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https://www.readbyqxmd.com/read/27913526/update-from-the-latest-who-classification-of-mpns-a-user-s-manual
#1
Francesco Passamonti, Margherita Maffioli
The 2016 multiparameter World Health Organization (WHO) classification for Philadelphia-negative myeloproliferative neoplasms (MPNs) integrates clinical features, morphology, and genetic data to diagnose polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The main novelties are: (1) the reduction of the hemoglobin (Hb) level threshold to diagnose PV, now established at 16.5 g/dL for men and 16 g/dL for women (based on the identification of MPN patients with PV-consistent bone marrow [BM] features and a Hb level lower than that established in the 2008 WHO classification for PV); (2) the recognition of prefibrotic/early PMF, distinguishable from ET on the basis of BM morphology, an entity having a higher tendency to develop overt myelofibrosis or acute leukemia, and characterized by inferior survival; (3) the central role of BM morphology in the diagnosis of ET, prefibrotic/early PMF, PMF, and PV with borderline Hb values; megakaryocyte number and morphology (typical in ET, atypical in both PMF forms) accompanied by a new distinction of reticulin fibrosis grade in PMF (grade 1 in prefibrotic/early PMF and grade 2-3 in PMF) constitute diagnostic criteria; and (4) the inclusion of all mutually exclusive MPN driver mutations (JAK2, CALR, and MPL) as major diagnostic criteria in ET and PMF; 10% to 15% of these patients are triple negative, and in these cases the search for an additional clonal marker (eg, mutations in ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, and SF3B1) is warranted...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913464/new-challenges-in-evaluating-anemia-in-older-persons-in-the-era-of-molecular-testing
#2
David P Steensma
Anemia is common in older persons, and often remains unexplained despite a thorough clinical history, physical examination, and focused laboratory testing, including marrow aspiration, biopsy, and karyotyping. The advent of molecular genetic testing panels in hematology clinical practice has complicated the evaluation of older patients with unexplained anemia. While the presence of a somatic mutation provides evidence of clonal hematopoiesis and may support a diagnosis of a hematologic neoplasm such as one of the myelodysplastic syndromes (MDS), with rare exceptions, individual mutations are not strongly associated with one specific diagnosis, nor are they by themselves diagnostic of neoplasia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27905671/long-term-survivors-of-childhood-bone-and-soft-tissue-sarcomas-are-at-risk-of-hospitalization
#3
Cristian D Gonzalez, R Lor Randall, Jennifer Wright, Holly Spraker-Perlman, Jian Ying, Carol Sweeney, Ken R Smith, Anne C Kirchhoff
BACKGROUND: Childhood cancer survivors can have a high burden of chronic conditions related to cancer treatment, some of which are debilitating or potentially life-threatening. Much remains to be learned about late effects in bone and soft tissue sarcoma survivors. PROCEDURES: The Utah Cancer Registry was used to identify survivors of bone (N = 71) and soft tissue sarcomas (N = 98) who were diagnosed at ages 0-20 years between 1973 and 2007 and were alive at least 5 years after diagnosis...
December 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27903019/osteochondroma-presenting-as-a-calcified-mass-in-the-sellar-region-and-review-of-the-literature
#4
Kazuya Sekiguchi, Satoshi Tsutsumi, Sho Arai, Senshu Nonaka, Takamoto Suzuki, Hisato Ishii, Hiroshi Izumi, Yukimasa Yasumoto
Objective Osteochondroma (OC) is the most common benign bone neoplasm. It infrequently occurs in the cranial cavity as a calcified lesion and very rarely presents in the sellar region. The present study summarizes the knowledge about OCs of the sellar region. Methods We searched the literature search for the clinical appearance of OCs and other calcified pathologies occurring in the sellar region. Results A total of 21 English-language articles published from 1961 to 2015 documented cases of calcified lesions in the sellar region including cerebral aneurysm, chondroid chordoma, chondroma, craniopharyngioma, OC, odontome, osteoma, pituitary adenoma, pituitary stone, Rathke cleft cyst, retinoblastoma, schwannoma, and xanthogranuloma...
November 30, 2016: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/27900869/-evaluation-of-five-years-of-treatment-of-erdheim-chester-disease-with-anakinra-case-report-and-overview-of-literature
#5
Zdeněk Adam, Hana Petrášová, Zdeněk Řehák, Renata Koukalová, Marta Krejčí, Luděk Pour, Eva Vetešníková, Aleš Čermák, Sabina Ševčíková, Petr Szturz, Zdeněk Král, Jiří Mayer
: Erdheim-Chester disease is a histiocytic neoplasm of diseases from the group of non-Langerhans-cell histiocytoses, formed by infiltrates of foamy histiocytes. These pathological histiocytes produce pro-inflammatory cytokines. Therefore Erdheim-Chester disease is called inflammatory histiocytary neoplasm. The disease is accompanied by clinical symptoms of systemic inflammatory response, i.e. B symptoms. Imaging examinations detect typical osteosclerotic changes affecting diaphyses and metaphyses of the lower long bones and fibrotic changes which affect the aorta wall and the vessels leading from it...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27899193/genetic-predisposition-to-leukemia-and-other-hematologic-malignancies
#6
REVIEW
Simone Feurstein, Michael W Drazer, Lucy A Godley
In this review, we provide an overview of familial myelodysplastic syndromes (MDS)/acute leukemia (AL) and bone marrow failure syndromes, as well as insights into familial myeloproliferative neoplasms (MPNs), familial multiple myeloma (MM), familial Waldenström macroglobulinemia (WM), familial lymphoma, and cancer predisposition syndromes with increased risk of MDS/AL. This field will continue to accelerate as next-generation sequencing (NGS) techniques identify novel predisposition alleles in families with a genetic predisposition to hematologic malignancies...
October 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/27894618/primary-pulmonary-pleural-melanoma-in-a-13-year-old-presenting-as-pleural-effusion
#7
Nick Baniak, Mark Podberezin, Selliah C Kanthan, Rani Kanthan
Melanoma in children, adolescents, and young adults is uncommon and reported almost exclusively as cutaneous melanoma. Melanoma presenting as a pleural effusion is very rare in adults and not reported in the pediatric population. Additionally, primary pulmonary melanoma is overall very rare and undocumented in pediatric patients. Furthermore, the distinction between a primary pulmonary/pleural melanoma versus a regressed cutaneous melanoma with pulmonary/pleural metastases remains extremely challenging. We discuss a case of a previously healthy 13-year-old girl that presented with a left-sided pleural effusion...
November 16, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27889325/-langerhans-cell-histiocytosis-and-erdheim-chester-disease-a-continuity
#8
S Parreau, J Haroche, I Pommepuy, J F Emile, J C Bourras, F Archambeaud
INTRODUCTION: Erdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described. OBSERVATION: A 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Bone biopsy showed langerhans cell histiocytosis CD1a positive with the presence of BRAF V600E mutation...
November 23, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27889090/focal-nodular-and-diffuse-haematopoietic-marrow-hyperplasia-in-patients-with-underlying-malignancies-a-radiological-mimic-of-malignancy-in-need-of-recognition
#9
L T C Chow, A W H Ng, S K C Wong
AIM: To report the authors' experience of focal nodular haematopoietic marrow hyperplasia (FNHMH) and diffuse haematopoietic marrow hyperplasia (DHMH) clinically masquerading as skip, distant, or disseminated metastasis in seven patients with underlying malignant neoplasms. MATERIALS AND METHODS: Five patients with FNHMH and two with DHMH mistaken radiologically as skip and disseminated metastasis, respectively, were compared and contrasted with four patients with osteosarcomas and two with chondrosarcomas harbouring skip metastasis, noting the temporal relationship with their haematological profile...
November 24, 2016: Clinical Radiology
https://www.readbyqxmd.com/read/27882239/desmoplastic-fibroma-of-the-distal-tibia-a-case-report-of-a-minimally-invasive-histological-diagnosis
#10
Gabriele Levrini, Pierpaolo Pattacini
Desmoplastic fibroma (DF) is a benign, rare fibroblastic intraosseous neoplasm histologically resembling a desmoid soft tissue tumor. Although classified as benign, DF frequently exhibits an aggressive behavior, has a moderate-to-high recurrence rate, and often causes pathological fractures and extensive bone destruction. This case report presents an incidentally detected DF of the tibia, which was diagnosed using a minimally invasive approach. A 36-year-old African female patient was referred to the Department of Diagnostic Imaging of Arcispedale Santa Maria Nuova-IRCCS (Reggio Emilia, Italy), to be examined by a computed tomography scan on an outpatient basis, after an x-ray examination of the tibia, which was performed after an injury to exclude the presence of a fracture, revealed a hyperlucency of unknown origin...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27878449/pseudomyogenic-hemangioendothelioma-a-vascular-tumor-previously-undescribed-in-the-oral-cavity
#11
Yeshwant B Rawal, Kenneth M Anderson, Thomas B Dodson
The pseudomyogenic hemangioendothelioma (PMH) is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. Primary tumors in the skeletal muscle and bone have also been diagnosed. The PMH was introduced into the WHO classification of tumors of soft tissue and bone in 2013. This is the first description of oral involvement. A 21-year-old female presented with a 2-month old swelling of her gingiva. The swelling appeared red in color and was soft in consistency. A clinical diagnosis of a pyogenic granuloma was made and an incisional biopsy was submitted for histopathological evaluation...
November 22, 2016: Head and Neck Pathology
https://www.readbyqxmd.com/read/27875373/revisiting-erythroleukemia
#12
Daniel A Arber
PURPOSE OF REVIEW: The 2016 WHO classification of hematopoietic and lymphoid neoplasms alters the diagnostic criteria for erythroleukemia, including eliminating the erythroid/myeloid type of acute erythroleukemia, which was a prior subcategory of acute myeloid leukemia, not otherwise specified. Only pure erythroid leukemia remains in the WHO classification. This review will summarize the literature that contributed to that classification change as well as recent literature on the significance of the change...
November 21, 2016: Current Opinion in Hematology
https://www.readbyqxmd.com/read/27873026/long-term-efficacy-and-side-effects-of-low-dose-tacrolimus-for-the-treatment-of-myasthenia-gravis
#13
Xiaoyong Tao, Wei Wang, Feng Jing, Zhongkui Wang, Yuping Chen, Dongning Wei, Xusheng Huang
The study evaluated the efficacy of low-dose tacrolimus for treating Myasthenia Gravis (MG). Data were collected from 97 patients treated with low-dose tacrolimus from February 2011 to April 2015. Metabolic analysis was performed to determine more accurate tacrolimus dosing and patients were followed-up within clinic every 6 months for up to 4 years. The myasthenia gravis-specific activities of daily living scale was used to assess MG symptoms and their effects on patients' daily activities. All side effects and adverse reactions were thoroughly documented...
November 21, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27870387/primary-myelofibrosis-2017-update-on-diagnosis-risk-stratification-and-management
#14
Ayalew Tefferi
: Disease overview: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR or MPL mutation, abnormal cytokine expression, bone marrow fibrosis, anemia, splenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression and shortened survival. DIAGNOSIS: Diagnosis is based on bone marrow morphology...
December 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27870069/suspected-myelodysplastic-myeloproliferative-neoplasm-in-a-feline-leukemia-virus-negative-cat
#15
Amy L Weeden, Kyle R Taylor, Scott P Terrell, Alexander E Gallagher, Heather L Wamsley
A 10-year-old castrated Domestic Short-Haired cat was presented to a primary care veterinarian for a wellness examination and laboratory examination for monitoring of diabetes mellitus. The CBC revealed marked thrombocytosis, leukopenia and macrocytic, normochromic anemia. The cat tested negative for FeLV and feline immunodeficiency virus, but was positive for Mycoplasma haemominutum by PCR. Hematologic abnormalities were not responsive to therapy, so a repeat CBC and a bone marrow aspiration for cytology were performed...
November 21, 2016: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/27869179/interleukins-17-and-23-in-patients-with-gastric-neoplasms
#16
Wojciech Błogowski, Anna Madej-Michniewicz, Natalia Marczuk, Barbara Dołęgowska, Teresa Starzyńska
Recently there has been heightened interest in the potential significance of interleukin (IL)-17 and IL-23 in the development/progression of human malignancies. Here, we analyzed the systemic levels of these cytokines in 75 patients with different types of gastric neoplasms (carcinoma, gastrointestinal stromal tumors, neuroendocrine neoplasms, and lymphomas) and 42 healthy volunteers. We found that patients with all types of gastric neoplasms have significantly lower IL-23 levels. However, in comparison to the levels in healthy individuals, IL-17 concentrations were lower only in patients with types of gastric neoplasms other than carcinoma...
November 21, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27868231/aprepitant-in-pediatric-patients-using-moderate-and-highly-emetogenic-protocols-a-systematic-review-and-meta-analyses-of-randomized-controlled-trials
#17
Lucas Miyake Okumura, Fernanda D' Athayde Rodrigues, Maria Angelica Pires Ferreira, Leila Beltrami Moreira
AIMS: To review the efficacy and safety of aprepitant in combination with ondansetron and dexamethasone (triple therapy) in children and adolescents on moderate to highly emetogenic chemotherapy. METHODS: Medline, Embase, Scielo, Lilacs, Cochrane and congress abstracts published until September 2016 were used as data sources. Two reviewers independently selected manuscripts and extracted data. A third reviewer solved discrepancies in study's selection and data extraction...
November 20, 2016: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/27867823/the-burden-of-metastatic-disease-of-the-femur-on-the-medicare-system
#18
Kirollos Gendi, David Hennessy, John Heiner
BACKGROUND: In the United States, over 1,650,000 new cases of cancer are being diagnosed yearly with almost 50 % of them being the top five bone-seeking cancers. Since cancer risk increases with age, this suggests that orthopedic oncology services may be a strain on the Medicare system. The femur is the most common site of long bone metastases. Prophylactic fixation techniques prevent pathologic fractures, reduce morbidities, and enhance the quality of life of patients with femoral metastases...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27866579/polycythemia-and-thrombocytosis
#19
REVIEW
Aric Parnes, Arvind Ravi
Myeloproliferative neoplasms (MPNs) are diseases of excess cell proliferation from bone marrow precursors. Two classic MPNs, polycythemia vera (PV) and essential thrombocytosis (ET), are conditions of excess proliferation of red blood cells and platelets, respectively. Although PV and ET involve different cells in the myeloid lineage, their clinical presentations have shared features, consistent with overlapping mutations in growth factor signaling. The management of both diseases involves minimizing the risk of thrombotic and hemorrhagic complications...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27865804/ghost-cell-tumors
#20
Jason Sheikh, Molly D Cohen, Naomi Ramer, Ali Payami
Ghost cell tumors are a family of lesions that range in presentation from cyst to solid neoplasm and in behavior from benign to locally aggressive or metastatic. All are characterized by the presence of ameloblastic epithelium, ghost cells, and calcifications. This report presents the cases of a 14-year-old girl with a calcifying cystic odontogenic tumor (CCOT) and a 65-year-old woman with a peripheral dentinogenic ghost cell tumor (DGCT) with dysplastic changes, a rare locally invasive tumor of odontogenic epithelium...
October 26, 2016: Journal of Oral and Maxillofacial Surgery
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