A single-centre cohort of patients with systemic light chain AL-amyloidosis treated with conventional chemotherapy or with high-dose chemotherapy and autologous stem cell transplantation

Raschle, Joëlle; Banz, Yara; Suter, Thomas M.; Pabst, Thomas

doi:10.4414/smw.2014.13922

Cited by 4 publications

(6 citation statements)

References 29 publications

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“…Because plasma cells are radiosensitive, low-dose radiation therapy remains the mainstay of treatment for tracheobronchial amyloidosis [17]. High-dose chemotherapy combined with autologous stem cell transplantation confers better prognosis in systemic light chain amyloidosis lesions [18]. Immunosuppressive agents and radiotherapy are ineffective in laryngeal amyloidosis [19].…”

Section: Resultsmentioning

confidence: 99%

Primary Laryngo-Tracheobronchial Amyloidosis: A Case Report and Literature Review

Jiao¹,

Liu²,

Liu³

et al. 2017

J Clin Case Rep

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Primary laryngo-tracheobronchial amyloidosis is a rare pulmonary disease for which there is currently no established treatment. Herein, we report a case involving a 51-year-old woman who was admitted to hospital primarily due to intermittent hoarseness. Botryoidal masses were visible at the entrance of the throat. On chest computed tomography, eccentric soft tissue density shadows were apparent in the tracheal bifurcation of the proximal segment of the trachea, and the lumen was narrowed. Electronic bronchoscopy revealed extensive new biological invasive growth from the nasopharynx to the glottis. The glottis and larynx were irregularly shaped with clear characteristics and congestion. New biological growth was also observed in the right upper lobe, right middle bronchus, and right lower lobe mucosa. The patient was ultimately diagnosed with primary laryngo-tracheobronchial amyloidosis via pathological analysis. Although amyloidosis is a benign lesion, to date, there are no curative treatments. The present article briefly highlights the pertinent literature, and discusses the clinical manifestations and progress of treatment in primary laryngo-tracheobronchial amyloidosis.

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Section: Resultsmentioning

confidence: 99%

Primary Laryngo-Tracheobronchial Amyloidosis: A Case Report and Literature Review

Jiao¹,

Liu²,

Liu³

et al. 2017

J Clin Case Rep

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“…With the availability and documented safety and efficacy of IMiDs and PIs in AL, [3][4][5][6][7], they have gradually replaced CC [8,9]. High-dose melphalan followed by autologous hematopoietic stem cell transplantation (auto-HCT) has been associated with longer survival in selected patients with AL when compared with CC agents [10,11]. It is considered an effective treatment strategy that has been associated with hematologic response (HR) and an improvement in organ function [12], quality of life, and survival [13].…”

Section: Introductionmentioning

confidence: 99%

“…A high treatment-related mortality rate (24%) was seen in the highdose melphalan group, which is more than twice the rate at centers that regularly perform auto-HCT for AL. That study was also notable for a suboptimal dose of melphalan in 10 of 37 patients (27%), the inability of 26% of patients in the HCT group to proceed to transplant because of early mortality, and inclusion of transplant centers that perform 1 or fewer auto-HCT for AL per year [10,12,13,15]. Despite evident clinical benefit, auto-HCT is associated with a significant nonrelapse mortality (NRM) [13,[16][17][18], and it should be used at specialized centers in a carefully selected patient population.…”

Section: Introductionmentioning

confidence: 99%

Impact of Induction Therapy on the Outcome of Immunoglobulin Light Chain Amyloidosis after Autologous Hematopoietic Stem Cell Transplantation

Afrough

Saliba

Hamdi

et al. 2018

Biology of Blood and Marrow Transplantation

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With the availability of immunomodulatory imide drugs (IMiDs) and proteasome inhibitors (PI), most patients with immunoglobulin light chain amyloidosis (AL) receive induction therapy before autologous hematopoietic stem cell transplantation (auto-HCT). In this study we evaluated the type of induction therapy and its impact on the outcome of auto-HCT in AL. We identified 128 patients with AL who underwent high-dose chemotherapy and auto-HCT at our institution between 1997 and 2013. Patients were divided into 3 groups: no induction, conventional chemotherapy (CC)-based induction (melphalan, steroids), and IMiD/PI-based induction (thalidomide, lenalidomide, or bortezomib). The hematologic response (HR) and organ response were defined according to the established criteria. Median age at auto-HCT was 58 years (range, 35 to 75). Twenty patients (15.5%) received no induction, 25 (19.5%) received CC, and 83 (65%) received IMiDs/PIs. One, 2, or 3 or more organs were involved in 90 (70%), 20 (16%), and 18 (14%) patients, respectively. After auto-HCT 12 of 20 (60%), 15 of 24 (62%), and 72 of 83 (87%) assessable patients achieved HR at 100 days in no induction, CC, and IMiD/PI groups, respectively (P = .001). Organ response at 1 year after auto-HCT was seen in 7 of 18 (39%), 14 of 24 (58%), and 37 of 79 (47%) assessable patients in no induction, CC, and IMiD/PI groups, respectively (P = .3). Achieving a hematologic complete response was associated with a significantly higher probability of achieving an organ response (P = .02). After a median follow-up of 26 months, rates of 2-year progression-free survival were 67%, 56%, and 73% in no induction, CC, and IMiD/PI groups, respectively (P = .07; hazard ratio, .5; 95% confidence interval [CI], .3 to 1.1). Rates of 2-year overall survival were 73%, 76%, and 87% in no induction, CC, and IMiD/PI groups, respectively (P = .05; hazard ratio, .4; 95% CI, .2 to .9). On multivariate analysis a low β-microglobulin (P = .01; hazard ratio, .3; 95% CI, .1 to .7) and induction therapy with IMiD/PI (P = .01; hazard ratio, .3; 95% CI, .1 to .7) were associated with a better overall survival. Induction therapy with either CC or IMiDs/PIs is safe and feasible in selected patients with AL. IMiD/PI-based induction is associated with a longer overall survival compared with patients who received no induction or CC before auto-HCT.

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“…[2] Compared with conventional chemotherapy, the introduction of high dose melphalan (HDM) followed by autologous hematopoietic stem cell transplantation (ASCT) has improved the outcome of AL amyloidosis. [3,4] The psoriatic skin lesion is an inflammatory reaction initiated by infiltrating of T cells and neutrophils in epidermis and dermis. [5] T cells play an important role in the pathogenesis of psoriasis, bone marrow transplantation may alter the course of the disease.…”

Section: Introductionmentioning

confidence: 99%

Complete remission of both immunoglobulin light chain amyloidosis and psoriasis after autologous hematopoietic stem cell transplantation

et al. 2018

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Rationale:Immunoglobulin light chain amyloidosis (AL amyloidosis) is characterized by the deposition of abnormal amyloid protein produced by a pathological plasma cell clone in various organs and soft tissues. Hematopoietic stem cell transplantation (HSCT) is an effective way to treat AL amyloidosis. Psoriasis is a common autoimmune disease (AID) and HSCT is a potential treatment for severe AIDs. We report a rare case of AL amyloidosis coincidence with psoriasis obtained continuous complete remission of the 2 diseases by autologous hematopoietic stem cell transplantation (ASCT).Patient concerns:A 58-year-old man with a 30-year history of psoriasis complaining of edema and hypotension for 2 weeks was referred to our institution. His urine protein was quantified 2.83 g/day, without hematuria and decrease of glomerular filtration rate.Diagnosis:Renal biopsy confirmed AL amyloidosis and multiple myeloma was excluded by bone marrow cytomorphologic examination.Interventions:Chemotherapy regimen based on bortezomib and thalidomide had achieved hematologic partial remission, but the kidney had no response and psoriasis was still active. Furthermore, he received a standard myeloablative conditioning with high dose melphalan followed by ASCT.Outcomes:The erythema with slivery scales of psoriasis vulgaris gradually improved and almost disappeared after granulocyte implantation. He obtained persistent hematological complete remission, organ response and recovery of psoriasis.Lessons:We report a rare case of AL amyloidosis coincidence with psoriasis treated by ASCT. The outcome of this patient indicated that ASCT has therapeutic values both in AL amyloidosis and AIDs.

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A single-centre cohort of patients with systemic light chain AL-amyloidosis treated with conventional chemotherapy or with high-dose chemotherapy and autologous stem cell transplantation

Cited by 4 publications

References 29 publications

Primary Laryngo-Tracheobronchial Amyloidosis: A Case Report and Literature Review

Primary Laryngo-Tracheobronchial Amyloidosis: A Case Report and Literature Review

Impact of Induction Therapy on the Outcome of Immunoglobulin Light Chain Amyloidosis after Autologous Hematopoietic Stem Cell Transplantation

Complete remission of both immunoglobulin light chain amyloidosis and psoriasis after autologous hematopoietic stem cell transplantation

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