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Plasma cell dyscrasias

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https://www.readbyqxmd.com/read/28716071/primary-solitary-plasmacytoma-of-the-liver-successful-treatment-with-fractionated-stereotactic-radiotherapy-cyberknife%C3%A2-a-case-report
#1
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
#2
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
#3
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
#4
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 about SLONM-MGUS, which could help to understand clinical and pathological features and treatment...
July 3, 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
#5
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
#6
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...
June 27, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28617766/notes-from-the-field-evaluation-of-a-perceived-cluster-of-plasma-cell-dyscrasias-among-workers-at-a-natural-gas-company-illinois-2014
#7
Marie A de Perio, Jayesh Mehta
No abstract text is available yet for this article.
June 16, 2017: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/28606401/monoclonal-gammopathy-with-both-nemaline-myopathy-and-amyloid-myopathy
#8
Min Wang, Lin Lei, Hai Chen, Li Di, Mi Pang, Yan Lu, Lu Lu, Xin-Ming Shen, Yuwei Da
Monoclonal gammopathies due to plasma cell dyscrasias can induce diverse rare neuromuscular disorders. Deposition of monoclonal antibody light chains in skeletal muscle causes amyloid myopathy. Monoclonal gammopathy is occasionally associated with sporadic late-onset nemaline myopathy. Here we report a monoclonal gammopathy patient with both sporadic late-onset nemaline myopathy and amyloid myopathy. The diagnoses were based on immunofixation electrophoresis of urine, and serum for free light chain assay, Congo red staining and Thioflavin S staining of muscle biopsies, as well as immunohistochemical staining and electron-microscopic observation...
May 11, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28584670/solitary-plasmacytoma-of-the-mesentery-a-systematic-clinician-s-diagnosis
#9
Georgia Mitropoulou, Adamantia Zizi-Sermpetzoglou, Hippokrates Moschouris, Athanasios Kountourogiannis, Despoina Myoteri, Dionysios Dellaportas
INTRODUCTION: Plasmacytoma is an uncommon plasma cell neoplasm and its localized form is solitary plasmacytoma of the bone and solitary extramedullary plasmacytoma. Solitary plasmacytoma of the mesentery is extremely rare, reported only in a handful of cases. CASE PRESENTATION: A 47-year-old man with nonspecific abdominal complains was found to have an ill-defined mass on his mesenteric root. Laparoscopic biopsy and stepwise histopathological examination revealed a mesenteric plasmacytoma, and extensive imaging and laboratory investigations led to the diagnosis of the solitary mesenteric plasmacytoma...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/28573329/therapy-assessment-in-multiple-myeloma-with-pet
#10
REVIEW
Cristina Nanni, Elena Zamagni
Multiple myeloma is a plasma cell dyscrasia producing bone lytic lesions. In recent years, a wide spectrum of therapeutic approaches are available to treat the disease: an accurate therapy assessment has, therefore, become of utmost importance. In this field, imaging is becoming a cornerstone, especially in association with clinical parameters. Among imaging procedures, FDG PET/CT is recognized to provide reliable information, achieved in a very safe and fast procedure.  The literature has produced very concordant results from different groups assessing the value of FDG PET/CT as a prognostic factor in general and in therapy assessment, but some issues remain regarding a standardization of image interpretation especially in borderline cases...
June 1, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28545410/combined-proximal-tubulopathy-crystal-storing-histiocytosis-and-cast-nephropathy-in-a-patient-with-light-chain-multiple-myeloma
#11
Chung-Kuan Wu, An-Hang Yang, Hung-Chih Lai, Bing-Shi Lin
BACKGROUND: The diagnosis of myeloma, a plasma dyscrasia, often results from the workup of unexplained renal disease. Persistent renal failure in myeloma is commonly caused by tubular nephropathy due to circulating immunoglobulins and free light chains. Myeloma cast nephropathy is characterized by crystalline precipitates of monoclonal light chains within distal tubules. Immunoglobulin crystallization rarely occurs intracellularly, within proximal tubular cells (light chain proximal tubulopathy) and interstitial histiocytes (crystal-storing histiocytosis)...
May 25, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28542843/next-generation-sequencing-of-a-family-with-a-high-penetrance-of-monoclonal-gammopathies-for-the-identification-of-candidate-risk-alleles
#12
Niccolo Bolli, Matteo Barcella, Erika Salvi, Francesca D'Avila, Antonio Vendramin, Chiara De Philippis, Nikhil C Munshi, Herve Avet-Loiseau, Peter J Campbell, Alberto Mussetti, Cristiana Carniti, Francesco Maura, Cristina Barlassina, Paolo Corradini, Vittorio Montefusco
BACKGROUND: The authors describe a family with a high penetrance of plasma cell dyscrasias, suggesting inheritance of an autosomal dominant risk allele. METHODS: The authors performed whole-exome sequencing and reported on a combined approach aimed at the identification of causative variants and risk loci, using the wealth of data provided by this approach. RESULTS: The authors identified gene mutations and single-nucleotide polymorphisms of potential significance, and pinpointed a known risk locus for myeloma as a potential area of transmissible risk in the family...
May 23, 2017: Cancer
https://www.readbyqxmd.com/read/28512562/morphologic-confounders-and-cd19-negativity-in-a-case-of-hairy-cell-leukemia
#13
Pulkit Rastogi, Sreejesh Sreedharanunni, Uday Yanamandra, Man Updesh Singh Sachdeva, Neelam Varma
OBJECTIVES: We report a case of hairy cell leukemia (HCL) initially misdiagnosed as plasma cell dyscrasia due to various clinical, morphological and immunophenotypic confounders. METHODS AND RESULTS: In a patient diagnosed of marrow plasmacytosis and serum monoclonal protein elsewhere and referred to our hospital, morphological evaluation of bone marrow aspirate smears and trephine biopsy, immunophenotyping, and molecular testing (BRAFV600E mutation) were done. Clinically, the patient was asymptomatic; bone marrow revealed plasmacytosis, mastocytosis, and lymphocytosis with a few "hairy" cells...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28505007/al-amyloidoma-of-the-skin-subcutis-cutaneous-amyloidosis-plasma-cell-dyscrasia-or-a-manifestation-of-primary-cutaneous-marginal-zone-lymphoma
#14
Noreen M Walsh, Ian Marie Lano, Peter Green, Christopher Gallant, Sylvia Pasternak, Thai Yen Ly, Luis Requena, Heinz Kutzner, Andreas Chott, Lorenzo Cerroni
It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits...
August 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28502031/vitamin-d-and-plasma-cell-dyscrasias-reviewing-the-significance
#15
REVIEW
Nicholas Burwick
Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder and precursor disease to multiple myeloma and other related cancers. While MGUS is considered a benign disorder, with a low risk of disease progression, patients have altered bone microarchitecture and an increased risk of bone fracture. In addition, alterations in immune function are regularly found to correlate with disease activity. Vitamin D, an important hormone for bone and immune health, is commonly deficient in multiple myeloma patients...
May 13, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28483281/preclinical-data-support-leveraging-cs1-chimeric-antigen-receptor-t-cell-therapy-for-systemic-light-chain-amyloidosis
#16
Michael Rosenzweig, Ryan Urak, Miriam Walter, Laura Lim, James F Sanchez, Amrita Krishnan, Stephen Forman, Xiuli Wang
BACKGROUND AIMS: Light chain amyloidosis (AL) is a protein deposition disorder that is a result of a plasma cell dyscrasia, similar to multiple myeloma (MM). Immunotherapy is an attractive approach because of the low burden of disease, but the optimal target for AL is unclear. CS1 and B-cell maturation antigen (BCMA) are two potential targets because they are expressed on normal plasma cells and MM cells. METHODS: We performed a prospective study evaluating bone marrow specimens of 20 patients with plasma cell diseases, 10 with AL and 10 with MM...
July 2017: Cytotherapy
https://www.readbyqxmd.com/read/28471126/multiple-myeloma-with-an-unusual-oral-presentation
#17
REVIEW
Anjali Nayak, Meghanand T Nayak
Multiple myeloma (MM) is a dyscrasia caused by neoplastic proliferation of somatically mutated plasma cells. Myeloma cells divide and expand within the bone marrow affecting multiple locations in the body where bone marrow is present. Oral manifestations, though common are usually not diagnosed. This report describes rare oral manifestations of multiple myeloma like oral petechiae and gingival swelling presented in a 40-year-old male patient. The diagnosis was established by blood examination & skeletal survey, while oral findings were later correlated with the diagnosis of MM...
July 2016: Journal of Experimental Therapeutics & Oncology
https://www.readbyqxmd.com/read/28469872/perioperative-intravenous-immunoglobulin-treatment-in-a-patient-with-severe-acquired-von-willebrand-syndrome-case-report-and-review-of-the-literature
#18
Eva Jennes, Dorothee Guggenberger, Rainer Zotz, Lora Thompson, Tim H Brümmendorf, Steffen Koschmieder, Edgar Jost
Acquired von Willebrand syndrome may be related to plasma cell dyscrasia and can cause severe bleeding complications. Treatment, for example, with intravenous immunoglobulins may be indicated in selected cases. Physicians treating plasma cell dyscrasia have to be aware of bleeding complications in these patients, and clarification is necessary.
May 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28451433/lenalidomide-induced-eosinophilic-pneumonia
#19
Andrew Toma, Aaron P Rapoport, Allen Burke, Ashutosh Sachdeva
Multiple myeloma is a plasma cell dyscrasia accounting for 10% of haematologic malignancies. Lenalidomide is an immunomodulatory drug analogous to thalidomide that is approved for use in patients with myelodysplastic syndrome, and in combination with dexamethasone for refractory or relapsed multiple myeloma. Lenalidomide is preferred to thalidomide because of reduced toxicity, and pulmonary side effects are considered rare. We present, to our knowledge, an unusual and first reported case of a patient with relapsed multiple myeloma who received lenalidomide after autologous stem cell transplant, then developed eosinophilic pneumonia presenting as dyspnoea, peripheral eosinophilia, and bilateral pulmonary opacities...
July 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28444965/serum-free-light-chain-quantitative-assays-dilemma-of-a-biomarker
#20
Giovanni Cigliana, Francesca Gulli, Cecilia Napodano, Krizia Pocino, Elena De Santis, Luigi Colacicco, Iole Cordone, Laura Conti, Umberto Basile
BACKGROUND: Serum free light chains detection assays are consistently meeting greater interest for the diagnosis and monitoring of monoclonal gammopathies and plasma cell dyscrasias. Nowadays, there are neither standardized methods nor reference material for the determination of free light chains; for this reason, it is important to compare two different assays used in clinical laboratory. METHODS: We evaluated 300 serum samples from patients with B-cell disorders and compared the analytical performances of both assay...
April 26, 2017: Journal of Clinical Laboratory Analysis
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