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Systemic Amyloidosis relapse

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https://www.readbyqxmd.com/read/27862787/two-diseases-one-remedy-systemic-amyloidosis-secondary-to-hidradenitis-suppurativa-treatment-with-infliximab
#1
İlkay Özer, Cengiz Karaçin, Esra Adışen, Galip Güz, Mehmet Ali Gürer
Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Herein, we present such a patient that had developed AA amyloidosis during the course of hidradenitis suppurativa...
November 12, 2016: Dermatologic Therapy
https://www.readbyqxmd.com/read/27804150/progressive-refractory-light-chain-amyloidosis-and-multiple-myeloma-patients-are-responsive-to-the-addition-of-clarithromycin-to-imid-based-therapy
#2
Adir Shaulov, Chezi Ganzel, Noam Benyamini, Yossef Barshay, Neta Goldschmidt, David Lavie, Diana Libster, Alex Gural, Batia Avni, Moshe E Gatt
Multiple myeloma (MM) and primary systemic light chain amyloidosis (AL) are both chronic plasma cell dyscrasias with different clinical expression but limited treatment options for relapsed refractory disease. We report the effect of the addition of clarithromycin on 31 MM and 17 AL with relapsed or refractory disease who had an insufficient response or disease progression while on an IMiD based therapy. In this high risk population, hematological response was reported in 48% of MM patients and 94% of AL patients...
November 2, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27780577/bendamustine-induced-nephrogenic-diabetes-insipidus-in%C3%A2-a%C3%A2-patient-with-al-amyloidosis
#3
Nsabimana A Uwumugambi, Vaishali Sanchorawala, Anthony C Shelton, Lauren Stern, Craig E Gordon
Nephrogenic diabetes insipidus is a condition characterized by polyuria with dilute urine due to the inability of the principal cells of the renal collecting ducts to respond to antidiuretic hormone and concentrate urine. Nephrogenic diabetes insipidus can be drug induced, and several chemotherapeutic agents have been reported to cause it. Bendamustine is a traditional chemotherapeutic agent being studied for treatment for relapsed systemic AL amyloidosis. We report a case of a 59-year-old man with AL amyloidosis who developed partial nephrogenic diabetes insipidus after receiving bendamustine for treatment of AL amyloidosis...
October 22, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27504090/nodular-cutaneous-amyloidosis-at-the-temple
#4
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
https://www.readbyqxmd.com/read/27497715/newer-therapies-for-amyloid-cardiomyopathy
#5
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)...
October 2016: Current Heart Failure Reports
https://www.readbyqxmd.com/read/27293389/nodular-cutaneous-amyloidosis-resembling-a-giant-tumor
#6
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
https://www.readbyqxmd.com/read/26846321/ixazomib-first-global-approval
#7
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
https://www.readbyqxmd.com/read/26806146/immunomodulatory-drugs-in-al-amyloidosis
#8
REVIEW
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
https://www.readbyqxmd.com/read/26606786/-association-of-psoriasis-with-other-diseases
#9
REVIEW
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
https://www.readbyqxmd.com/read/26065437/co-existent-b-cell-and-plasma-cell-neoplasms-a-case-series-providing-novel-clinical-insight
#10
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
https://www.readbyqxmd.com/read/25974382/a-phase-ii-trial-of-lenalidomide-dexamethasone-and-cyclophosphamide-for-newly-diagnosed-patients-with-systemic-immunoglobulin-light-chain-amyloidosis
#11
MULTICENTER STUDY
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
https://www.readbyqxmd.com/read/25693618/-the-role-of-the-assessment-of-heavy-light-chain-pairs-of-immunoglobulin-in-monoclonal-gammopathies
#12
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
https://www.readbyqxmd.com/read/25504056/computed-tomography-findings-of-tracheobronchial-system-diseases-a-pictorial-essay
#13
REVIEW
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
https://www.readbyqxmd.com/read/25302026/an-evidence-based-review-of-ixazomib-citrate-and-its-potential-in-the-treatment-of-newly-diagnosed-multiple-myeloma
#14
REVIEW
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
https://www.readbyqxmd.com/read/25218739/%C3%A2-%C3%A2-f-fdg-pet-ct-a-review-of-diagnostic-and-prognostic-features-in-multiple-myeloma-and-related-disorders
#15
REVIEW
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
https://www.readbyqxmd.com/read/25202139/long-term-follow-up-from-a-phase-1-2-study-of-single-agent-bortezomib-in-relapsed-systemic-al-amyloidosis
#16
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
https://www.readbyqxmd.com/read/25069027/serum-amyloid-protein-a-concentration-in-cryopyrin-associated-periodic-syndromes-patients-treated-with-interleukin-1-beta-antagonist
#17
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
https://www.readbyqxmd.com/read/24930361/lenalidomide-and-dexamethasone-for-systemic-al-amyloidosis-following-prior-treatment-with-thalidomide-or-bortezomib-regimens
#18
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
https://www.readbyqxmd.com/read/24887378/auto-sct-improves-survival-in-systemic-light-chain-amyloidosis-a-retrospective-analysis-with-14-year-follow-up
#19
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
https://www.readbyqxmd.com/read/24250091/primary-systemic-amyloidosis-of-tongue-with-chondroid-metaplasia
#20
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
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