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Systemic Amyloidosis relapse

Kathrin Schucht, Josef Schröder, Heiko Siegmund, Claudia Grafe, Stephan Schreml
A 52-year-old woman presented with a large partially yellow and erythematous tumor on her right temple. She reported that it had grown over the last 4 years. Regional lymph nodes were impalpable. A punch biopsy showed eosinophilic material in the dermis and subcutis. Immunohistochemistry showed positive staining for kappa and lambda light chains. Electron microscopy showed the typical amyloid fibrils (7-10 nm in diameter). There was no evidence of systemic amyloidosis, paraproteinemia or underlying plasmacytoma...
May 2016: Case Reports in Dermatology
Rajshekhar Chakraborty, Eli Muchtar, Morie A Gertz
The heart and the kidneys are the most commonly involved organs in systemic amyloidosis. Cardiac involvement is associated with an increased morbidity, treatment intolerance, and poorer overall survival. The most common types of amyloidosis that are associated with cardiac involvement include light chain (AL) amyloidosis and transthyretin (TTR) amyloidosis (both mutant and wild type). The traditional first-line treatment for AL amyloidosis includes alkylator-based chemotherapy or high-dose melphalan followed by autologous stem cell transplantation (ASCT)...
August 6, 2016: Current Heart Failure Reports
Sonja Haverkampf, Katja Evert, Josef Schröder, Stephan Schreml
A 79-year-old man presented with a large tumor on the left side of his head, which had grown over 5 years. Regional lymph nodes were impalpable and computed tomography revealed no signs of bone infiltration. Histology showed that the entire dermis was filled with amorphous eosinophilic material. Immunohistochemistry was negative for cytokeratin, but showed that the dermis and parts of the subcutis were filled with amyloid consisting of immunoglobulin light chains. There were no signs of paraproteinemia or underlying plasmocytoma...
January 2016: Case Reports in Dermatology
Matt Shirley
Ixazomib (Ninlaro(®)) is an orally bioavailable, reversible proteasome inhibitor developed by Millennium Pharmaceuticals, Inc. (now Takeda Oncology). Ixazomib acts by binding to and inhibiting the β5 subunit of the 20S proteasome. In November 2015, the US FDA approved ixazomib for use in combination with lenalidomide and dexamethasone for the treatment of patients with multiple myeloma who have received at least one prior therapy. Ixazomib is under regulatory review for this indication in the EU. Phase III development of ixazomib is underway worldwide for newly-diagnosed multiple myeloma (in patients who are not eligible for stem cell transplant, or as maintenance therapy) and for relapsed or refractory systemic light chain (AL) amyloidosis...
March 2016: Drugs
T Jelinek, Z Kufova, R Hajek
Immunoglobulin light chain amyloidosis (AL amyloidosis) is indeed a rare plasma cell disorder, yet the most common of the systemic amyloidoses. The choice of adequate treatment modality is complicated and depends dominantly on the risk stratification of these fragile patients. Immunomodulatory drugs (IMiDs) are currently used in newly diagnosed patients as well as in salvage therapy in relapsed/refractory patients. IMiDs have a pleiotropic effect on malignant cells and the exact mechanism of their action has been described recently...
March 2016: Critical Reviews in Oncology/hematology
Leo Čabrijan, Tatjana Kehler
Psoriasis is a chronic relapsing autoimmune disease with a multigenetic predisposition, which occurs in about 2% of patients in Croatia and shows variable occurrence in the world. Psoriasis can be associated with various diseases, including autoimmune diseases (pemphigus, pemphigoid, vitiligo), and slightly less with allergic diseases (atopic dermatitis, asthma, urticaria, allergic contact dermatitis). According to clinical manifestations, psoriasis appears as plaque psoriasis, erythrodermic form and pustular psoriasis...
March 2015: Acta Medica Croatica: C̆asopis Hravatske Akademije Medicinskih Znanosti
Kareem Jamani, Peter Duggan, Paola Neri, Nizar Bahlis, Victor H Jimenez-Zepeda
Concomitant plasma cell (PCN) and B-cell neoplasms (BCN) in a single patient have been infrequently reported. This study reviewed nine such patients at the institution - six had multiple myeloma (MM) associated with a BCN (MM/B group) and three had AL amyloidosis (ALA) with a BCN (ALA/B group). This study describes two syndromes of MM/B - three patients presented with CLL and subsequently developed MM, while three presented with MM and monoclonal B-cell lymphocytosis. In the ALA/B group, all three patients had systemic ALA and a BCN...
2016: Leukemia & Lymphoma
Maria T Cibeira, Albert Oriol, Juan J Lahuerta, Maria-Victoria Mateos, Javier de la Rubia, Miguel T Hernández, Miquel Granell, Carlos Fernández de Larrea, Jesús F San Miguel, Joan Bladé
Immunomodulatory drugs have been shown to be of benefit in relapsed/refractory immunoglobulin light-chain (AL) amyloidosis. We designed a prospective, multicentre phase II trial of lenalidomide, dexamethasone and cyclophosphamide for newly diagnosed patients with AL amyloidosis not eligible for autologous stem-cell transplantation. Twenty-eight patients were included in the study. Cardiac involvement was present in 23 patients; 14 of them had cardiac stage III. The overall haematological response rate was 46%, including complete and very good partial responses in 25% and 18% of patients respectively...
September 2015: British Journal of Haematology
Vlastimil Ščudla, Tomáš Pika, Jiří Minařík
The aim of the paper is to inform about the contribution of novel, highly sensitive analytic technique for the assessment of serum immunoglobulins (Hevylite), enabling separate quantitative assessment of heavy/light chain pairs of immunoglobulin (HLC), i. e. the monoclonal ("involved") and polyclonal ("noninvolved") isotype including their ratio (HLC-r) in monoclonal gammopathies. We particularly target the characteristics of this technique, the compari-son of its clinical contribution with standard methods used in the diagnostics, course and the detection of relapse and progression of the disease, as well as the stratification, assessment of therapeutic outcome and prognosis in monoclonal gammopathy of undetermined significance, multiple myeloma, Waldenström´s macroglobulinemia, systemic AL-amyloidosis and some non-Hodgkin lymphomas...
January 2015: Vnitr̆ní Lékar̆ství
Turker Acar, Selen Bayraktaroglu, Naim Ceylan, Recep Savas
The diseases affecting the trachea and main bronchi can be categorized into congenital or acquired abnormalities and also into focal or diffuse diseases. Major congenital bronchial abnormalities cover accessory cardiac bronchus and tracheal bronchus. Tracheobronchial strictures, such as post-intubation and post-infectious stenosis and tracheobronchial neoplasms are regarded as focal diseases, whereas tracheobronchomegaly, relapsing polychondritis, tracheobronchopathia osteochondroplastica, amyloidosis, granulomatosis with polyangiitis, tracheobronchomalacia, saber-sheath trachea and tracheobronchitis associated with ulcerative colitis are examples of diffuse diseases...
February 2015: Japanese Journal of Radiology
Massimo Offidani, Laura Corvatta, Patrizia Caraffa, Silvia Gentili, Laura Maracci, Pietro Leoni
Proteasome inhibition represents one of the more important therapeutic targets in the treatment of multiple myeloma (MM), since by suppressing nuclear factor-κB activity, which promotes myelomagenesis, it makes plasma cells susceptible to proapoptotic signals. Bortezomib, the first proteasome inhibitor approved for MM therapy, has been shown to increase response rate and improve outcome in patients with relapsed/refractory disease and in the frontline setting, particularly when combined with immunomodulatory drugs and alkylating agents...
2014: OncoTargets and Therapy
Franco Dammacco, Giuseppe Rubini, Cristina Ferrari, Angelo Vacca, Vito Racanelli
Conventional radiographic skeletal survey has been for many years the gold standard to detect the occurrence of osteolytic lesions in patients with multiple myeloma (MM). However, the introduction of more sensitive imaging procedures has resulted in an updated anatomic and functional Durie and Salmon "plus" staging system and has remarkably changed the diagnostic and prognostic approach to this tumor. It is now established that (18)fluorine-fluorodeoxyglucose ((18)F-FDG) positron-emission tomography (PET) combined with low-dose computed tomography (CT), shortly designated PET/CT, exhibits a higher screening and diagnostic sensitivity and specificity over the skeleton X-ray...
February 2015: Clinical and Experimental Medicine
Donna E Reece, Ute Hegenbart, Vaishali Sanchorawala, Giampaolo Merlini, Giovanni Palladini, Joan Bladé, Jean-Paul Fermand, Hani Hassoun, Leonard Heffner, Vishal Kukreti, Robert A Vescio, Lixia Pei, Christopher Enny, Dixie-Lee Esseltine, Helgi van de Velde, Andrew Cakana, Raymond L Comenzo
CAN2007 was a phase 1/2 study of once- and twice-weekly single-agent bortezomib in relapsed primary systemic amyloid light chain amyloidosis (AL) amyloidosis. Seventy patients were treated, including 18 and 34 patients at the maximum planned doses on the once- and twice-weekly schedules. This prespecified final analysis provides mature response and long-term outcomes data after 3-year additional follow-up since the last report. In the once-weekly 1.6 mg/m(2) and twice-weekly 1.3 mg/m(2) bortezomib groups, final hematologic response rates were 68...
October 16, 2014: Blood
Serena Pastore, Giulia Paloni, Roberta Caorsi, Luca Ronfani, Andrea Taddio, Loredana Lepore
OBJECTIVES: Cryopyrin-associated periodic syndromes (CAPS) are a group of chronic, relapsing autoinflammatory disorders which may be complicated by systemic AA amyloidosis. The aim of our study was to evaluate serum amyloid protein A (SAA) level in CAPS patients treated with Interleukin-1beta (IL-1β) antagonist and to correlate its level with treatment response. METHODS: All patients of CAPS Italian Register treated with IL-1β inhibitor were enrolled. SAA levels before starting therapy, and at last visit were evaluated...
July 2014: Clinical and Experimental Rheumatology
Shameem Mahmood, Christopher P Venner, Sajitha Sachchithanantham, Thirusha Lane, Lisa Rannigan, Darren Foard, Jenny H Pinney, Simon D J Gibbs, Carol J Whelan, Helen J Lachmann, Julian D Gillmore, Philip N Hawkins, Ashutosh D Wechalekar
The outcomes and responses to treatment remain poorly studied among patients with systemic AL amyloidosis who require further treatment following prior novel agent-based therapy. We report here treatment with lenalidomide-dexamethasone in 84 AL amyloidosis patients with relapsed/refractory clonal disease following prior treatment with thalidomide (76%) and/or bortezomib (68%). On an intention-to-treat (ITT) basis, the overall haematological response rate was 61%, including 20% complete responses. The median overall survival (OS) has not been reached; 2-year OS and progression-free survival (PFS) was 84% and 73%, respectively...
September 2014: British Journal of Haematology
S Parmar, P Kongtim, R Champlin, Y Dinh, Y Elgharably, M Wang, Q Bashir, J J Shah, N Shah, U Popat, S A Giralt, R Z Orlowski, M H Qazilbash
Optimal treatment approach continues to remain a challenge for systemic light chain amyloidosis (AL). So far, Auto-SCT is the only modality associated with long-term survival. However, failure to show survival benefit in randomized study raises questions regarding its efficacy. We present a comparative outcome analysis of Auto-SCT to conventional therapies (CTR) in AL patients treated over a 14-year period at our institution. Out of the 145 AL amyloidosis patients, Auto-SCT was performed in 80 patients with 1-year non-relapse mortality rate of 12...
August 2014: Bone Marrow Transplantation
Jayasudha Arundhathi Vasudevan, Thara Somanathan, Shridhan Anand Patil, Jayasree Kattoor
Amyloid is a pathologic proteinaceous substance deposited between cells in various tissues in a variety of clinical conditions. We report a case of amyloidosis of tongue with extensive chondroid metaplasia diagnosed on incisional biopsy in a multiple myeloma patient, who underwent autologous peripheral blood stem cell transplant for the same in 2010 and now presented with disease relapse after 2 years.
May 2013: Journal of Oral and Maxillofacial Pathology: JOMFP
Gunjan Shah, Esha Kaul, Shelly Fallo, Furha Cossor, Hedy Smith, Kellie Sprague, Andreas Klein, Kenneth Miller, Raymond Comenzo
BACKGROUND: Bortezomib is a first-in-class proteasome inhibitor approved by the US Food and Drug Administration for the treatment of all phases of multiple myeloma (MM) and it is also used for the treatment of [corrected] light-chain amyloidosis (AL). The subcutaneous formulation of bortezomib was approved in 2012 based on data from Phase III studies in patients with relapsed MM. OBJECTIVE: This article reports experience with subcutaneous bortezomib in patients with newly diagnosed MM or AL in a tertiary care center...
October 2013: Clinical Therapeutics
Flora Magnotti, Antonio Vitale, Donato Rigante, Orso Maria Lucherini, Rolando Cimaz, Isabella Muscari, Atila Granados Afonso de Faria, Bruno Frediani, Mauro Galeazzi, Luca Cantarini
Tumour necrosis factor-receptor associated periodic syndrome (TRAPS) is a rare autosomal dominant autoinflammatory disorder characterised by recurrent episodes of long-lasting fever and inflammation in different regions of the body, as musculo-skeletal system, skin, gastrointestinal tube, serosal membranes and eye. Inflammatory attacks usually start in the pediatric age with initial corticosteroid-responsiveness. Most reported cases of TRAPS involve patients of European ancestry and diagnosis can be formulated by the combination of genetic analysis and a compatible phenotype...
May 2013: Clinical and Experimental Rheumatology
Ardeshir Ghavamzadeh, Kamran Alimoghaddam, Fatemeh Ghaffari, Roshanak Derakhshandeh, Arash Jalali, Mohammad Jahani
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is a new window to therapy of many diseases. From March 1991 through April 2011, a total of 3237 HSCT were performed in the Hematology-Oncology and Stem Cell Transplantation Research Center, affiliated to Tehran University of Medical Sciences. Here we report 20 years experience of HSCT. OBJECTIVES: Our strategy and aim include the protraction of cytogenetic and molecular biological diagnostic tests, the expansion of the first Iranian Cord Blood Bank (ICBB) and development of the first Iranian Stem Cell Donor Program (ISCDP), and improvement the researches in new therapeutic fields...
February 2013: Iranian Red Crescent Medical Journal
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