Nephrotic Syndrome Due to Primary AL Amyloidosis, Successfully Treated with VAD and Subsequent High-dose Melphalan Followed by Autologous Peripheral Blood Stem Cell Transplantation.

Gono, Takahisa; Matsuda, Masayuki; Dohi, Naoko; Hoshi, Kenichi; Tada, Tsuyoshi; Sakashita, Kazuo; Koike, Kenichi; Aizawa, Masatsugu; Ikeda, Shu‐ichi

doi:10.2169/internalmedicine.42.72

Cited by 20 publications

(19 citation statements)

References 24 publications

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“…The combination of VAD and HDM for treatment of AL amyloidosis with nephrotic syndrome has been shown to be feasible in one patient with nephrotic syndrome (Sezer et al, 1999) and in reports on small cohorts (van Gameren et al, 2002;Gono et al, 2003). However, data on toxicity and efficacy of VAD chemotherapy prior to HDM and ASCT are limited in patients with AL amyloidosis.…”

Section: Discussionmentioning

confidence: 99%

“…They demonstrated a significantly better survival rate for the group of patients treated with HDM chemotherapy (Dispenzieri et al, 2004). The use of additional induction chemotherapy prior to HDM has been evaluated in a few studies to date (Sezer et al, 1999;van Gameren et al, 2002;Gono et al, 2003). New regimens, including induction chemotherapy prior to HDM need to be tested in order to assess whether the results of this regimen can be improved in terms of toxicity and efficacy.…”

Section: Discussionmentioning

confidence: 99%

“…Vincristine, adriamycin and dexamethasone is well known to be effective in multiple myeloma (Barlogie et al, 1984) and has been reported to be feasible and effective in patients with AL amyloidosis (Sezer et al, 1999;van Gameren et al, 2002;Gono et al, 2003). High-dose dexamethasone, which is probably the most effective part of this combination chemotherapy has been evaluated in combination with low-dose melphalan (Palladini et al, 2001(Palladini et al, , 2004.…”

Section: Discussionmentioning

confidence: 99%

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High‐dose melphalan with autologous stem cell transplantation after VAD induction chemotherapy for treatment of amyloid light chain amyloidosis: a single centre prospective phase II study

Perz¹,

Schönland²,

Hundemer³

et al. 2004

Br J Haematol

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SummaryAmyloid light chain (AL) amyloidosis is the result of a clonal plasma cell expansion, in which monoclonal light chains transform to amyloid deposit in various tissues and can lead to organ dysfunction and organ failure. The median survival of patients with AL amyloidosis without therapy is 10–14 months. With high‐dose melphalan (HDM) and autologous stem cell transplantation (ASCT), haematological and clinical remission rates of up to 50% of treated patients have been reported from phase II studies. HDM followed by ASCT appears to prolong survival in patients, if haematological remission can be reached. In this phase II study, we evaluated vincristine, adriamycin and dexamethasone (VAD) as induction chemotherapy prior to stem cell mobilization and HDM with ASCT. The regimen was, in general, feasible in patients with AL amyloidosis, but VAD chemotherapy had a considerable World Health Organization (WHO) grade III–IV toxicity (25%) and mortality (7%) rate. VAD pretreatment did not interfere with stem cell mobilization and HDM with ASCT was possible in 86% of patients. The overall treatment efficacy was comparable with reported results of HDM and ASCT without preceding chemotherapy. We could not show an additional benefit of VAD induction in terms of increasing haematological response rate; however the 13% mortality rate after HDM and ASCT in our series was lower than the previous report.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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High‐dose melphalan with autologous stem cell transplantation after VAD induction chemotherapy for treatment of amyloid light chain amyloidosis: a single centre prospective phase II study

Perz¹,

Schönland²,

Hundemer³

et al. 2004

Br J Haematol

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“…S o l i d l i n e : t h e r e mi s s i o n g r o u p ( n = 4 ) , b r o k e n l i n e : t h e n o n -r e mi s s i o n g r o u p ( n = 4 ) . Ce n s o r e d p a t i e n t s a r e t i c k e d a l o n g t h e s u r v i v a l c u r v (6)(7)(8)13). Several recent reports have demonstrated that some patients with primary systemic AL amyloidosis show good therapeutic outcomes after cyclic administration of VAD alone (5,14).…”

Section: Therapeutic Outcomesmentioning

confidence: 99%

Therapeutic Outcome of Cyclic VAD (Vincristine, Doxorubicin and Dexamethasone) Therapy in Primary Systemic AL Amyloidosis Patients

et al. 2008

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“…Details of the intensive chemotherapy are described elsewhere (11,12). Briefly, stem cells were collected using etoposide and granulocyte-colony stimulating factor after 2 courses of VAD as an induction treatment, and high-dose melphalan was administered intravenously at 140 mg/m 2 total dose over 2 days.…”

Section: Treatmentmentioning

confidence: 99%

Serum Levels of Free Light Chain before and after Chemotherapy in Primary Systemic AL Amyloidosis

et al. 2005

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Objective Immunoglobulin-related free light chains (FLCs) in serum have recently become quantitatively detectable using the nephelometric assay in plasma cell disorders, including multiple myeloma and AL amyloidosis. To investigate whether FLCs are useful as a diagnostic and therapeutic marker in Japanese patients with primary systemic AL amyloidosis, we determined these values in serum before and after chemotherapy.Patients and Methods The serum FLC analysis was carried out in 25 patients with primary systemic AL amyloidosis (mean age, 60.1±8.4 years). All of the patients were shown to have either AL -or ALimmunoreactive amyloid deposits on biopsied tissues. Thirteen patients were treated with VAD (vincristine, doxorubicin and dexamethasone) alone (n=6) or VAD and subsequent high-dose melphalan followed by autologous stem cell support (n=7), and serum FLCs were serially determined before and after the chemotherapy.Results Before chemotherapy the amyloidogenic FLC was elevated in serum with or without abnormal / ratios in 24 patients, including 5 with undetectable Mprotein in both serum and urine on immunofixation. After chemotherapy the amyloidogenic FLC in serum was significantly decreased irrespective of high-dose melphalan (p<0.05), and all the patients with normalized / ratios showed a good prognosis. Conclusions With respect to sensitivity and quantification serum FLCs will be a key marker for diagnosis and therapeutic effects in primary systemic AL amyloidosis. The prognosis of patients with this disease may be improved if the / ratio in serum can be normalized by intensive chemotherapy.

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Nephrotic Syndrome Due to Primary AL Amyloidosis, Successfully Treated with VAD and Subsequent High-dose Melphalan Followed by Autologous Peripheral Blood Stem Cell Transplantation.

Abstract: Primary ALamyloidosis involves vital organs from the early phase of illness, resulting in a poor prognosis. We report a patient with nephrotic syndrome due to this type

Cited by 20 publications

References 24 publications

High‐dose melphalan with autologous stem cell transplantation after VAD induction chemotherapy for treatment of amyloid light chain amyloidosis: a single centre prospective phase II study

High‐dose melphalan with autologous stem cell transplantation after VAD induction chemotherapy for treatment of amyloid light chain amyloidosis: a single centre prospective phase II study

Therapeutic Outcome of Cyclic VAD (Vincristine, Doxorubicin and Dexamethasone) Therapy in Primary Systemic AL Amyloidosis Patients

Serum Levels of Free Light Chain before and after Chemotherapy in Primary Systemic AL Amyloidosis

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