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Plasma cell dyscrasias

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https://www.readbyqxmd.com/read/29035194/clinicopathologic-characteristics-of-light-chain-proximal-tubulopathy-with-light-chain-inclusions-involving-multiple-renal-cell-types%C3%A2
#1
Xiaomei Li, Feng Xu, Dandan Liang, Shaoshan Liang, Xiaodong Zhu, Mingchao Zhang, Xianghua Huang, Zhihong Liu, Caihong Zeng
Light chain proximal tubulopathy (LCPT) associated with plasma cell dyscrasias is a rare abnormality, especially cases involving multiple cell types. The aim of this study is to explore the characteristics and outcomes of these diseases. We comprehensively evaluated the clinical-pathological data, treatment, and outcomes of 6 LCPT patients with involvement of multiple cell types. In 3 cases, we found that the inclusions largely existed in tubular cells, while in 2 cases they coexisted in podocytes and tubular cells, and in 1 case they coexisted in histiocytes and tubular cells...
October 16, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28985955/paraprotein-deposits-in-the-skin
#2
REVIEW
Victoria Alegría-Landa, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
Cutaneous manifestations secondary to paraprotein deposits in the skin include a group of different disorders that although rare, may be the first clinical manifestation of the underlying hematologic dyscrasia. In this article we review the clinical manifestations and histopathologic findings of the processes that result from specific deposition of the paraprotein in different structures of the skin. Paraneoplastic processes frequently associated with hematologic malignancies will not be covered in this review...
October 3, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28969143/a-rare-case-of-primary-plasma-cell-leukaemia-with-monosomy-x
#3
Smita Surendra Masamatti, Aparna Narasimha, Archana C Shetty, C Vijaya
Primary Plasma Cell Leukaemia (pPCL) is a very rare and aggressive subtype of plasma cell dyscrasias with a very poor outcome and characterized by presence of >2x109/l circulating plasma cells. Overall incidence being 1%-2% of all malignant plasma cell diseases and 0.9% of all acute leukaemias. Patients with primary and secondary PCL have similar clinical features but differences do exist. Here we report a rare and interesting case diagnosed as primary PCL in a 44-year-old female with unusual clinical presentation and complex karyotyping...
August 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28956112/at-least-partial-hematological-response-after-first-cycle-of-treatment-predicts-organ-response-and-long-term-survival-for-patients-with-al-amyloidosis-receiving-bortezomib-based-treatment
#4
Kai-Ni Shen, Jun Feng, Xu-Fei Huang, Chun-Lan Zhang, Cong-Li Zhang, Xin-Xin Cao, Lu Zhang, Dao-Bin Zhou, Jian Li
AL amyloidosis is a rare plasma cell dyscrasia characterized by multi-organ involvement and poor prognosis. We retrospectively evaluated the organ response (OR) and long-term survival of newly diagnosed AL amyloidosis patients who received first-line bortezomib-containing induction therapy, aiming to identify the clinical indication of a 50% reduction in the difference between involved and uninvolved free light chains (dFLC) after first cycle of treatment. Among the 89 patients included, 78.7% had cardiac involvement and 42...
September 27, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28944095/intermolecular-interactions-involving-an-acidic-patch-on-immunoglobulin-variable-domain-and-the-%C3%AE-2-constant-region-mediate-crystalline-inclusion-body-formation-in-the-endoplasmic-reticulum
#5
Haruki Hasegawa, Mei Geng, Randal R Ketchem, Ling Liu, Kevin Graham, Frederick Jacobsen
Full-length immunoglobulins (Igs) are widely considered difficult to crystallize because of their large size, N-linked glycosylation, and flexible hinge region. However, numerous cases of intracellular Ig crystallization are reported in plasma cell dyscrasias. What makes some Ig clones more prone to crystallize during biosynthesis as well as the biochemical and cell biological requirements for this cryptic event are poorly understood. To investigate the underlying process of intracellular Ig crystallization we searched for model IgGs that can induce crystalline inclusions during recombinant overexpression...
2017: Cellular Logistics
https://www.readbyqxmd.com/read/28916438/analytical-sensitivity-and-diagnostic-performance-of-serum-protein-electrophoresis-on-the-hydragel-30-protein-e-%C3%AE-1-%C3%AE-2-sebia-hydrasys-system
#6
Albert K Y Tsui, Dylan Thomas, Alison Hunt, Mathew Estey, Cathy-Lou Christensen, Trefor Higgins, Irwindeep Sandhu, Karina Rodriguez-Capote
BACKGROUND: Serum protein electrophoresis (SPE) and immunofixation electrophoresis (IFE) are used in the diagnosis and monitoring of plasma cell dyscrasias. IFE is considered the most sensitive method for the detection of monoclonal proteins (M-proteins), but it is not quantitative. The goal of this study was to establish the analytical sensitivity and diagnostic performance of SPE on the Sebia Hydrasys using HYDRAGEL 30 PROTEIN(E) β1-β2. METHODOLOGY: Patient sera with a previously identified M-protein (IgG, IgA or IgM) were serially diluted with a normal serum pool and electrophoresed on the Sebia Hydrasys using HYDRAGEL 30 PROTEIN(E) β1-β2...
September 12, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28903574/whole-exome-sequencing-of-aberrant-plasma-cells-in-a-patient-with-multiple-myeloma-minimal-residual-disease
#7
M Zatopkova, J Filipová, T Jelínek, P Vojta, T Sevcikova, M Simicek, L Rihova, R Bezdekova, K Growkova, Z Kufová, J Smejkalová, M Hajdúch, L Pour, J Minárik, A Jungová, V Maisnar, F Kryukov, R Hájek
Multiple myeloma is a plasma cell dyscrasia. It is the second most common hematological malignancy which is characterized by proliferation of clonal plasma cells producing harmful monoclonal immunoglobulin. Despite treatment modalities greatly evolved during the last decade, small amount of aberrant residual cells reside in patients after therapy and can cause relapse of the disease. Characterization of the residual, resistant clones can help to reveal important therapeutic targets for application of effective and precious treatment...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28893029/poems-syndrome-with-biclonal-gammopathy-a-rare-association
#8
Dibyendu De, Sandeepan Halder, Suvro Sankha Datta
Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes (POEMS) syndrome is rare plasma cell dyscrasia with multisystem involvement. The name comes from the five characteristic features: Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The presence of biclonal M band is a rare manifestation. Here, we are describing the cases of a 60-year-old lady, presented with bilateral pedal oedema and pericardial effusion and peripheral neuropathy. She also had hepatosplenomegaly, hyperpigmented rash and hypothyroidism and hyperparathyroidism...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28886228/the-serum-heavy-light-chain-immunoassay-a-valuable-tool-for-sensitive-paraprotein-assessment-risk-and-disease-monitoring-in-monoclonal-gammopathies
#9
Christine Greil, Gabriele Ihorst, Felix Gaiser, Ulrich Salzer, Emanuel Bisse, Efstathios Kastritis, Heinz Ludwig, Ralph Wäsch, Monika Engelhardt
OBJECTIVE: The heavy/light chain (HLC)-immunoassay quantifies light chain types of each immunoglobulin class in patients with monoclonal gammopathies. METHODS: We assessed 147 consecutive patients with different forms and stages of plasma cell dyscrasias (PCD) who received standard tests (serum and urine protein electrophoresis [SPEP, UPEP], immunofixation [IFE], serum-free light chain [SFLC]), and HLC-immunoassay. Patients with multiple myeloma (MM, n = 102), smoldering MM (SMM, n = 5), monoclonal gammopathy of undetermined significance (MGUS, n = 28), and Waldenström's macroglobulinemia (WM, n = 12) were included...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28879698/undetectable-vancomycin-concentrations-utilizing-a-particle-enhanced-turbidimetric-inhibition-immunoassay-in-a-patient-with-an-elevated-igm-level
#10
April A Cooper, Kevin Cowart, Amanda Clayton, Josee Paul
BACKGROUND: A case of undetectable vancomycin concentrations with the use of a particle enhanced turbidimetric inhibition immunoassay is reported. METHODS: A 73-year-old woman with B-cell lymphoma, chronic neutropenia with myelodysplastic syndrome and elevated IgM levels displayed repeated undetectable vancomycin concentrations, despite appropriate empiric vancomycin dosing. The vancomycin concentrations were processed utilizing a particle enhanced turbidimetric inhibition immunoassay (PETINIA)...
September 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28861330/a-plasma-cell-dyscrasia-presenting-as-amyloid-cardiomyopathy-and-autonomic-dysfunction-in-a-healthy-patient
#11
Rabih Tabet, Julie Zaidan, Boutros Karam, Samer Saouma, Foad Ghavami
Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient...
June 29, 2017: Curēus
https://www.readbyqxmd.com/read/28845377/an-extremely-rare-manifestation-of-multiple-myeloma-an-immunoglobulin-d-secreting-testicular-plasmacytoma
#12
Ashish Sharma, Tina Binazir, Alexandre Sintow, Chi Chan Lee, Sameer Shaharyar, Jason Tache
Multiple myelomas (MM) of the immunoglobulin D (IgD) subtype is rare amongst plasma cell malignancies. It can present a diagnostic challenge because of the low amount of immunoglobulin in the serum. The amount of monoclonal (M)-protein is often undetectable on electrophoresis. Historically, survival in these patients was typically shorter compared to the immunoglobulin A (IgA) and immunoglobulin G (IgG) subtypes due to advanced disease upon presentation. With the advent of better diagnostic techniques, the prognosis of this disease is changing...
June 27, 2017: Curēus
https://www.readbyqxmd.com/read/28770116/cisplatin-induced-nephrotoxicity-and-hiv-associated-nephropathy-mimickers-of-myeloma-like-cast-nephropathy
#13
Muhammad Siddique Khurram, Ahmed Alrajjal, Warda Ibrar, Jacob Edens, Umer Sheikh, Ameer Hamza, Hong Qu
Myeloma cast nephropathy is an obstructing disorder of renal tubules, caused by precipitation of Bence Jones proteins. Myeloma-like cast nephropathy (MLCN) has been reported in the literature to occur in various primary renal and nonrenal diseases. We present a series of three rare cases of cast nephropathy, two of which are HIV patients, and the third patient is receiving cisplatin-based chemotherapy. However, in all three patients plasma cell dyscrasia has been ruled out. A 30-year-old male was admitted to the hospital with facial cellulitis...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28757000/characteristics-of-vitamin-b12-deficiency-in-patients-with-plasma-cell-disorders
#14
Caitlyn Braschi, John Doucette, Ajai Chari
BACKGROUND: Although increased rates of vitamin B12 deficiency have been reported in patients with plasma cell dyscrasias (PCDs), no mechanism has been identified. Excess free light chains (FLCs) could disrupt the renal proximal tubule receptors where B12 is reabsorbed. We sought to characterize the relationship between B12 deficiency and PCDs. We hypothesized that rates of B12 deficiency would be highest in patients with PCDs with high FLC burdens. METHODS: We reviewed the electronic medical records of 501 patients who met inclusion criteria (diagnosed PCD with documented serum B12 and FLC levels) to obtain clinical data recorded prior to patients' lowest B12 levels...
July 12, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28716071/primary-solitary-plasmacytoma-of-the-liver-successful-treatment-with-fractionated-stereotactic-radiotherapy-cyberknife%C3%A2-a-case-report
#15
Thomas Chalopin, Isabelle Barillot, Jean-Paul Biny, Flavie Arbion, Marie Besson, Maria Santiago-Ribeiro, Eric Piver, Olivier Herault, Emmanuel Gyan, Lotfi Benboubker
BACKGROUND: Solitary plasmacytoma of the liver is a very rare and aggressive form of plasma cell dyscrasia. To the best of our knowledge, very few cases have been reported without systemic disease. We reported a rare case of hepatic solitary plasmacytoma that successfully responded to fractionated stereotactic radiotherapy. CASE PRESENTATION: A 64-year-old white French man had monoclonal gammopathy of the immune globulin G lambda type; he developed a cholestasis and cytolysis with the discovery of a subscapular nodule...
July 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#16
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28710302/a-rare-case-of-atypical-chronic-lymphocytic-leukaemia-presenting-as-nephrotic-syndrome
#17
Martina Soldarini, Lucia Farina, Augusto Genderini, Niccolo Bolli
Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28678037/sporadic-late-onset-nemaline-myopathy-with-monoclonal-gammopathy-of-undetermined-significance
#18
Akinori Uruha, Olivier Benveniste
PURPOSE OF REVIEW: Sporadic late-onset nemaline myopathy (SLONM) with monoclonal gammopathy of undetermined significance (MGUS) is a rare subacute progressive muscle disease. The prognosis is poor due to severe respiratory insufficiency. Recently, however, autologous stem-cell transplantation following high-dose melphalan has been shown to be effective unless there is delay before the treatment. Therefore, early recognition of the disease is important. This review gives an overview of recent advances in SLONM-MGUS, which could help to understand clinical and pathological features and treatment...
October 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28664017/coexistence-of-quasi-moyamoya-disease-and-poems-syndrome-in-a-patient-with-intracranial-hemorrhage-a-case-report-and-literature-review
#19
Izumi Yamaguchi, Junichiro Satomi, Nobuaki Yamamoto, Shotaro Yoshioka, Yoshiteru Tada, Kenji Yagi, Yasuhisa Kanematsu, Shinji Nagahiro
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare paraneoplastic syndrome elicited by plasma cell dyscrasia. Its clinical manifestations are multiple and stroke is not a recognized feature. A 44-year-old woman with a 3-month history of bilateral lower extremity sensorimotor disturbance was admitted to our hospital. Examinations revealed polyneuropathy, organomegaly, hypothyroidism, monoclonal gammopathy, pelvic plasmacytoma, and elevated serum vascular endothelial growth factor (VEGF) levels...
January 2017: NMC Case Rep J
https://www.readbyqxmd.com/read/28653323/increased-fracture-risk-in-plasma-cell-dyscrasias-is-associated-with-poorer-overall-survival
#20
Graham McIlroy, Jemma Mytton, Felicity Evison, Punit Yadav, Mark T Drayson, Mark Cook, Guy Pratt, Paul Cockwell, Jennifer H Pinney
Pathological fractures are a common complication of plasma cell dyscrasias (PCD) and are associated with significant morbidity. Routine use of bisphosphonates over the past decade has aimed to reduce the risk of fractures in patients with multiple myeloma, but despite this, fractures continue to represent a significant burden of disease. In this study we report the fracture rate of hospital in-patients with PCD in England. Data from the national registry Hospital Episode Statistics between 2001 and 2015 were used to determine fracture rate and its effect on overall survival...
October 2017: British Journal of Haematology
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