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https://www.readbyqxmd.com/read/29468265/the-tridimensional-geometry-of-the-proximal-femur-should-determine-the-design-of-cementless-femoral-stem-in-total-hip-arthroplasty
#1
Julien Wegrzyn, Jean-Paul Roux, Charlotte Loriau, Nicolas Bonin, Vincent Pibarot
PURPOSE: Using a cementless femoral stem in total hip arthroplasty (THA), optimal filling of the proximal femoral metaphyseal volume (PFMV) and restoration of the extramedullary proximal femoral (PF) parameters (i.e., femoral offset (FO), neck length (FNL), and head height (FHH)) constitute key goals for optimal hip biomechanics, functional outcome, and THA survivorship. However, almost 30% of mismatch between the PF anatomy and implant geometry of the most widely implanted non-modular cementless femoral stem has been demonstrated in a computed tomography scan (CT scan) study...
February 22, 2018: International Orthopaedics
https://www.readbyqxmd.com/read/29464054/rapid-development-of-myeloproliferative-neoplasm-in-mice-with-ptpn11-d61y-mutation-and-haploinsufficient-for-dnmt3a
#2
Lisa Deng, Briana M Richine, Elizabeth L Virts, Victoria N Jideonwo-Auman, Rebecca J Chan, Reuben Kapur
PTPN11 gain-of-function mutation is the most common mutation found in patients with juvenile myelomonocytic leukemia and DNMT3A loss occurs in over 20% of acute myeloid leukemia patients. We studied the combined effect of both Ptpn11 gain-of-function mutation (D61Y) and Dnmt3a haploinsufficiency on mouse hematopoiesis, the presence of which has been described in both juvenile myelomonocytic leukemia and acute myeloid leukemia patients. Double mutant mice rapidly become moribund relative to any of the other genotypes, which is associated with enlargement of the spleen and an increase in white blood cell counts...
January 19, 2018: Oncotarget
https://www.readbyqxmd.com/read/29463311/myeloid-transformation-of-plasma-cell-myeloma-molecular-evidence-of-clonal-evolution-revealed-by-next-generation-sequencing
#3
Jonathon H Gralewski, Ginell R Post, Frits van Rhee, Youzhong Yuan
BACKGROUND: Plasma cell myeloma (PCM) is a neoplasm of terminally differentiated B lymphocytes with molecular heterogeneity. Although therapy-related myeloid neoplasms are common in plasma cell myeloma patients after chemotherapy, transdifferentiation of plasma cell myeloma into myeloid neoplasms has not been reported in literature. Here we report a very rare case of myeloid neoplasm transformed from plasma cell myeloma. CASE PRESENTATION: A 60-year-old man with a history of plasma cell myeloma with IGH-MAF gene rearrangement and RAS/RAF mutations developed multiple soft tissue lesions one year following melphalan-based chemotherapy and autologous stem cell transplant...
February 20, 2018: Diagnostic Pathology
https://www.readbyqxmd.com/read/29458388/a-novel-generation-1928zt2-car-t-cells-induce-remission-in-extramedullary-relapse-of-acute-lymphoblastic-leukemia
#4
Jianyu Weng, Peilong Lai, Le Qin, Yunxin Lai, Zhiwu Jiang, Chenwei Luo, Xin Huang, Suijing Wu, Dan Shao, Chengxin Deng, Lisi Huang, Zesheng Lu, Maohua Zhou, Lingji Zeng, Dongmei Chen, Yulian Wang, Xiaomei Chen, Suxia Geng, Weinkove Robert, Zhaoyang Tang, Chang He, Peng Li, Xin Du
BACKGROUND: Anti-CD19 chimeric antigen receptor (CAR) T cells have shown promise in the treatment of B cell acute lymphocytic leukemia (B-ALL). However, its efficacy in B-ALL patients with extramedullary involvement is limited due to poor responses and neurotoxicity. Here, we utilized a third generation of CAR T cell vector, which contains the Toll/interleukin-1 receptor (ITR) domain of Toll-like receptor 2 (TLR2), to generate 1928zT2 T cells targeting CD19, and evaluated the efficacy of 1928zT2 T cells in relapse or refractory B-ALL patients with extramedullary involvement...
February 20, 2018: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/29456361/upper-cervical-bronchogenic-cyst-a-rare-condition-at-a-rare-location
#5
Ranjeet Kumar Jha, Chandan B Mohanty, Chandrashekhar E Deopujari, Salman Tehran Shaikh
Intraspinal bronchogenic cyst (SBC) is a rare but important cause of spinal cord compression, commonly seen in the cervicothoracic spine. We report a case of a 43-year-old male, presenting with complaints of neck pain, radiating to right shoulder, with numbness of right hand and fingers. Magnetic resonance imaging of the cervical spine revealed an intradural extramedullary, ventral cystic lesion extending from C2 to C4 vertebral levels. Complete surgical excision was performed, and the patient had a complete relief of symptoms postoperatively...
January 2018: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29456358/surgically-treated-primary-spinal-cord-neoplasms-in-southeastern-nigeria
#6
Mark Chukwunweike Chikani, Okwuoma Okwunodulu, Mathew Mesi, Wilfred C Mezue, Samuel C Ohaegbulam, Chika C Ndubuisi
Background: Primary spinal cord tumors are not as frequently encountered as their cranial counterparts. They could present in such an indolent manner that requires a reasonable index of suspicion for their diagnosis to be considered. Objective: The objective of this study is to analyze the incidence and pattern of primary spinal cord and appendage neoplasms in patients surgically treated in our institution over a decade of practice. Materials and Methods: A retrospective review of clinical, radiological, and histopathology profiles of patients surgically treated for primary spinal cord tumor from 2006 to 2016 was carried out...
January 2018: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29455369/intradural-spinal-tumors-in-adults-update-on-management-and-outcome
#7
REVIEW
Malte Ottenhausen, Georgios Ntoulias, Imithri Bodhinayake, Finn-Hannes Ruppert, Stefan Schreiber, Annette Förschler, John A Boockvar, Andreas Jödicke
Among spinal tumors that occur intradurally, meningiomas, nerve sheath tumors, ependymomas, and astrocytomas are the most common. While a spinal MRI is the state of the art to diagnose intradural spinal tumors, in some cases CT scans, angiography, CSF analyses, and neurophysiological examination can be valuable. The management of these lesions depends not only on the histopathological diagnosis but also on the clinical presentation and the anatomical location, allowing either radical resection as with most extramedullary lesions or less invasive strategies as with intramedullary lesions...
February 17, 2018: Neurosurgical Review
https://www.readbyqxmd.com/read/29453884/daratumumab-resistance-is-frequent-in-advanced-stage-multiple-myeloma-patients-irrespectively-of-cd38-expression-and-is-related-to-dismal-prognosis
#8
M Pick, V Vainstein, N Goldschmidt, D Lavie, D Libster, A Gural, S Grisariu, B Avni, D Ben Yehuda, M E Gatt
OBJECTIVE: Daratumumab is a promising new anti-myeloma agent. We report a single center "real world" series of multiple myeloma (MM) and amyloidosis (AL) patients treated with daratumumab. METHODS: Forty-one patients were included: 7 second line MM, 30 heavily pretreated (median number of therapies of 5) advanced MM, and 4 with AL. RESULTS: Second line patients and advanced AL showed high rate of durable overall responses. However, advanced MM patients had a dismal prognosis with an ORR of 36%, and a short median progression free and overall survival of 2...
February 17, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29451228/a-persistent-cough-as-atypical-clinical-presentation-of-intrathoracic-extramedullary-hematopoiesis-emh-in-a-female-with-thalassemia-intermedia
#9
Mohammad Aj Abdulla, Mohamed A Yassin, Mohamed Abdelrazek, Deena Mudawi, Firyal Ibrahim, Dina S Soliman, Halima ElOmri, Abdulqadir J Nashwan, Liam Fernihough, Vincenzo De Sanctis, Ashraf T Soliman
Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside the bone marrow or peripheral blood. The most common sites of EMH are liver and spleen, but it has been documented in other organs such as the mediastinum, lymph nodes, breast, and central nervous system. EMH occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. We report a case of EMH presenting as a posterior mediastinal mass in a 34-year-old woman with thalassemia intermedia with chronic cough and shortness of breath on exertion...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29446979/synovial-sarcoma-mimicking-a-thoracic-dumbell-schwannoma-a-case-report
#10
Susanth Subramanian, Gandham Edmond Jonathan, Bimal Patel, Krishna Prabhu
INTRODUCTION: Synovial sarcoma is a rare mesenchymal malignant neoplasm that accounts for less than 10% of soft tissue sarcomas. About 95% of the sarcomas occur in the extremities. Primary synovial sarcomas of the spine are a rare tumor arising from the paravertebral regions, paraspinal muscles or epidural spaces. PURPOSE: To report an atypical radiological presentation of synovial sarcoma of the thoracic spine mimicking a nerve sheath tumor in an elderly adult and describe the management with review of the literature...
February 15, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29433801/risk-factors-of-fixation-failure-in-basicervical-femoral-neck-fracture-which-device-is-optimal-for-fixation
#11
Young-Kyun Lee, Byung-Ho Yoon, Ji Sup Hwang, Yong-Han Cha, Ki-Choul Kim, Kyung-Hoi Koo
BACKGROUND: Basicervical femur neck fracture (FNF) is a rare type of fracture, and is associated with increased risk of fixation failure due to its inherent instability. The purpose of this study was (1) to investigate the incidence of fixation failure and (2) to determine risk factors for fixation failure in basicervical FNF after internal fixation. METHODS: To identify basicervical FNF with a minimum of 12 months follow-up, we retrospectively reviewed records of 3217 patients who underwent hip fracture surgery from May 2003 to March 2016...
February 9, 2018: Injury
https://www.readbyqxmd.com/read/29433178/radiological-evaluation-of-510-cases-of-basilar-invagination-with-evidence-of-atlantoaxial-instability-group-a-basilar-invagination
#12
Atul Goel, Sonal Jain, Abhidha Shah
OBJECTIVE: To evaluate the musculoskeletal and soft tissue neural alterations in cases with group A basilar invagination. METHODS: Between January 2007 and August 2016, 510 patients with group A basilar invagination were surgically treated. The radiologic images of these patients were reviewed retrospectively. The patients were divided into 4 groups: group A1, comprising 60 patients with syringomyelia; group A2, comprising 354 patients with "external syrinx," marked by excessive cerebrospinal fluid (CSF) in the extramedullary space; group A3, comprising 51 patients with both syringomyelia and external syrinx; and group A4, comprising 45 patients with no abnormality of CSF cavitation in the spinal canal...
February 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29423036/salvaged-allogeneic-hematopoietic-stem-cell-transplantation-for-pediatric-chemotherapy-refractory-acute-leukemia
#13
Jingbo Wang, Lei Yuan, Haoyu Cheng, Xinhong Fei, Yumin Yin, Jiangying Gu, Song Xue, Junbao He, Fan Yang, Xiaocan Wang, Yixin Yang, Weijie Zhang
There is an ongoing debate concerning the performance of salvaged allogeneic hematopoietic stem cell transplantation (allo-HSCT) in pediatric patients with acute refractory leukemia, in whom the prognosis is quite dismal. Few studies have ever been conducted on this subject. This may be partly due to missed opportunities by majority of the patients in such situations. To investigate the feasibility, evaluate the efficiency, and identify the prognostic factors of allo-HSCT in this sub-setting, the authors performed a single institution-based retrospective analysis...
January 9, 2018: Oncotarget
https://www.readbyqxmd.com/read/29422310/multifocal-spinal-cord-nephroblastoma-in-a-dog
#14
L C Henker, R M Bianchi, T P Vargas, E C de Oliveira, D Driemeier, S P Pavarini
A 1-year-old male American pit bull terrier was presented with a history of proprioceptive deficits and mild lameness of the right hindlimb, which progressed after 5 months to paraparesis, culminating in tetraparesis after 2 weeks. Necropsy findings were limited to the spinal cord and consisted of multiple, intradural, extramedullary, slightly red masses which produced segmental areas of medullary swelling located in the cervical intumescence, thoracolumbar column, sacral segment and cauda equina. Histological evaluation revealed a tumour, composed of epithelial, stromal and blastemal cells, with structures resembling tubules, acini and embryonic glomeruli...
January 2018: Journal of Comparative Pathology
https://www.readbyqxmd.com/read/29419433/impact-of-extramedullary-disease-in-patients-with-newly-diagnosed-multiple-myeloma-undergoing-autologous-stem-cell-transplantation-a-study-from-the-chronic-malignancies-working-party-of-the-ebmt
#15
Nico Gagelmann, Diderik-Jan Eikema, Simona Iacobelli, Linda Koster, Hareth Nahi, Anne-Marie Stoppa, Tamás Masszi, Denis Caillot, Stig Lenhoff, Miklos Udvardy, Charles Crawley, William Arcese, Clara Mariette, Ann Hunter, Xavier Leleu, Martin Schipperus, Michel Delforge, Pietro Pioltelli, John A Snowden, Maija Itälä-Remes, Maurizio Musso, Anja van Biezen, Laurent Garderet, Nicolaus Kröger
We investigated extramedullary disease in newly diagnosed multiple myeloma patients and its impact on outcome following first line autologous stem cell transplantation. We identified 3744 adult myeloma patients who received upfront single (n = 3391) or tandem transplantation (n = 353) between 2005 and 2014 with available data on extramedullary in-volvement at diagnosis. The overall incidence of extramedullary disease was 18.2% (n = 682) and increased per year from 6.5% (2005) to 23.7% (2014). Paraskeletal involvement was found in 543 (14...
February 1, 2018: Haematologica
https://www.readbyqxmd.com/read/29415939/-acute-leukemia-recurring-as-extramedullary-tumors-of-the-ovary-following-allogeneic-hematopoietic-stem-cell-transplantation
#16
Ryouta Imanaka, Yuta Katayama, Koji Iwato, Kouhei Kyo, Takeshi Okatani, Mitsuhiro Itagaki, Shinya Katsutani, Hideki Asaoku
The poor prognosis of extramedullary recurrence of acute leukemia following allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains a therapeutic challenge, and thus far no effective treatment method has been established. Here, we report two patients who presented with relapsed leukemia as extramedullary tumor in the ovary following allo-HSCT. Case 1: A 23-year-old female underwent unrelated allogeneic bone marrow transplantation during the second remission of acute myeloid leukemia. After 706 days post-transplant, bilateral ovarian tumors were detected during the pelvic ultrasound, and extramedullary recurrence in the bilateral ovaries was subsequently established on right salpingo-oophorectomy and biopsy of the left ovary...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29403257/idiopathic-hypertrophic-pachymeningitis-as-a-rare-cause-of-cervical-compressive-myelopathy
#17
Aya Fukuda, Elizabete Punaro, Fábio Rogério, Luciano de Souza Queiroz, Fabiano Reis
The spinal form of idiopathic hypertrophic pachymeningitis (IHP) is a rare condition characterized by a chronic progressive diffuse inflammatory fibrosis of the dura mater, which may evolve to the compression of the spinal cord. We present a case report about IHP focusing on its features in magnetic resonance imaging, which are determined by an intradural extramedullary mass in the cervical spine showing hypointensity on T2-weighted images and peripheral enhancement, causing compression of the spinal cord. Histological analysis showed a nonspecific chronic inflammatory process in dense fibrous tissue...
October 2017: Journal of Craniovertebral Junction and Spine
https://www.readbyqxmd.com/read/29403081/hepatotoxicity-of-immune-checkpoint-inhibitors-a-histology-study-of-seven-cases-in-comparison-with-autoimmune-hepatitis-and-idiosyncratic-drug-induced-liver-injury
#18
Yoh Zen, Matthew M Yeh
The adverse effects of immune checkpoint inhibitors in various organs may be attributed to immune-mediated processes triggered by disrupted self-tolerance; however, it remains unclear whether they are similar or dissimilar to classic organ-specific autoimmune diseases. The present study aimed to compare clinicopathologic features between checkpoint inhibitor-induced liver injury and acutely presenting autoimmune hepatitis or idiosyncratic drug-induced liver injury. Seven patients treated with nivolumab (n = 5) or ipilimumab (n = 2) presented with liver dysfunction a median of 41 days (range 21-120) after the initiation of immunotherapy...
February 5, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29401143/false-positive-18f-fdg-pet-ct-due-to-filgrastim-that-induced-extramedullary-liver-hematopoiesis-in-a-burkitt-lymphoma
#19
Anna Margherita Maffione, Lucia Rampin, Paolini Rossella, Sara Pecori, Patrick M Colletti, Domenico Rubello
We report a case of Burkitt lymphoma with largely extranodal disease localizations at staging. Chemotherapy was given, thus obtaining a complete metabolic response in all previous disease sites as shown at a control PET, however associated to the appearance of new focal uptake areas in the liver; these findings were confirmed at US and MRI. Chemotherapy determined also neutropenia that was treated by filgrastim, followed by a prompt and important medullary response. Liver biopsy revealed extramedullary hematopoiesis, probably filgrastim induced...
February 3, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29399415/melanoma-induced-immunosuppression-is-mediated-by-hematopoietic-dysregulation
#20
Neha Kamran, Youping Li, Maria Sierra, Mahmoud S Alghamri, Padma Kadiyala, Henry D Appelman, Marta Edwards, Pedro R Lowenstein, Maria G Castro
Tumors are associated with expansion of immunosuppressive cells such as tumor associated macrophages (TAMs), regulatory T cells (Tregs) and myeloid derived suppressor cells (MDSCs). These cells promote tumor growth, angiogenesis, metastasis and immune escape. Cancer patients frequently present symptoms such as anemia, leukocytosis and/or cytopenia; associated with poor prognosis. To uncover tumor-mediated hematopoietic abnormalities and identify novel targets that can be harnessed to improve tumor-specific immune responses, we investigated the hematopoietic stem and progenitor cell compartment in melanoma bearing mice...
2018: Oncoimmunology
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