Treatment of Waldenström macroglobulinaemia – the experience of one centre
Authors:
Z. Adam; L. Pour; M. Krejčí; Z. Kořístek; M. Navrátil; A. Křivanová; L. Zahradová; R. Hájek
Authors place of work:
Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.
Published in the journal:
Vnitř Lék 2009; 55(1): 9-17
Category:
Original Contributions
Summary
Waldenström macroglobulinaemia (WM) is adisease the incidence of which is approximately 10 times lower than that of multiple myeloma. The incidence of WM is reported to be respectively 0.34 per 100,000 men and 0.7 per 100,000 women. It is one of the less aggressive lymphoproliferative diseases in which therapy is indicated only when the disease symptoms start to manifest. We present aretrospective analysis of patients with the symptomatic form of WM who were treated with chemotherapy in our centre over the last 9 years, i.e. 19 WM patients (of which 7 women and 12 men). The median age upon diagnosis of the symptomatic form of WM and start of treatment was 59 (51–78) years. The median follow up for all patients was 31(7–121) months. The most frequent indication for WM treatment was anaemia in 57% (11/19), thrombocytopaenia in 21% (4/19), plasma hyperviscosity in 42% (8/19), symptomatic lymphadenopathy in 15% (3/19), and pathologic osteolysis in 15% (3/19) of cases, respectively. Decrease in immunoglobulin concentration under the lower physiological limit is not referred to as acharacteristic sign of WM, yet it was recorded in 7out of 19patients in our patient group and continued after chemotherapy, with the affected patients suffering from infections in remission more often than those with physiological values of immunoglobulins. 36% (7/19) patients were treated with R‑FC (rituximab+fludarabine+cyclophosphamide) therapy, 15% (3/19) with FC therapy, 15% (3/19) with R‑CHOP concluded by high‑dose chemotherapy with autologous transplantation in 1case, and 5% (1/19) with CHOP therapy. VAD (vancristine, ariamycine and dexamethasone) therapy was used in 10% (2/19) of patients and 10% (2/19) of patients were treated with oral chlorambucil. The first line of treatment achieved complete remission (CR) in 15% cases (3/15). Two patients who achieved CR were treated with R‑FC, and one patient with VAD. Partial remission (PR) was achieved by 63% of patients (12/19). Minor response (MR) was achieved by 10% (2/19), and stable disease (SD) was recorded in 10% of patients (2/19) after initial treatment. The first relaps of the disease was recorded in 5patients (2 of whom had PR and 3 MR) who achieved remission after additional lines of therapy. Thirteen patients are in the first PR. With afollow up median of 31months (7–121), only 2 patients died from other than the underlying disease. Additional malignant disease was diagnosed in 3 patients: 1colon carcinoma, 1acute myeloid leukaemia (AML) and 1myelodysplastic syndrome (MDS). All three received fludarabine and cyclophopsphamide as initial therapy. Of the three patients only the patient with MDS has survived and is now without any evidence of the presence of WM, i.e. in complete remission at 41months after the start of therapy. High‑dose chemotherapy with autologous transplantation was used in 2 cases, once as part of initial therapy and once as part of the therapy for the first relaps resistant to R‑CHOP chemotherapy. It is not possible to determine the median of the first remission or the median of total survival due to the short follow up interval.
Key words:
Waldenström macroglobulinaemia (Morbus Waldenström)– lymphoplasmacytic lymphoma– fludarabine– rituximab– myelodysplastic syndrome– acute myeloid leukaemia
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Štítky
Diabetology Endocrinology Internal medicineČlánok vyšiel v časopise
Internal Medicine
2009 Číslo 1
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