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

Tazawa, Ko-ichi; Matsuda, Masayuki; Yoshida, Takuhiro; Gono, Takahisa; Katoh, Nagaaki; Shimojima, Yasuhiro; Ishii, Wataru; Fushimi, Tomohisa; Koyama, Jun; Ikeda, Shu‐ichi

doi:10.2169/internalmedicine.47.0949

Cited by 7 publications

(3 citation statements)

References 16 publications

(9 reference statements)

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“…Although the mechanisms by which VAD ameliorate the infiltrative cardiomyopathy are still unclear, a recent report suggests that the reduction of amyloid depositions resulting from decreased precursor protein may be the mechanism by which VAD exert their therapeutic effects [8]. The cardiac function of this patient remained unchanged after administration of VAD chemotherapy; however, a similar pathogenic mechanism may be involved in the development of refractory pleural effusion without cardiac decompensation.…”

Section: Discussionmentioning

confidence: 88%

Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report

et al. 2010

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IntroductionRefractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation is rarely reported and has a poor prognosis in general (a median survival of 1.6 months). Moreover, the optimum treatment for this condition is still undecided. This is the first report on the successful use of vincristine, adriamycin and dexamethasone chemotherapy for refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis without cardiac decompensation.Case presentationWe report the case of a 68-year old Japanese male with systemic immunoglobulin light chain amyloidosis presenting with bilateral pleural effusion (more severe on the right side) in the absence of cardiac decompensation that was refractory to diuretic therapy. The patient was admitted for fatigue, exertional dyspnea, and bilateral lower extremity edema. He had been receiving intermittent melphalan and prednisone chemotherapy for seven years. One month before admission, his dyspnea had got worse, and his chest radiograph showed bilateral pleural effusion; the pleural effusion was ascertained to be a transudate. The conventionally used therapeutic measures, including diuretics and thoracocentesis, failed to control pleural effusion. Administration of vincristine, adriamycin, and dexamethasone chemotherapy led to successful resolution of the effusion.ConclusionTreatment with vincristine, adriamycin, and dexamethasone chemotherapy was effective for the refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation and appears to be associated with improvement in our patient's prognosis.

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Section: Discussionmentioning

confidence: 88%

Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report

et al. 2010

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“…Four fulfi lled criteria for an objective hematologic response [46]. Vincristine use is contraindicated in patients with amyloid peripheral neuropathy, and doxorubicin is a poor choice for those with amyloid cardiomyopathy, which has a high prevalence.…”

Section: Dexamethasone-based Regimensmentioning

confidence: 99%

Treatment of immunoglobulin light chain amyloidosis

Gertz

Zeldenrust

2009

Curr Hematol Malig Rep

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No therapy is uniformly effective in the management of immunoglobulin light chain amyloidosis (AL amyloidosis). Despite the common generalization, therapy is highly effective. Options available to patients with AL amyloidosis include high-dose therapy, but this is applicable to only about one fourth of patients. Therapies shown to be effective are based on alkylators, dexamethasone, or combinations of an alkylator and steroids. In the past 5 years, novel agents previously shown to be effective in multiple myeloma (eg, thalidomide, lenalidomide, and bortezomib) have been shown to have efficacy in the management of AL amyloidosis. Predictors of outcome include the serum brain natriuretic peptide, the number of organs involved, and the severity of cardiac involvement detected by echocardiography. Virtually all patients are candidates for a trial of therapy, and it is possible to find a nontoxic regimen that can be administered to virtually any patient.

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“…Chronic liver injury due to amyloidosis can be successfully treated with cytotoxic substances [11,12]. A chemotherapy regimen similar to the treatment given in multiple myeloma might be promising [13,14]. Moreover, a specific therapy dissolving the amyloid deposits already incorporated into the tissue does not exist.…”

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confidence: 99%

Spontaneous Hepatic Rupture in Amyloidosis – Report of a Case

et al. 2012

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In systemic amyloidosis spontaneous rupture of the liver is a rare complication. Here we report on a patient with unrecognized systemic amyloidosis who presented to an outside hospital with unspecific abdominal pain. Under the signs of a hemorrhagic shock, distended abdomen and intraabdominal bleeding a laparotomy was performed. Due to uncontrollable hemorrhage of the ruptured right liver lobe a packing was performed. After being transferred to the reporting institution a CT scan was performed showing a grade IV laceration of the right liver. Therefore a transarterial embolization of the right hepatic artery was carried out. The following day an infection of the abdominal cavity and the abdominal wall with gas-producing bacteria was noticed and a relaparotomy, necrectomy and repacking due to diffuse bleeding were performed. The situation deteriorated and at the second relaparotomy the following day an almost completely necrotic liver was found. The patient deceased the following day. Life-threatening spontaneous liver rupture due to systemic amyloidosis might only successfully be cured by high urgency liver transplantation as presented in the literature. However, in two published cases interventional therapy by embolization of bleeding vessels has saved patients' life.

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

Abstract: Objective Intensive chemotherapy targeting plasma cell dyscrasia has been recently employed for the treatment of primary systemic AL amyloidosis. We prospectively studied the clinical usefulness of cyclic VAD (vincristine, doxorubicin and dexamethasone)

Cited by 7 publications

References 16 publications

Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report

Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report

Treatment of immunoglobulin light chain amyloidosis

Spontaneous Hepatic Rupture in Amyloidosis – Report of a Case

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