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Plasma cell dyscrasia

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https://www.readbyqxmd.com/read/28100909/impact-of-pretransplant-induction-therapy-on-autologous-stem-cell-transplantation-for-patients-with-newly-diagnosed-poems-syndrome
#1
J Li, M-H Duan, C Wang, X-F Huang, W Zhang, X-X Cao, T-N Zhu, J-L Zhuang, B Han, H Cai, H-C Cai, D-B Zhou
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, Monoclonal gammopathy and skin changes) is a rare plasma cell dyscrasia sometimes treated with a haematopoietic cell autotransplant. We analyzed data from 138 subjects with newly-diagnosed POEMS syndrome receiving a autotransplant at our centre. Thirty-two subjects with severe end-organ dysfunction ineligible for immediate autotransplant received pretransplant therapy which made a subsequent autotransplant feasible. Pretransplant therapy resulted in vascular endothelial growth factor (VEGF) remissions in 15 (47%)...
January 19, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28061985/multiple-myeloma-epidemiology-and-survival-a-unique-malignancy
#2
REVIEW
Dickran Kazandjian
Multiple myeloma (MM), although a rare disease, is the second most common hematologic malignancy. It is found in the spectrum of plasma cell dyscrasias, which begins with monoclonal gammopathy of unknown significance (MGUS) to overt plasma cell leukemia and extramedullary myeloma. MM is associated with significant morbidity due to its end-organ destruction. It is a disease of the older population and its incidence in the African American population is twice that of the European American population. Improvements in the treatment of MM in the past couple of decades, beginning with the use of autologous stem cell transplantation followed by availability of novel treatments such as immunomodulatory drugs (ImIDs) and proteasome inhibitors (PIs) has transformed the natural history of the disease leading to longer survival times...
December 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/28004444/changing-survival-of-people-with-myeloma-and-end-stage-kidney-disease-a-cohort-study-using-anzdata-1963-2013
#3
Angela C Webster, Ashley B Irish, Patrick J Kelly
BACKGROUND: It is unclear whether recent advances in myeloma therapy have improved survival for all those with myeloma and end stage kidney disease (ESKD). METHODS: Population based registry cohort study using Australia and New Zealand Dialysis and Transplant Registry data 1963-2013. We measured survival of people with myeloma and other plasma cell dyscrasias and ESKD over time, and investigated prognostic factors for improved survival using survival analysis (results expressed as hazard ratios HR with 95% confidence intervals)...
December 21, 2016: Nephrology
https://www.readbyqxmd.com/read/28002971/new-and-developing-therapies-for-al-amyloidosis
#4
Giulia Zumbo, Omid Sadeghi-Alavijeh, Philip N Hawkins, Marianna Fontana
Systemic light-chain (AL) amyloidosis is an infiltrative disorder associated with an underlying plasma cells dyscrasia, in which monoclonal immunoglobulin light chains accumulate in an abnormal misfolded form as amyloid fibrils in the extracellular space. Symptoms and prognosis are governed by which organs are affected, and cardiac involvement is the major determinant of survival. Diagnosis requires demonstration of amyloid deposition and confirmation of the fibril protein type. Areas covered: This review will focus on the available treatments for systemic AL amyloidosis and on new drug targets and therapeutic approaches...
December 29, 2016: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/27999737/immunotherapies-targeting-cd38-in-multiple-myeloma
#5
REVIEW
Djordje Atanackovic, Mary Steinbach, Sabarinath Venniyil Radhakrishnan, Tim Luetkens
Recently, the monoclonal antibody daratumumab was approved as a single agent for the treatment of patients with relapsed/refractory Multiple Myeloma (MM). Daratumumab is an antibody targeting surface molecule CD38 on myeloma cells and the agent is already widely being used based on its good tolerability and proven efficacy. We believe, however, that the efficacy of this drug and other anti-CD38 monoclonal antibodies can be further improved by combining it with other types of immunotherapies. Furthermore, surface molecule CD38 can be used as a target for immunotherapies other than just naked monoclonal antibodies...
2016: Oncoimmunology
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#6
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27979847/concomitant-multiple-myeloma-gastric-adenocarcinoma-and-evan-s-syndrome-in-a-patient-presenting-with-anaemia
#7
Maged Al-Ammari, Soheir Adam
An association between multiple myeloma (MM) and solid tumours has been previously described.(1) Furthermore, autoimmune disorders can precede plasma cell dyscrasias, and the pathogenesis of MM maybe linked to chronic immune stimulation. (2) We describe a case of concomitant MM and gastric adenocarcinoma preceded by Evan's syndrome. A previously healthy woman presented to the emergency room with symptomatic anaemia. Her initial workup was compatible with autoimmune haemolytic anaemia and monoclonal gammopathy of undetermined significance...
December 15, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27885144/treatment-of-multiple-myeloma-in-a-heart-transplant-recipient-focus-on-the-immunomodulatory-effects-of-lenalidomide
#8
Lola Xie, Bartosz Jozwik, Phillip Weeks, L Maximilian Buja, Robert Brown, Sriram Nathan, Keshava Rajagopal, Christina Paruthi, Biswajit Kar, Pranav Loyalka, Indranee Rajapreyar
Malignancy following solid organ transplant remains a significant threat to the survival of cardiac transplant recipients. Plasma cell dyscrasias including multiple myeloma have been encountered in this population, and medication treatments traditionally used to treat these disorders demonstrate immunomodulatory effects that may have implications on the transplanted allograft. Lenalidomide is an immunomodulatory agent that has been used to treat plasma cell disorders, including light-chain amyloidosis (AL) and multiple myeloma, and represents such a class of medications in which the risks and benefits in the solid organ transplant population remain to be fully elucidated...
November 24, 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27829380/design-and-development-of-the-australian-and-new-zealand-anz-myeloma-and-related-diseases-registry
#9
Krystal Bergin, Elizabeth Moore, Zoe McQuilten, Erica Wood, Bradley Augustson, Hilary Blacklock, Joy Ho, Noemi Horvath, Tracy King, John McNeil, Peter Mollee, Hang Quach, Christopher M Reid, Brian Rosengarten, Patricia Walker, Andrew Spencer
BACKGROUND: Plasma cell dyscrasias (PCD) are a spectrum of disorders resulting from the clonal expansion of plasma cells, ranging from the pre-malignant condition monoclonal gammopathy of undetermined significance (MGUS) to multiple myeloma (MM). MM generates a significant burden of disease on the community and it is predicted that it will increase in both incidence and prevalence owing to an ageing population and longer survival secondary to new therapeutic options. Robust and comprehensive clinical data are currently lacking but are required to define current diagnostic, investigational and management patterns in Australia and New Zealand (ANZ) for comparison to both local and international guidelines for standards of care...
November 9, 2016: BMC Medical Research Methodology
https://www.readbyqxmd.com/read/27822103/the-danish-national-multiple-myeloma-registry
#10
REVIEW
Peter Gimsing, Morten O Holmström, Tobias Wirenfelt Klausen, Niels Frost Andersen, Henrik Gregersen, Robert Schou Pedersen, Torben Plesner, Per Trøllund Pedersen, Mikael Frederiksen, Ulf Frølund, Carsten Helleberg, Annette Vangsted, Peter de Nully Brown, Niels Abildgaard
AIM: The Danish National Multiple Myeloma Registry (DMMR) is a population-based clinical quality database established in January 2005. The primary aim of the database is to ensure that diagnosis and treatment of plasma cell dyscrasia are of uniform quality throughout the country. Another aim is to support research. Patients are registered with their unique Danish personal identification number, and the combined use of DMMR, other Danish National registries, and the Danish National Cancer Biobank offers a unique platform for population-based translational research...
2016: Clinical Epidemiology
https://www.readbyqxmd.com/read/27819179/plasma-cell-leukemia-update-on-biology-and-therapy
#11
Roberto Mina, Mattia D'Agostino, Chiara Cerrato, Francesca Gay, Antonio Palumbo
Plasma cell leukemia (PCL) is a rare, but very aggressive, plasma cell dyscrasia, representing a distinct clinicopathological entity as compared to multiple myeloma (MM), with peculiar biological and clinical features. A hundred times rarer than MM, the disease course is characterized by short remissions and poor survival. PCL is defined by an increased percentage (>20%) and absolute number (>2 × 10(9)/l) of plasma cells in the peripheral blood. PCL is defined as 'primary' when peripheral plasmacytosis is detected at diagnosis, 'secondary' when leukemization occurs in a patient with preexisting MM...
November 6, 2016: Leukemia & Lymphoma
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
#12
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/27784371/-application-value-of-cd138-macs-fish-in-the-genetic-diagnosis-of-plasma-cell-dyscrasia
#13
Jian-Gang Mei, Han-Qing Li, Hong-Qin Cao, Jing-Jing Shao, Yong-Ping Zhai
OBJECTIVE: To investigate the cytogenetic characteristics of the various plasma cell dyscrasia using the CD138 MACS-FISH, to elucidate the application value of MACS-FISH in the genetic diagnosis of plasma cell dyscrasia, and to explore the standardization of FISH detection for plasma cell dyscrasia. METHODS: A total of 232 patients with newly diagnosed plasma cell dyscrasia were collected, including 203 cases of MM, 24 cases of AL amyloidosis and 5 cases of MGUS, whose cytogenetic abnormalities were detected by MACS-FISH, and the differences of the positive detection rates of chromosome karyotype analysis, C-FISH and MACS-FISH in MM cytogenetic abnormality were compared...
October 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27775221/light-chain-podocytopathy-mimicking-recurrent-focal-segmental-glomerulosclerosis
#14
M A Khalighi, M P Revelo, J D Abraham, F Shihab, F Ahmed
Kidney injury related to paraproteinemia is common and typically occurs after the fourth decade of life in association with an underlying plasma cell dyscrasia or other lymphoproliferative disease. Kidney transplantation in paraprotein-related kidney disease can be successful in conjunction with treatment of the underlying hematopoietic process; however, when hematologic response to therapy is not achieved, recurrent kidney injury is frequently seen. We describe a young male patient who presented at the age of 23 years with end-stage kidney disease thought to be secondary to focal segmental glomerulosclerosis; this patient ultimately received two kidney allografts...
October 24, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27772802/the-incidence-clinical-and-prognostic-significance-of-plasma-cell-dyscrasias-with-aberrant-cd4-and-or-cd8-expression
#15
Gregory Orchard, Johan Niemann, Cyriac Abraham, Raymond Banh
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27734524/bendamustine-associated-infections-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#16
Anat Gafter-Gvili, Ronit Gurion, Pia Raanani, Ofer Shpilberg, Liat Vidal
Data in the literature are lacking regarding the infection-related adverse events of bendamustine-containing regimens. Therefore, we aimed to assess this risk. We conducted a systematic review and meta-analysis of all randomized controlled trials including bendamustine-containing regimens and those administered for any lymphoproliferative disorder or plasma cell dyscrasia compared with any other regimens. A comprehensive search was conducted until December 2015. Two reviewers appraised the quality of trials and extracted data...
October 13, 2016: Hematological Oncology
https://www.readbyqxmd.com/read/27725920/flow-cytometric-analysis-four-year-experience-in-a-tertiary-care-centre-of-pakistan
#17
Imran N Ahmad, Salman Assad, Muhammad Rahman, Haider Ghazanfar
PURPOSE:   This study summarizes a four-year experience from the analysis of hematolymphoid malignancies in Pakistani population using a database of six-colored flow cytometry. METHODS: A cross-sectional survey of 323 specimens of hematolymphoid malignancies using six-colored flow cytometry (FC) was carried out in Shifa International Hospital, Islamabad, Pakistan from June 2012 to June 2016. The criterion for specimen adequacy was that the cases have abnormal populations by FC, and the specimen age (time from biopsy to being examined by the six-color FC tube) of three days or less was to be included in the study...
September 1, 2016: Curēus
https://www.readbyqxmd.com/read/27721294/primary-plasma-cell-leukemia-a-report-of-two-cases-of-a-rare-and-aggressive-variant-of-plasma-cell-myeloma-with-the-review-of-literature
#18
Prithal Gangadhar, Zulfikar Ahmed, Muktha R Pai, I Sandhya
Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma accounting for 2-3% of all plasma cell dyscrasias characterized by the presence of circulating plasma cells. The diagnosis is based on the % (≥20%) and absolute number (≥2x10 9 /L) of plasma cells in the peripheral blood. The incidence of primary PCL (pPCL) is very rare and reported to occur in <1 in a million. It is classified as either pPCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. pPCL is a distinct clinicopathological entity with different cytogenetic and molecular findings...
October 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/27677679/immunoglobulin-heavy-light-chain-test-quantifies-clonal-disease-in-patients-with-al-amyloidosis-and-normal-serum-free-light-chain-ratio
#19
Tatiana Prokaeva, Brian Spencer, Fangui Sun, Richard M O'Hara, David C Seldin, Lawreen H Connors, Vaishali Sanchorawala
BACKGROUND: Serum and urine immunofixation electrophoreses (SIFE/UIFE) are used for clonal detection in plasma cell dyscrasias, while serum free light chain (sFLC) testing provides quantitation of clonal disease. Up to 20% of patients with light chain (AL) amyloidosis may present with normal FLC ratio (FLCr). METHODS: We assessed the diagnostic, quantitative and prognostic potential of serum heavy light chain ratio (HLCr) in 199 untreated patients at initial evaluation...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27663153/localized-axillary-milia-en-plaque-a-rare-cutaneous-case-presentation-of-systemic-amyloidosis
#20
P Dickison, V Howard, B Wylie, S D Smith
Systemic AL amyloidosis is known to be associated with plasma cell dyscrasias, including multiple myeloma. The cutaneous manifestations of systemic AL amyloidosis are varied, but typically include waxy plaques or subcutaneous nodules. We report a woman who presented with bilateral eruptions of hyperpigmented plaques in her axillae, which were diagnosed as milia en plaque. She had a history of multiple myeloma, for which she was under the care of a haematologist. This is the first documented case, to our knowledge, of an eruption in the axillae being milia en plaque...
October 2016: Clinical and Experimental Dermatology
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