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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/27647123/proteasome-inhibitors-in-al-amyloidosis-focus-on-mechanism-of-action-and-clinical-activity
#4
T Jelinek, E Kryukova, Z Kufova, F Kryukov, R Hajek
Proteasome inhibitors are the backbone in the treatment of multiple myeloma with 3 of its representatives (bortezomib, carfilzomib, and ixazomib) having already been approved. There is a different situation altogether in the treatment of amyloid light chain (AL) amyloidosis where owing to the rarity of this entity neither of these drugs has currently gained approval. Amyloid light chain plasma cells are possibly more vulnerable to bortezomib than myeloma plasmocytes because of a slightly distinct mechanism of action, which is described in depth in this manuscript...
September 20, 2016: Hematological Oncology
https://www.readbyqxmd.com/read/27580556/aa-negative-and-kappa-positive-amyloidosis-in-a-patient-with-rheumatoid-arthritis
#5
Toshiharu Ueno, Keiichi Sumida, Junichi Hoshino, Tatsuya Suwabe, Koki Mise, Ryo Hazue, Noriko Hayami, Rikako Hiramatsu, Masahiro Kawada, Aya Imafuku, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Keiichi Kinowaki, Kenichi Ohashi, Takeshi Fujii, Aya Nishida, Yoshifumi Ubara
A 57-year-old Japanese woman with a 5-year history of rheumatoid arthritis (RA) was admitted to our hospital for an evaluation of nephrotic range proteinuria (4.8 g/day). A renal biopsy led to the diagnosis of amyloidosis according to strong positivity for Congo red staining and the detection of microfibrillar structures on electron microscopy that were negative for AA and positive for kappa light chain. Combination therapy with high-dose melphalan and autologous stem cell transplantation was performed according to the regimen for AL amyloidosis...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27504090/nodular-cutaneous-amyloidosis-at-the-temple
#6
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
#7
REVIEW
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/27296954/the-effect-of-bone-marrow-plasma-cell-burden-on-survival-in-patients-with-light-chain-amyloidosis-undergoing-high-dose-melphalan-and-autologous-stem-cell-transplantation
#8
Christopher Dittus, Nsabimana Uwumugambi, Fangui Sun, J Mark Sloan, Vaishali Sanchorawala
The prognosis in light chain (AL) amyloidosis has been linked to several variables, which are primarily related to end-organ damage. Recently, bone marrow plasma cell (BMPC) burden >10% has also been described as an adverse prognostic factor. We reviewed data pertaining to 546 patients with AL amyloidosis who underwent high-dose melphalan (HDM) and stem cell transplantation (SCT) to determine if BMPC > 10% was a negative prognostic factor. Of these patients, 445 had a BMPC burden ≤ 10% and 101 had a BMPC burden > 10%...
September 2016: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/27293389/nodular-cutaneous-amyloidosis-resembling-a-giant-tumor
#9
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/27155897/-pulmonary-malt-lymphoma-and-paraneoplastic-syndromes
#10
E Monge, N Coolen-Allou, P Mascarel, V Gazaille
INTRODUCTION: Primary pulmonary lymphoma is a rare disease; diagnosis is often delayed because of atypical clinical presentation and slow progression. OBSERVATION: A 42-year-old woman consulted because of haemoptysis. Chest CT-scan showed multiple nodular calcified masses. A lung biopsy led to the diagnosis of pulmonary amyloidosis with pulmonary MALT lymphoma (mucosa-associated lymphoid tissue). The patient developed two paraneoplastic syndromes: a hypertrophic osteoarthropathy and mucinosis...
May 4, 2016: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/27101985/bortezomib-containing-regimens-for-the-treatment-of-newly-diagnosed-and-relapsed-amyloid-light-chain-amyloidosis-a-single-center-experience
#11
Victor H Jimenez-Zepeda, Peter Duggan, Paola Neri, Nizar J Bahlis
BACKGROUND: The proteasome is an exciting target for the development of novel anticancer therapies. Recent evidence has suggested that bortezomib, a dipeptide boronate proteasome inhibitor, exhibits unprecedented single-agent activity in amyloid light chain (AL) amyloidosis. PATIENTS AND METHODS: We performed a retrospective review of the use of bortezomib-containing regimens to assess the rapidity and quality of response at our institution. RESULTS: A total of 52 patients with documented newly diagnosed and relapsed AL amyloidosis treated with bortezomib-containing regimens were identified from our institutional database...
June 2016: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/27086816/tracheobronchial-amyloidosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#12
Takeshi Saraya, Hiroki Nunokawa, Masachika Fujiwara, Kosuke Ohkuma, Naoki Tsujimoto, Yayoi Tsukahara, Haruyuki Ishii, Hajime Goto, Hajime Takizawa
A 65-year-old woman was referred to our respiratory department because of incidentally detected endobronchial deposits. She had been diagnosed with Sjögren's syndrome 12 years earlier. Bronchoscopy showed protrusion of the reddened, shiny or edematous mucosa at the orifice of the lower lobe bronchus, suggesting a submucosal tumor. Based on the pathological findings of the transbronchial biopsied specimens, the patient was diagnosed with non-classified type tracheobronchial amyloidosis associated with Sjögren's syndrome, which was negative for both λ and κ chains, transthyretin and amyloid A...
2016: Internal Medicine
https://www.readbyqxmd.com/read/26846321/ixazomib-first-global-approval
#13
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
#14
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
#15
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/26371138/improved-outcomes-after-autologous-hematopoietic-cell-transplantation-for-light-chain-amyloidosis-a-center-for-international-blood-and-marrow-transplant-research-study
#16
Anita D'Souza, Angela Dispenzieri, Baldeep Wirk, Mei-Jie Zhang, Jiaxing Huang, Morie A Gertz, Robert A Kyle, Shaji Kumar, Raymond L Comenzo, Robert Peter Gale, Hillard M Lazarus, Bipin N Savani, Robert F Cornell, Brendan M Weiss, Dan T Vogl, César O Freytes, Emma C Scott, Heather J Landau, Jan S Moreb, Luciano J Costa, Muthalagu Ramanathan, Natalie S Callander, Rammurti T Kamble, Richard F Olsson, Siddhartha Ganguly, Taiga Nishihori, Tamila L Kindwall-Keller, William A Wood, Tomer M Mark, Parameswaran Hari
PURPOSE: Autologous hematopoietic cell transplantation, or autotransplantation, is effective in light-chain amyloidosis (AL), but it is associated with a high risk of early mortality (EM). In a multicenter randomized comparison against oral chemotherapy, autotransplantation was associated with 24% EM. We analyzed trends in outcomes after autologous hematopoietic cell transplantation for AL in North America. PATIENTS AND METHODS: Between 1995 and 2012, 1,536 patients with AL who underwent autotransplantation at 134 centers were identified in the Center for International Blood and Marrow Transplant Research database...
November 10, 2015: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/26065437/co-existent-b-cell-and-plasma-cell-neoplasms-a-case-series-providing-novel-clinical-insight
#17
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
#18
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/25870085/autologous-stem-cell-transplantation-without-hematopoietic-support-for-the-treatment-of-hematologic-malignancies-in-jehovah-s-witnesses
#19
Patricia A Ford, Shakira J Grant, Rosemarie Mick, Gina Keck
PURPOSE: Autologous stem-cell transplantation (ASCT) has shown to provide curative benefit in patients with relapsed lymphoma and multiple myeloma (MM), often requiring hematopoietic support until marrow engraftment. Because of Jehovah's Witnesses' (JW) refusal of blood products, treatment challenges arise. This study represents 125 JWs with lymphoma (n = 55), MM (n = 68), or amyloidosis (n = 2), treated with high-dose chemotherapy (HDC) and ASCT without transfusions. PATIENTS AND METHODS: Priming with intravenous iron and erythropoietin occurred to increase hemoglobin (Hb) pretransplantation...
May 20, 2015: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/25730192/high-dose-therapy-with-auto-sct-is-feasible-in-high-risk-cardiac-amyloidosis
#20
P Kongtim, M H Qazilbash, J J Shah, A Hamdi, N Shah, Q Bashir, M Wang, R Champlin, E E Manasanch, D Weber, R Z Orlowski, S Parmar
Cardiac involvement in light-chain amyloidosis (AL) predicts poor prognosis and is associated with higher TRM and morbidity during high-dose therapy and auto-SCT (HDT-ASCT). We studied the outcomes of 30 patients with cardiac amyloidosis undergoing HDT-ASCT at our center between January 1998 and March 2012. The median age of the patients was 53 years (range, 36-74) with a median follow-up of 35 months (range, 0.4-97 months). Twenty-seven patients (90%) had more than one organ involved besides the heart with 37% with cardiac stage ⩾3...
May 2015: Bone Marrow Transplantation
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