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https://www.readbyqxmd.com/read/28806822/-multiple-myeloma-current-status-in-diagnostic-testing-and-therapy
#1
Michael Kehrer, Sebastian Koob, Andreas Strauss, Dieter Christian Wirtz, Jan Schmolders
Background Multiple myeloma is a haematological blood cancer of the bone marrow and is classified by the World Health Organisation (WHO) as a plasma cell neoplasm. In multiple myeloma, normal plasma cells transform into malignant myeloma cells and produce large quantities of an abnormal immunoglobulin called monoclonal protein or M protein. This ultimately causes multiple myeloma symptoms such as bone damage or kidney problems. The annual worldwide incidence of multiple myeloma is estimated to be 6 - 7/100,000 and accounts for 1% of all cancer...
August 14, 2017: Zeitschrift Für Orthopädie und Unfallchirurgie
https://www.readbyqxmd.com/read/28802501/myeloid-neoplasms
#2
REVIEW
Antonio Subtil
The classification of myeloid neoplasms has undergone major changes and currently relies heavily on genetic abnormalities. Cutaneous manifestations of myeloid neoplasms may be the presenting sign of underlying bone marrow disease. Dermal infiltration by neoplastic cells may occur in otherwise normal skin or in sites of cutaneous inflammation. Leukemia cutis occasionally precedes evidence of blood and/or bone marrow involvement (aleukemic leukemia cutis).
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28798246/extramedullary-haematopoiesis-presenting-with-cardiac-tamponade-in-a-patient-with-polycythaemia-vera
#3
Faysal Haroun, Viktoria Elkis, Anne Chen, Elsie Lee
A 71-year-old man with a history of polycythaemia vera, diagnosed 4 years ago, presented to the emergency room with shortness of breath. A bedside echocardiogram revealed a large pericardial effusion with features concerning for pericardial tamponade. A left anterior thoracotomy and a pericardial window were emergently performed in the operating room and relieved the patient's symptoms. Histology evaluation of the pericardial fragments and pericardial fluid revealed the presence of trilineage haematopoietic elements without any increase in the blasts...
August 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28797996/detection-mutations-of-jak2-exon-12-in-patients-with-jak2-v617f-negative-myeloproliferative-disorders
#4
S Z Makani, N Parsamanesh, S Mirzaahmadi, M Hashemi, F Shaveisi-Zadeh, N Mansouri, M Ghazi, A Movafagh
BACKGROUND: Mutations in exon 12 of JAK2 gene are detected as clonal markers in hematopoietic lineages in myeloproliferative disorders (MPNs). Our aim was, to study the relation between N542-E543del mutation of JAK2 gene and myeloproliferative neoplasms in V617Fnegative patients. PATIENTS AND METHODS: DNA specimen from 34 patients and 44 healthy controls were genotyped using ARMs- PCR method. We analyzed exon 12 JAK2 aberration in 34 myeloproliferative cases to be readily detected by both ARMS-PCR and DNA analysis regardless of whether peripheral blood or bone marrow cells was manipulated as the origin of RNA...
May 2017: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28796790/mesenchymal-stromal-cells-msc-from-jak2-myeloproliferative-neoplasms-differ-from-normal-msc-and-contribute-to-the-maintenance-of-neoplastic-hematopoiesis
#5
Teresa L Ramos, Luis Ignacio Sánchez-Abarca, Beatriz Rosón-Burgo, Alba Redondo, Ana Rico, Silvia Preciado, Rebeca Ortega, Concepción Rodríguez, Sandra Muntión, Ángel Hernández-Hernández, Javier De Las Rivas, Marcos González, José Ramón González Porras, Consuelo Del Cañizo, Fermín Sánchez-Guijo
There is evidence of continuous bidirectional cross-talk between malignant cells and bone marrow-derived mesenchymal stromal cells (BM-MSC), which favors the emergence and progression of myeloproliferative neoplastic (MPN) diseases. In the current work we have compared the function and gene expression profile of BM-MSC from healthy donors (HD-MSC) and patients with MPN (JAK2V617F), showing no differences in the morphology, proliferation and differentiation capacity between both groups. However, BM-MSC from MPN expressed higher mean fluorescence intensity (MIF) of CD73, CD44 and CD90, whereas CD105 was lower when compared to controls...
2017: PloS One
https://www.readbyqxmd.com/read/28793931/strategy-for-the-treatment-and-follow-up-of-sinonasal-solitary-extramedullary-plasmacytoma-a-case-series
#6
Elena Cantone, Antonella Miriam Di Lullo, Luana Marano, Elia Guadagno, Gelsomina Mansueto, Pasquale Capriglione, Lucio Catalano, Maurizio Iengo
BACKGROUND: Extramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells outside bone marrow. It accounts for 4% of all non-epithelial sinonasal tumors. According to the literature, radiotherapy is the standard therapy for extramedullary plasmacytoma. However, the conversion rate of extramedullary plasmacytoma to multiple myeloma is reported to be between 11 and 33% over 10 years. The highest risk of conversion is reported during the first 2 years after diagnosis, but conversion has been noted up to 15 years after diagnosis...
August 10, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28792693/serum-hepcidin-levels-in-multiple-myeloma
#7
Manolov Victor, Hadjiev Evgeniy, Tzvetkova Gergana, Petrova Julia, Vasilev Vasil, Marinov Borislav, Bogov Ivo, Gramatikova Zlatina, Tzatchev Kamen
BACKGROUND: Multiple myeloma (MM) is a malignant disease with a 10% frequency among all haematology neoplasms. It is characterized by clone proliferation of plasmatic cells in bone marrow, monoclonal gammopathy, and anemia, hypercalcemia, and kidney failure and bone lesions. IL-6 is an inflammatory cytokine, potential growth factor for myeloma cells, as elevated serum levels are connected with poor disease prognosis. IL-6 modulates many gene transcriptions, encoding synthesis of acute phase proteins, including C-reactive protein (CRP) and hepcidin...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28791654/pharmacotherapy-of-myelofibrosis
#8
REVIEW
Douglas Tremblay, Bridget Marcellino, John Mascarenhas
Myelofibrosis (MF) is a myeloproliferative neoplasm that is pathologically characterized by bone marrow myeloproliferation, reticulin and collagen fibrosis, and extramedullary hematopoiesis. Constitutive activation of the Janus associated kinase (JAK)-signal transducers and activators of transcription signaling pathway with resultant elevation in pro-inflammatory cytokine levels is the pathogenic hallmark of MF. JAK inhibitors, namely ruxolitinib, have been successful in alleviating symptoms and reducing splenomegaly, but therapy-related myelosuppression has led to the further development of highly selective JAK2 inhibitors...
August 8, 2017: Drugs
https://www.readbyqxmd.com/read/28777856/-clinical-and-laboratory-features-of-13-cases-of-myeloid-neoplasms-with-double-del-20q
#9
Shuxiao Bai, Chunxiao Wu, Jun Zhang, Suning Chen, Jinlan Pan
OBJECTIVE: To report on clinical and laboratory features of myeloid neoplasms with double del(20q). METHODS: Cytogenetic examination of bone marrow was performed on 13 cases of myeloid neophasms with double del(20q) after 24 hours of cell culture. R-banding was used to analyze the karyotypes. Interphase fluorescence in situ hybridization (FISH) was performed using dual-color probes for 20q11/20q12. RESULTS: Double del(20q) was found to be the sole abnormality in 9 cases, double del(20q) and trisomy 9 was found in 1 case, trisomy del(20q) was found in 1 case, and sole del(20q) clone and double del(20q) clone were found to coexist in 2 cases...
August 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28775205/therapy-related-hematological-malignancies-after-peptide-receptor-radionuclide-therapy-with-177-lu-dota-octreotate-incidence-course-predicting-factors-in-patients-with-gep-nets
#10
Hendrik Bergsma, Kirsten van Lom, Mark Konijnenberg, Boen Kam, Jaap Teunissen, Wouter de Herder, Eric Krenning, Dik Kwekkeboom
Peptide Receptor Radionuclide Therapy (PRRT) may induce long-term toxicity to the bone marrow (BM). The aim of this study was to analyze persistent dysfunction of the hematopoietic system after PRRT with (177)Lu-DOTATATE in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Methods: The incidence and course of persistent hematological dysfunction (PHD) was analyzed in 274 (=GEP-NET) out of 367 patients with somatostatin receptor-positive tumors. PHD was defined as diagnosis of Myelodysplastic Syndrome (MDS), Acute Myeloid Leukemia (AML), Myeloproliferative Neoplasms (MPN), Myelodysplastic/Myeloproliferative neoplasms (MDS/MPN) or otherwise unexplained cytopenia (for more than 6 months)...
August 3, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28770949/treatment-of-patients-with-primary-myelofibrosis-using-dasatinib
#11
Q-L Song, B Zhang, Y Xu, R-X Xia, X-H Lu, Z-X Pei, Q-W Xu, W-Y Li, Z-D Li
OBJECTIVE: Primary myelofibrosis (PMF) is a chronic clonal myeloproliferative neoplasm. It is associated with a poor prognosis, with a median survival time of approximately five years. Thus far, there are no specific targeted drugs for PMF. In this study, we evaluated the efficacy and safety of dasatinib, a second-generation tyrosine kinase inhibitor, in six PMF patients. PATIENTS AND METHODS: From June 1, 2015 to February 29, 2016, six patients with PMF in our department were enrolled into this trial...
July 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28760297/progress-in-myelodysplastic-syndromes-clinicopathologic-correlations-and-immune%C3%A2-checkpoints
#12
Juliana E Hidalgo-López, Rashmi Kanagal-Shamanna, Andrés E Quesada, Beenu Thakral, Zhihong Hu, Takayuki Mitsuhashi, Mariko Yabe, Guillermo Garcia-Manero, Carlos E Bueso-Ramos
BACKGROUND: Myelodysplastic syndromes (MDS) are a group of clonal neoplasms characterized by ineffective hematopoiesis. Hypomethylating agent (HMA) therapy is one of the mainstays of MDS therapy. Failure of HMA therapy is related to poor outcome; hence, new therapeutic approaches are warranted in these patients. In MDS, the immune system has a pivotal role in modulation of hematopoiesis and clonal expansion. In neoplastic conditions, immune checkpoint (PD-1 and CTLA4 molecules) hide tumor cells from immune surveillance...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28745329/involvement-of-maf-spp1-axis-in-the-development-of-bone-marrow-fibrosis-in-pmf-patients
#13
S Ruberti, E Bianchi, P Guglielmelli, S Rontauroli, G Barbieri, L Tavernari, T Fanelli, R Norfo, V Pennucci, G C Fattori, C Mannarelli, N Bartalucci, B Mora, L Elli, M A Avanzini, C Rossi, S Salmoiraghi, R Zini, S Salati, Z Prudente, V Rosti, F Passamonti, A Rambaldi, S Ferrari, E Tagliafico, A M Vannucchi, R Manfredini
Primary Myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by hyperplastic megakaryopoiesis and myelofibrosis. We recently described the upregulation of MAF in PMF CD34+ hematopoietic progenitor cells (HPCs) compared to healthy donor. Here we demonstrated that MAF is also upregulated in PMF compared to Essential Thrombocytemia (ET) and Polycytemia Vera (PV) HPCs. MAF overexpression and knockdown experiments shed some light into the role of MAF in PMF pathogenesis, by demonstrating that MAF favours the megakaryocyte and monocyte/macrophage commitment of HPCs and leads to the increased expression of proinflammatory and profibrotic mediators...
July 12, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28744009/efficacy-and-safety-of-midostaurin-in-patients-with-advanced-systemic-mastocytosis-10-year-median-follow-up-of-a-phase-ii-trial
#14
D J DeAngelo, T I George, A Linder, C Langford, C Perkins, J Ma, P Westervelt, J D Merker, C Berube, S Coutre, M Liedtke, B Medeiros, D Sternberg, C Dutreix, P-A Ruffie, C Corless, T J Graubert, J Gotlib
Patients with advanced systemic mastocytosis (SM) (aggressive SM [ASM], SM with an associated hematologic neoplasm [SM-AHN], and mast cell leukemia [MCL]) have limited treatment options and exhibit reduced survival. Midostaurin is an oral multikinase inhibitor that inhibits D816V-mutated KIT, a primary driver of SM pathogenesis. We conducted a phase II trial of midostaurin 100 mg twice daily, administered as 28-day cycles, in 26 patients (ASM, n=3; SM-AHN, n=17; MCL, n=6) with at least one sign of organ damage...
July 24, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28743360/signs-and-symptoms-of-rheumatic-diseases-as-first-manifestation-of-pediatric-cancer-diagnosis-and-prognosis-implications
#15
Mariana Bertoldi Fonseca, Francisco Hugo Rodrigues Gomes, Elvis Terci Valera, Gecilmara Salviato Pileggi, Paula Braga Gonfiantini, Marcela Braga Gonfiantini, Virgínia Paes Leme Ferriani, Luciana Martins de Carvalho
OBJECTIVE: To assess the prevalence and describe the clinical, laboratory and radiological findings, treatment and outcome of children with cancer initially referred to a tertiary outpatient pediatric rheumatology clinic. METHODS: Retrospective analysis of medical records from patients identified in a list of 250 new patients attending the tertiary Pediatric Rheumatology Clinic, Ribeirão Preto Medical School hospital, University of São Paulo, from July 2013 to July 2015, whose final diagnosis was cancer...
July 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28737134/nephrotic-syndrome-in-primary-myelofibrosis-with-renal-extramedullary-hematopoiesis-and-glomerulopathy-in-the-jak-inhibitor-era
#16
Rachele Del Sordo, Rachele Brugnano, Carla Covarelli, Gioia Fiorucci, Franca Falzetti, Giorgio Barbatelli, Emidio Nunzi, Angelo Sidoni
Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure...
July 24, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28723285/case-244-systemic-amyloidosis-a-complication-of-waldenstr%C3%A3-m-macroglobulinemia
#17
Maja Hrabak-Paar, Marko Kralik
History A 68-year-old man was admitted to the hospital for work-up because of generalized fatigue, anorexia, chronic diarrhea, and weight loss. Laboratory work-up revealed an erythrocyte sedimentation rate of 58 mm/h (reference range, 3-23 mm/h), a hemoglobin level of 14.1 g/dL (reference range, 13.8-17.5 g/dL), a leukocyte count of 8.1 × 10(9)/L (reference range, [3.4-9.7] × 10(9)/L), a platelet count of 223 × 10(9)/L (reference range, [158-424] × 10(9)/L), an alkaline phosphatase level of 85 U/L (1.42 μkat/L) (normal level, <142 U/L [2...
August 2017: Radiology
https://www.readbyqxmd.com/read/28711573/distribution-and-impact-of-comorbidities-on-survival-and-leukemic-transformation-in-myeloproliferative-neoplasm-associated-myelofibrosis-a-retrospective-cohort-study
#18
Justyna Bartoszko, Tony Panzarella, Caroline Jane McNamara, Anthea Lau, Aaron D Schimmer, Andre C Schuh, Hassan Sibai, Dawn Maze, Karen W L Yee, Rebecca Devlin, Vikas Gupta
BACKGROUND: We sought to describe the distribution and impact of comorbidities on outcomes in patients with myelofibrosis, a disease characterized by aberrant bone marrow function with eventual fibrosis. Comorbidities were scored using the Adult Comorbidity Evaluation-27 (ACE-27) and the Hematopoietic Cell Transplant Comorbidity Index (HCT-CI), in which a score ≥ 3 indicates severe comorbidities. PATIENTS AND METHODS: We conducted a retrospective study of 306 patients with a confirmed diagnosis of myelofibrosis...
June 29, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28702412/megakaryocytic-morphology-in-janus-kinase-2-v617f-positive-myeloproliferative-neoplasm
#19
Shuchi Ghai, Sharada Rai
CONTEXT: Alterations in megakaryocyte morphology are the hallmark of myeloproliferative neoplasms (MPNs). These neoplasm are also associated with Janus kinase 2 (JAK2) V617F mutation in nearly 95% patients with polycythemia vera (PV), 40% patients of essential thrombocythemia (ET) and 50% patients of myelofibrosis (MF). The utility of megakaryocyte morphology in these disorders in correlation with JAK2 V617F remains unresolved. AIMS: The aim of the study was to assess the morphology of megakaryocytes in bone marrow aspirates (BMAs) and bone marrow biopsies of patients of BCR-ABL negative MPNs with JAK2 V617F mutation...
April 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28699668/poems-syndrome-2017-update-on-diagnosis-risk-stratification-and-management
#20
REVIEW
Angela Dispenzieri
DISEASE OVERVIEW: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy...
August 2017: American Journal of Hematology
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