here, we report an EBMT registry
study of an extended series of 135 patients with PCL and 9887
patients with multiple myeloma undergoing first ASCT. Data was
obtained from Med-A ( limited data set ) and Med-B ( extended
data set ) forms. Patient groups were comparable with regard to
gender and performance status pre-transplantation. PCL patients
were significantly younger at diagnosis ( 51.9 v 53.6 years, p =
0.022 ) and transplantation ( 52.7 v 55.1 years, p = 0.002 ). There
were significant differences in presenting serum creatinine ( 114
v 91 micro mol/l, p = 0.021 ) and haemoglobin ( 8.75 v 10.9 g/dl,
p = 0.000 ), while beta 2 microglobulin was raised at diagnosis in
71.8 v 30.8% of PCL v multiple myeloma patients ( p = 0.000 )
respectively. PCL patients were more likely than myeloma
patients to have normal bone structure ( 54.1% v 32.1%, p=0.02 ).
No difference was seen in levels of M protein, calcium or
albumen at diagnosis. There was a non-significant trend to higher
stage at diagnosis in the PCL patients with 64.5 % of commontype
myeloma patients and 78.7% of PCL patients in Stage III ( p
= 0.077). There was a tendency for more favourable disease
status pre-transplantation in the PCL patients with 32% and
28.8% of PCL patients and 16.1% and 15% of myeloma patients
in complete remission at mobilisation ( p = 0.074 ) and transplant
conditioning ( p = 0.000 ) respectively. PCL patients were
transplanted a median of 6.57 months from diagnosis compared
to 8.25 months for the myeloma group ( p = 0.000 ). Use of TBI
in the conditioning regime and graft-type ( marrow, peripheral
blood or both ) were similar for the groups. Survival of PCL
patients post-ASCT was markedly inferior to that of myeloma
patients with a median survival of 20.9 months ( 95% confidence
299
Efficacy of thalodomid treatment after up- front high
dose chemotherapy followed by autologous peripheral
stem cell transplantation in AL amyloidosis
M. Maerevoet (1.2), E. Van Den Neste (2), J. Jamez(1), M.
Donnay(3), A. Ferrant(2) and L. Michaux(2)
Clinique Saint- Pierre, Ottignies ;Cliniques Universitaires Saint-Luc,
UCL, Bruxelles ;IPG,Loverval ;Belgium
Introduction: Primary amyoidosis( P.A) is a rare plasma cell
dyscrasia in which insoluble immunoglobulin light chain
fragments are produced and polymerize into fibrils that deposit
extracellularly, causing viceral organ dysfunction. The most
important prognostic factor is the presence of cardiac
involvement.
Case: A 51-year-old woman was admitted because of systemic
oedema and proteinuria.Her blood pressure was 120/90 mmHg, ,
weight 63.7 Kg. Urinalysis demontrated severe proteinuria at
3.588g/day. Blood urea and creatinine were 30 and 0.8 mg/dl,
respectively. Total serum protein /albumin was 4.5/2 g/dl . Blood
and urine Immunoelectrophoresis demonstrated two light chains.
The level of total and LDL cholesterol was 432 and 328 mg/dl.
The blood test normal for WBC, platelet and RBC. Cardiac
echography as normal. Kidney biopsy showed amyloid deposition
(congo red histology and immunofluorescence study) leading to a
diagnosis of AL amyloidosis.
Myelogram and Bone Rx examination were negative.
The proteinurie rapidly increased to 6.315 mg/dl.
High dose Melphalan (200 mg/m2) followed by autologous stem
cell Tx was performed in augustus 2001, 8 month after diagnosis.
The havest was performed under GCSF alone.
After the Tx, proteinuria initially regressed to 4.8 g/24h. Because
this patient suufered from HTA,Lisinopril was given at dose of
20 mg/day.
Quickly, proteinuria raised to 6.2 g /24H 5 month after the PBSC;
aspirin at 100 mg/ day and sartan at 50 mg were added.
Thalidomide 400 mg/day was startet 6 month after Tx,and slowly
regression of proteinuria was seen with normalisation of
albuminemia and total serum proteinemia, The patient developed
hypothyroidy 5 month after Thal.
In January 2003, 13 months after Thal, the proteinuria as 2.1 g%,
albuminemia and total serum proteinemia as 2.9 and 4.9g/dl.
But, she developed polyneuropathy, the doses of Thal was
reduced to 100 mg/day.
Conclusion: Thalidomid showed efficacy in a case of refractory
amyloidosis but the efficacy of this observation needs more
patients to be studied.
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