Therapeutic Outcome of Lenalidomide-dexamethasone in Patients with Relapsed or Refractory Systemic Immunoglobulin Light Chain (AL) Amyloidosis : A Single-center Analysis and Review of the Literature

Article信州医学雑誌 70(1) : 29-38(2022)departmental bulletin pape

Marked shrinkage of amyloid lymphadenopathy after an intensive chemotherapy in a patient with IgM-associated AL amyloidosis

This is an electronic version of an article published in Amyloid 2009, Vol. 16, No. 3 : Pages 183-185. Amyloid is available online at: http://informahealthcare.com/doi/pdf/10.1080/13506120903151825A male patient with primary AL amyloidosis who had been suffering from systemic lymphadenopathy with IgM kappa-type M-proteinemia received two courses of VAD and high-dose melphalan with in vivo elimination of CD20(+) cells using rituximab followed by autologous peripheral blood stem cell transplantation. Four years after complete hematological remission he showed marked reduction in size of the amyloid-laden lymph nodes. Deposits of AL amyloid may regress from the tissue if the chemotherapy succeeds in persistent inhibition of the production of amyloidogenic immunoglobulin light chains.ArticleAMYLOID. 16(3):183-185 (2009)journal articl

Rituximab therapy in nephrotic syndrome due to AH amyloidosis

This is an electronic version of an article published in Amyloid 2009, Vol. 16, No. 3 : Pages 178-180. Amyloid is available online at: http://informahealthcare.com/doi/pdf/10.1080/13506120903090940We report a patient with AH amyloidosis associated with lymphoplasmacytic leukemia that has remained in a stable state with a nephrotic syndrome for 17 months since the commencement of cyclic rituximab therapy aimed at suppression of pathogenetic gamma heavy chains. Free light chains in serum and CD20-positive cells in peripheral blood were useful as hematological markers in the patient. Rituximab might be a potent therapeutic option for AH amyloidosis associated with a B-cell lymphoproliferative disorder.ArticleAMYLOID. 16(3):178-180 (2009)journal articl

Surgical removal of amyloid-laden lymph nodes: a possible therapeutic approach in a primary systemic AL amyloidosis patient with focal lymphadenopathy

We report a patient with primary systemic AL amyloidosis who suffered from remarkable bilateral cervical lymphadenopathy. Intensive chemotherapies, including two cycles of high-dose melphalan with autologous peripheral blood stem cell transplantation, were insufficiently effective for both the lymphadenopathy and amyloidogenic IgG lambda lambda-type M-protein in serum, but the patient showed complete haematological remission after extensive surgical removal of enlarged lymph nodes that had massive depositions of lambda lambda-type immunoglobulin light chain-derived amyloid. Lymphadenectomy may be a possible therapeutic approach with regard to both cosmetic and haematological aspects in primary systemic AL amyloidosis patients with focal lymphadenopathy.ArticleAMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS. 18(2):79-82 (2011)journal articl

Coadministration of Cyclosporin A with Prednisolone in Acute Interstitial Pneumonia Complicating Polymyositis/Dermatomyositis

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Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve

Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various tissues. Localized amyloidosis has different characteristics than those of systemic amyloidosis. In this paper, we present the case of a middle-aged woman who presented with worsening ulnar nerve palsy. Electrophysiological examination and MRI indicated a tumor surrounding the ulnar nerve in the forearm. However, the operative findings revealed that ulnar nerve fascicles were replaced with a yellow tissue, which was diagnosed as amyloid light-chain λ amyloidosis, based on histopathological examination. Systemic amyloidosis was ruled out after the screening examinations. This paper is the first report of the ulnar nerve as the sole site of localized immunoglobulin light-chain amyloidosis manifestation

Surgical removal of amyloid-laden lymph nodes: a possible therapeutic approach in a primary systemic AL amyloidosis patient with focal lymphadenopathy

We report a patient with primary systemic AL amyloidosis who suffered from remarkable bilateral cervical lymphadenopathy. Intensive chemotherapies, including two cycles of high-dose melphalan with autologous peripheral blood stem cell transplantation, were insufficiently effective for both the lymphadenopathy and amyloidogenic IgG lambda lambda-type M-protein in serum, but the patient showed complete haematological remission after extensive surgical removal of enlarged lymph nodes that had massive depositions of lambda lambda-type immunoglobulin light chain-derived amyloid. Lymphadenectomy may be a possible therapeutic approach with regard to both cosmetic and haematological aspects in primary systemic AL amyloidosis patients with focal lymphadenopathy.ArticleAMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS. 18(2):79-82 (2011)journal articl

Successful Treatment with Rituximab in a Patient with Stiff-Person Syndrome Complicated by Dysthyroid Ophthalmopathy

We report a patient with stiff-person syndrome and insulin-dependent diabetes mellitus with anti-glutamic acid decarboxylase (GAD) antibody, who suddenly complained of diplopia due to dysthyroid ophthalmopathy. Therapeutic efficacy of plasmapheresis and high-dose intravenous immunoglobulin was transient. After starting administration of rituximab, the patient showed obvious improvement of muscle spasms due to stiff-person syndrome and ophthalmoplegia following quick depletion of CD20-positive cells in peripheral blood. The anti-GAD and anti-thyroid antibodies decreased slowly. This drug might be a potent therapeutic option for refractory patients with stiff-person syndrome, particularly in those associated with dysthyroid ophthalmopathy.ArticleINTERNAL MEDICINE. 49(3):237-241 (2010)journal articl

Takayasu's Arteritis in a Patient with Crohn's Disease: An Unexpected Association during Infliximab Therapy

We report a 20-year-old woman with Crohn's disease (CD) who developed anterior neck pain while being treated with the anti-tumor necrosis factor (TNF)-alpha monoclonal antibody, infliximab. She showed no symptoms suggestive of active CD except for tenderness along the left common carotid artery with marked increases in serum TNF-alpha and inflammatory reactions. Based on thickened walls of large vessels with enhancement effects on computed tomography, she was diagnosed as having associated Takayasu's arteritis (TA), which was successfully treated with corticosteroid. Even if CD is controlled by infliximab, other autoimmune disorders, such as TA, may develop as a complication.ArticleINTERNAL MEDICINE. 49(2):179-182 (2010)journal articl

Thermal Stability Threshold for Amyloid Formation in Light Chain Amyloidosis

Light chain (AL) amyloidosis is a devastating disease characterized by amyloid deposits formed by immunoglobulin light chains. Current available treatments involve conventional chemotherapy and autologous stem cell transplant. We have recently concluded a phase III trial comparing these two treatments. AL amyloidosis patients who achieve hematological complete response (CR) do not necessarily achieve organ response regardless of the treatment they received. In order to investigate the possible correlation between amyloid formation kinetics and organ response, we selected AL amyloidosis patients from the trial with kidney involvement and CR after treatment. Six patients were selected and their monoclonal immunoglobulin light chains were characterized. The proteins showed differences in their stability and their kinetics of amyloid formation. A correlation was detected at pH 7.4, showing that less stable proteins are more likely to form amyloid fibrils. AL-T03 is too unstable to form amyloid fibrils at pH 7.4. This protein was found in the only patient in the study that had organ response, suggesting that partially folded species are required for amyloid formation to occur in AL amyloidosis