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Plasma cell dyscrasia

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https://www.readbyqxmd.com/read/29128071/diagnosis-of-plasma-cell-dyscrasias-and-monitoring-of-minimal-residual-disease-by-multiparametric-flow-cytometry
#1
REVIEW
Kah Teong Soh, Joseph D Tario, Paul K Wallace
Plasma cell dyscrasia (PCD) is a heterogeneous disease that has seen a tremendous change in outcomes due to improved therapies. Over the past few decades, multiparametric flow cytometry has played an important role in the detection and monitoring of PCDs. Flow cytometry is a high-sensitivity assay for early detection of minimal residual disease (MRD) that correlates well with progression-free survival and overall survival. Before flow cytometry can be effectively implemented in the clinical setting, sample preparation, panel configuration, analysis, and gating strategies must be optimized to ensure accurate results...
December 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/29111449/editorial-on-laboratory-diagnosis-and-management-of-plasma-cell-dyscrasias-special-issue
#2
EDITORIAL
David Keren, Christopher R McCudden, Ronald A Booth
No abstract text is available yet for this article.
October 27, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/29107338/prevalence-and-clinicopathologic-characteristics-of-multiple-myeloma-with-cutaneous-involvement-a-case-series-from-korea
#3
Yu Ri Woo, Jong Sic Kim, Ji Hong Lim, Sewon Hwang, Miri Kim, Jung Min Bae, Young Min Park, Chang-Ki Min, Dong-Wook Kim, Hyun Jeong Park
BACKGROUND: Multiple myeloma (MM) is a plasma cell dyscrasia characterized by the presence of a clonal proliferation of tumor cells. Cutaneous involvement of MM is very rare and remains poorly understood. OBJECTIVE: The aim of this study was to examine the clinical and histopathologic characteristics of cutaneous involvement in MM and identify factors associated with overall survival of MM with cutaneous involvement. METHODS: The medical records of 1228 patients with MM were retrieved and analyzed...
October 26, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29106288/localized-gastrointestinal-amyloidosis-presenting-with-protein-losing-enteropathy-and-massive-hemorrhage
#4
Bárbara Corrêa, Cristiane Kibune Nagasako, Ciro Garcia Montes, Marlone Cunha-Silva, Maria Aparecida Mesquita
Amyloidosis of the gastrointestinal tract is usually a systemic disease. Localized gastrointestinal amyloidosis without evidence of extraintestinal involvement or an associated plasma cell dyscrasia is uncommon and does not usually cause death. We report a case of a patient with localized gastrointestinal amyloidosis who presented with protein-losing enteropathy and a fatal upper gastrointestinal bleed.
November 6, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29065679/localized-gastric-amyloidosis-with-kappa-and-lambda-light-chain-co-expression
#5
Yong Hwan Ahn, Ye Young Rhee, Suck Chei Choi, Geom Seog Seo
Esophagogastroduodenoscopy for cancer screening was performed in a 55-year-old woman as part of a health screening program, and revealed a depressed lesion approximately 20 mm in diameter in the lesser curvature of the mid-gastric body. Several biopsy specimens were collected as the lesion resembled early gastric cancer; however, histopathologic evaluation revealed chronic active gastritis with an ulcer and amorphous eosinophilic material deposition. Congo red staining identified amyloid proteins, and apple-green birefringence was shown using polarized light microscopy...
October 26, 2017: Clinical Endoscopy
https://www.readbyqxmd.com/read/29057130/cutaneous-vasculitis-an-unusual-presentation-of-a-biclonal-nodal-plasma-cell-dyscrasia
#6
D Swan, M Murphy, E Elhassadi
We describe an unusual case of a biclonal nodal plasma cell dyscrasia, presenting with a vasculitic rash, end-organ damage, and cytopenias. Serum protein electrophoresis demonstrated a biclonal kappa-restricted paraprotein, with a negative skeletal survey and no bone marrow disease. Fluorodeoxyglucose-PET-CT (FDG-PET-CT) revealed nodal involvement, which was not appreciable clinically, and facilitated biopsy, confirming the diagnosis of a nodal plasmacytoma. Complete biochemical response and resolution of the vasculitic rash were achieved with bortezomib-based therapy...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29056130/an-adult-with-polyneuropathy-and-hypogonadism-due-to-poems-syndrome
#7
Saba Zaidi, Sidra Sattar, Khealani Bhojo Asumal
POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein myeloma and skin changes), is a rare disease which occurs in the setting of plasma cell dyscrasias. We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. Nerve conduction study showed severe sensorimotor polyneuropathy. Serum immunofixation showed lambda light chain restricted monoclonal gammopathy...
October 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29053157/current-applications-of-multiparameter-flow-cytometry-in-plasma-cell-disorders
#8
REVIEW
T Jelinek, R Bezdekova, M Zatopkova, L Burgos, M Simicek, T Sevcikova, B Paiva, R Hajek
Multiparameter flow cytometry (MFC) has become standard in the management of patients with plasma cell (PC) dyscrasias, and could be considered mandatory in specific areas of routine clinical practice. It plays a significant role during the differential diagnostic work-up because of its fast and conclusive readout of PC clonality, and simultaneously provides prognostic information in most monoclonal gammopathies. Recent advances in the treatment and outcomes of multiple myeloma led to the implementation of new response criteria, including minimal residual disease (MRD) status as one of the most relevant clinical endpoints with the potential to act as surrogate for survival...
October 20, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/29046749/an-unusual-case-of-chronic-lymphocytic-leukemia-multiple-myeloma-and-cardiac-amyloidosis
#9
Dongyan Liu, Hakim T Uqdah, Alisha D Gordy
Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted multiple myeloma with plasma cells causing AL amyloidosis involving the heart. While monoclonal immunoglobulins occasionallyproduced by CLL have previously been implicated in AL amyloidosis, there only a few cases reported of AL amyloidosis resulting from a distinct plasma cell dyscrasia that is not clonally related to the concurrent CLL...
October 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29035194/clinicopathologic-characteristics-of-light-chain-proximal-tubulopathy-with-light-chain-inclusions-involving-multiple-renal-cell-types%C3%A2
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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