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[Outcomes of AL-amyloidosis treatment with bortezomib, dexamethasone and cyclophosphamide or doxorubicin-containing regimens].
Vnitr Lek. 2012 Dec; 58(12):896-903.VL

Abstract

According to the criteria for multiple myeloma, systemic AL-amyloidosis may be divided into primary systemic AL-amyloidosis, where monoclonal gametopathy is present but the criteria for multiple myeloma are not satisfied, and systemic AL-amyloidosis with underlying multiple myeloma. There is a continuous transition between the two units. The present paper describes treatment of patients with established systemic AL-amyloidosis who satisfy the 2003 International Myeloma Working Groups criteria for symptomatic multiple myeloma (confirmed monoclonal immunoglobulin, clonal plasmocytes confirmed in the bone marrow and at least one clinical symptom of myeloma - confirmed amyloid). From 2009, a total of 10 patients with AL-amyloidosis and underlying multiple myeloma have been treated at our centre with combined bortezomib-containing regimens. The cohort includes 5 women and 5 men. Median age of these AL-amyloidosis patients at the diagnosis was 65.5 years. All 10 patients were treated with a combination of 3 drugs, bortezomib, cyclophosphamide and dexamethasone or bortezomib, doxorubicin a dexamethasone. Two of the 10 patients died during the first month of treatment. Treatment response cannot be evaluated in these patients. Haematological treatment response was evaluable in 8 patients only. Monoclonal immunoglobulin disappearance with negative urine and serum immunofixation and normalization of free light chain immunoglobulins was observed in six of the 8 patients. Treatment response according to the current IMWG was evaluated as very good partial remission (VGPR) as we did not perform bone marrow testing after the treatment to confirm complete remission according to the current criteria. One of the 8 evaluated patients died due to disease progression in the third month of treatment and there was no haematological treatment response in one who was considered to have a stable disease. Organ treatment response was evaluated in patients who were followed up for longer than 3 months of treatment only. Organ treatment response (reduced cardiac impairment) was not evaluable in a patient who had heart transplantation and then received chemotherapy. A total of 5 (83%) of the 6 evaluated patients fulfilled the criteria of organ treatment response.

CONCLUSION

Our small cohort showed a high number of haematological treatment responses (VGPR in 75% of patients) as well as organ treatment response in patients with systemic AL-amyloidosis who were treated with bortezomib-containing treatment regimens.

Authors+Show Affiliations

Interni hematologicka a onkologicka klinika lekarske fakulty MU a FN Brno. z.adam@fnbrno.czNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

English Abstract
Journal Article

Language

cze

PubMed ID

23427947

Citation

Adam, Z, et al. "[Outcomes of AL-amyloidosis Treatment With Bortezomib, Dexamethasone and Cyclophosphamide or Doxorubicin-containing Regimens]." Vnitrni Lekarstvi, vol. 58, no. 12, 2012, pp. 896-903.
Adam Z, Stork M, Pour L, et al. [Outcomes of AL-amyloidosis treatment with bortezomib, dexamethasone and cyclophosphamide or doxorubicin-containing regimens]. Vnitr Lek. 2012;58(12):896-903.
Adam, Z., Stork, M., Pour, L., Krejčí, M., Zahradová, L., Sandecká, V., Hájek, R., Cermáková, Z., Pospíšilová, Y., Navrátil, M., Král, Z., & Mayer, J. (2012). [Outcomes of AL-amyloidosis treatment with bortezomib, dexamethasone and cyclophosphamide or doxorubicin-containing regimens]. Vnitrni Lekarstvi, 58(12), 896-903.
Adam Z, et al. [Outcomes of AL-amyloidosis Treatment With Bortezomib, Dexamethasone and Cyclophosphamide or Doxorubicin-containing Regimens]. Vnitr Lek. 2012;58(12):896-903. PubMed PMID: 23427947.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Outcomes of AL-amyloidosis treatment with bortezomib, dexamethasone and cyclophosphamide or doxorubicin-containing regimens]. AU - Adam,Z, AU - Stork,M, AU - Pour,L, AU - Krejčí,M, AU - Zahradová,L, AU - Sandecká,V, AU - Hájek,R, AU - Cermáková,Z, AU - Pospíšilová,Y, AU - Navrátil,M, AU - Král,Z, AU - Mayer,J, PY - 2013/2/23/entrez PY - 2013/2/23/pubmed PY - 2013/10/23/medline SP - 896 EP - 903 JF - Vnitrni lekarstvi JO - Vnitr Lek VL - 58 IS - 12 N2 - UNLABELLED: According to the criteria for multiple myeloma, systemic AL-amyloidosis may be divided into primary systemic AL-amyloidosis, where monoclonal gametopathy is present but the criteria for multiple myeloma are not satisfied, and systemic AL-amyloidosis with underlying multiple myeloma. There is a continuous transition between the two units. The present paper describes treatment of patients with established systemic AL-amyloidosis who satisfy the 2003 International Myeloma Working Groups criteria for symptomatic multiple myeloma (confirmed monoclonal immunoglobulin, clonal plasmocytes confirmed in the bone marrow and at least one clinical symptom of myeloma - confirmed amyloid). From 2009, a total of 10 patients with AL-amyloidosis and underlying multiple myeloma have been treated at our centre with combined bortezomib-containing regimens. The cohort includes 5 women and 5 men. Median age of these AL-amyloidosis patients at the diagnosis was 65.5 years. All 10 patients were treated with a combination of 3 drugs, bortezomib, cyclophosphamide and dexamethasone or bortezomib, doxorubicin a dexamethasone. Two of the 10 patients died during the first month of treatment. Treatment response cannot be evaluated in these patients. Haematological treatment response was evaluable in 8 patients only. Monoclonal immunoglobulin disappearance with negative urine and serum immunofixation and normalization of free light chain immunoglobulins was observed in six of the 8 patients. Treatment response according to the current IMWG was evaluated as very good partial remission (VGPR) as we did not perform bone marrow testing after the treatment to confirm complete remission according to the current criteria. One of the 8 evaluated patients died due to disease progression in the third month of treatment and there was no haematological treatment response in one who was considered to have a stable disease. Organ treatment response was evaluated in patients who were followed up for longer than 3 months of treatment only. Organ treatment response (reduced cardiac impairment) was not evaluable in a patient who had heart transplantation and then received chemotherapy. A total of 5 (83%) of the 6 evaluated patients fulfilled the criteria of organ treatment response. CONCLUSION: Our small cohort showed a high number of haematological treatment responses (VGPR in 75% of patients) as well as organ treatment response in patients with systemic AL-amyloidosis who were treated with bortezomib-containing treatment regimens. SN - 0042-773X UR - https://www.unboundmedicine.com/medline/citation/23427947/[Outcomes_of_AL_amyloidosis_treatment_with_bortezomib_dexamethasone_and_cyclophosphamide_or_doxorubicin_containing_regimens]_ L2 - https://www.prolekare.cz/linkout/39783 DB - PRIME DP - Unbound Medicine ER -