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https://www.readbyqxmd.com/read/29774790/clear-cell-chondrosarcoma-with-chondroblastoma-like-features-a-case-for-team-diagnosis
#1
Ryan M Kiefer, John Wojcik, Kumarasen Cooper, Kristy L Weber, Ronnie Sebro
Clear cell chondrosarcoma (CCCS) is a rare variant of conventional chondrosarcoma with low-grade malignant features that may be confused radiographically and histologically with chondroblastoma. We report a case of a 50-year-old female who presented with 6 months of left hip pain. Initial radiographs demonstrated an osteolytic lesion with adjacent area of sclerosis in the proximal left femur. Magnetic resonance imaging demonstrated a marrow-infiltrative lesion with periosteal reaction and thickened enhancing periosteum...
May 1, 2018: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29772353/cardiac-profile-of-chimeric-antigen-receptor-car-t-cell-therapy-in-children-a-single-institution-experience
#2
Danielle Burstein, Shannon Maude, Stephen Grupp, Heather Griffis, Joseph Rossano, Kimberly Lin
BACKGROUND: Immunotherapy with chimeric antigen receptor (CAR)-modified T-cells targeting CD19 for pediatric acute lymphoblastic leukemia (ALL) has demonstrated significant efficacy. The principle toxicity is cytokine release syndrome with resultant hypotension. However, the spectrum of cardiovascular effects associated with CAR T-cell therapy has not been systematically evaluated. METHODS: We reviewed all patients who received CD19-directed CAR T-cells at the Children's Hospital of Philadelphia between April 2012 and September 2016...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29766466/concurrent-isolated-igg2-positive-membranous-nephropathy-and-malignant-b-cell-lymphoma
#3
Satoshi Shimada, Takashi Nakamichi, Gen Yamada, Kaori Narumi, Hajime Usubuchi, Tae Yamamoto, Satoshi Ichikawa, Noriko Fukuhara, Mariko Miyazaki, Hideo Harigae, Hiroshi Sato, Sadayoshi Ito
A recent systematic review showed that hematological malignancy is often complicated by membranous nephropathy (MN). Histologically, the deposition of IgG subclasses other than IgG4 may imply secondary MN, such as malignancy-associated MN (M-MN). We describe a very rare case of concurrent isolated IgG2-positive MN and B-cell lymphoma. An 83-year-old woman was hospitalized at our institute for facial and lower extremity edema persisting for 2 months. Laboratory tests showed urinary protein level of 10.8 g/day, serum albumin level of 1...
May 15, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29759150/comprehensive-characterization-of-circulating-and-bone-marrow-derived-multiple-myeloma-cells-at-minimal-residual-disease
#4
REVIEW
Johannes M Waldschmidt, Praveen Anand, Birgit Knoechel, Jens G Lohr
The presence or absence of minimal residual disease (MRD) in patients with multiple myeloma (MM) has emerged as a useful marker to determine the depth of remission. MRD negativity as an endpoint has been shown to be associated with improved progression-free survival in many studies. MRD detection is therefore part of numerous clinical trial protocols for MM. At the present time, two methodologies are most widely accepted for MRD detection: (1) multicolor flow cytometry and (2) next-generation sequencing-based clonotype detection...
January 2018: Seminars in Hematology
https://www.readbyqxmd.com/read/29759148/minimal-residual-disease-in-multiple-myeloma-use-of-magnetic-resonance-imaging
#5
REVIEW
Jens Hillengass, Maximilian Merz, Stefan Delorme
The increasing percentage of patients achieving deep responses in multiple myeloma has led to the need for more sophisticated instruments to measure residual disease as a potential source of relapse. As minimal residual disease assessment is mostly performed on a bone marrow specimen from a certain area of the body, such samples have the limitation that they might not really represent the actual tumor burden, because focal accumulations of malignant cells might be either hit or missed. Magnetic resonance imaging is a highly sensitive technique for the assessment of tumor burden and can be performed as whole-body protocol, overcoming the problem of sampling error for minimal residual disease assessment...
January 2018: Seminars in Hematology
https://www.readbyqxmd.com/read/29755847/-18-f-fdg-pet-ct-findings-in-hepatosplenic-gamma-delta-t-cell-lymphoma-case-reports-and-review-of-the-literature
#6
Michael W Cho, Bennett B Chin
Hepatosplenic Gamma Delta T cell lymphoma (γδHSTL) is a rare, highly aggressive, and rapidly lethal T cell lymphoma which manifests 18 F-FDG PET/CT findings that can mimic benign conditions. Patients with γδHSTL present with unexplained symptoms of a hematologic malignancy like the B symptoms of lymphoma including weight loss, fevers, and night sweats, as well as, splenomegaly and hepatomegaly. Thrombocytopenia, anemia, or neutropenia are also common due to spleen, liver and bone marrow involvement. The peripheral blood, however, typically does not show abnormal T cells...
2018: American Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29754839/-retrospective-study-of-25-cases-of-pulmonary-mucormycosis-in-acute-leukaemia
#7
REVIEW
D Caillot, C Legouge, I Lafon, E Ferrant, P B Pagès, A Plocque, L Estivalet, S Valot, F Dalle, H Abou Hanna, M-L Chretien
INTRODUCTION: In acute leukaemia (AL), the occurrence of pulmonary mucormycosis (PM), the incidence of which is increasing, as a result of chemotherapy induced marrow aplasia, remains a life threatening complication. METHODS: Analysis of clinical, biological and thoracic CT characteristics of patients with PM developing during the treatment of AL between 2000 and 2015. Day 0 (D0) was defined as the day with first CT evidence of PM. RESULTS: Among 1193 patients, 25 cases of PM were recorded during 2099 episodes of bone marrow aplasia...
May 10, 2018: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29743733/bone-marrow-uptake-of-18-f-fluorodeoxyglucose-in-hodgkin-lymphoma-without-bone-involvement-comparison-between-patients-with-and-without-b-symptoms
#8
Rômulo Hermeto Bueno do Vale, Daniela Andrade Ferraro, Paulo Schiavom Duarte, Giovana Carvalho, Marcos Santos Lima, George Barbério Coura Filho, Marcelo Tatit Sapienza, Carlos Alberto Buchpiguel
Objective: To compare the degree of benign bone marrow uptake of 18 F-fluorodeoxyglucose (18 F-FDG) between Hodgkin lymphoma patients with and without B symptoms. Materials and Methods: We analyzed the medical charts of 74 Hodgkin lymphoma patients who underwent 18 F-FDG positron emission tomography/computed tomography (PET/CT) prior to the initiation of therapy between October 2010 and September 2013. In all of the patients, the bone marrow biopsy was negative and the 18 F-FDG PET/CT images did not suggest bone marrow involvement...
March 2018: Radiologia Brasileira
https://www.readbyqxmd.com/read/29743401/-hypoplastic-acute-promyelocytic-leukemia-with-continuous-hypocellular-bone-marrow-after-remission
#9
Toshiki Nakamura, Junya Makiyama, Ayumi Matsuura, Hirokazu Kurohama, Hideaki Kitanosono, Masahiro Ito, Shinichiro Yoshida, Yasushi Miyazaki
An 87-year old female presented with unsteady gait and occasional subcutaneous hematomas. Blood examination findings revealed pancytopenia and mild coagulopathy. Both the histopathological evaluation of bone marrow smears and bone marrow biopsy revealed a hypocellular bone marrow. However, APL cells were observed and PML-RARA fusion gene was detected. On the basis of these findings, the patient was diagnosed with hypoplastic acute promyelocytic leukemia. She received ATRA treatment and achieved complete remission (CR) 29 days from the commencement of therapy...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29743399/-successful-treatment-of-x-linked-sideroblastic-anemia-with-alas2-r452h-mutation-using-vitamin-b-6
#10
Toru Kawakami, Hideyuki Nakazawa, Fumihiro Kawakami, Shuji Matsuzawa, Yuriko Sudo, Hitoshi Sakai, Sayaka Nishina, Noriko Senoo, Yasushi Senoo, Michiharu Komatsu, Takeji Umemura, Tomomi Yamaguchi, Tomoki Kosho, Tohru Fujiwara, Hideo Harigae, Fumihiro Ishida
A 45-year-old man presented with fatigue and pain in the finger joints. Despite having a history of suspected sideroblastic anemia since the age of 18 years, he had not been followed up for years. Upon presentation, laboratory data revealed microcytic anemia and elevated serum ferritin levels. In addition, ringed sideroblasts were increased in the bone marrow. A liver biopsy revealed hemochromatosis and cirrhosis. Furthermore, genetic analysis revealed that he harbored the ALAS2 R452H mutation, leading to the diagnosis of X-linked sideroblastic anemia (XLSA)...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29743397/-transplantation-associated-thrombotic-microangiopathy-confirmed-by-renal-biopsy
#11
Satoshi Miyamoto, Shunsuke Kimura, Yosuke Hosoya, Daisuke Hasegawa, Hisashi Ishida, Atsuro Daida, Toshihiro Matsui, Yuri Yoshimoto, Shinsuke Hirabayashi, Takuya Fujimaru, Tadashi Kumamoto, Shin-Ichiro Mori, Koyu Suzuki, Atsushi Manabe
An eight-year-old girl with myelodysplastic syndrome (refractory cytopenia) received a bone marrow transplant (BMT) from an unrelated donor because of immunosuppressive therapy failure. Following administration of foscarnet for cytomegalovirus reactivation at day40 post-BMT, serum creatinine increased, and proteinuria, hematuria, and hypertension gradually exacerbated and became prolonged. However, neither schistocytosis nor other organ damage was evident. At six months post-BMT, renal biopsy revealed diffuse glomerular damage with glomerular lobulation, a double contour of the glomerular basement membrane, erythrocyte congestion and thrombi in the glomerular endocapillaries, and mesangiolysis, confirming the diagnosis of transplantation-associated thrombotic microangiopathy (TA-TMA)...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29742693/a-successful-treatment-of-severe-systemic-lupus-erythematosus-caused-by-occult-pulmonary-infection-associated-with-hemophagocytic-syndrome-a-case-report
#12
Weihong Shi, Mingyang Duan, Ligang Jie, Weifeng Sun
RATIONALE: A 27-year-old woman with a history of systemic lupus erythaematosus (SLE) developed hemophagocytic syndrome (HPS) secondary due to an unrecognized infection that led to severe SLE with a prolonged recovery. PATIENT CONCERNS: The patient showed a high spiking fever and myalgia. Laboratory data revealed pancytopenia and immunological abnormalities. Pulse methylprednisone plus intravenous immunoglobulin (IVIG) failed to improve the clinical symptoms and laboratory data...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29741522/an-incidental-finding-of-severe-hyperferritinaemia-a-lesson-to-be-learned
#13
T B Fretwell, M Hanna
Haemophagocytic lymphohistiocytosis is a rare, under-recognised and often misdiagnosed condition, characterised by a hyperinflammatory response to malignancy or infection. In this case, the cause was a bone marrow isolated anaplastic large cell lymphoma without radiological evidence of systemic disease, a phenomenon rarely described. We present the case of a previously fit and well 64-year-old female who presented on multiple occasions to primary and secondary care in a stable condition with an undifferentiated illness with the only consistent feature being a marked, unexplained hyperferritinaemia...
March 2018: Journal of the Royal College of Physicians of Edinburgh
https://www.readbyqxmd.com/read/29739754/dexpramipexole-as-an-oral-steroid-sparing-agent-in-hypereosinophilic-syndromes
#14
Sandhya R Panch, Michael E Bozik, Thomas Brown, Michelle Makiya, Calman Prussin, Donald G Archibald, Gregory T Hebrank, Mary Sullivan, Xiaoping Sun, Lauren Wetzler, JeanAnne Ware, Michael P Fay, Cynthia E Dunbar, Steven I Dworetzky, Paneez Khoury, Irina Maric, Amy D Klion
Hypereosinophilic syndromes (HES) are a heterogeneous group of disorders characterized by peripheral eosinophilia and eosinophil-related end organ damage. Whereas most patients respond to glucocorticoid (GC) therapy, high doses are often necessary and side effects are common. Dexpramipexole (KNS 760704), an orally bioavailable synthetic amino-benzothiazole, showed an excellent safety profile and was coincidentally noted to significantly decrease absolute eosinophil counts (AEC) in a phase 3 trial for amyotrophic lateral sclerosis...
May 8, 2018: Blood
https://www.readbyqxmd.com/read/29739722/italian-multicenter-study-on-accuracy-of-18-f-fdg-pet-ct-in-assessing-bone-marrow-involvement-in-pediatric-hodgkin-lymphoma
#15
Angelina Cistaro, Laura Cassalia, Cinzia Ferrara, Natale Quartuccio, Laura Evangelista, Maurizio Bianchi, Franca Fagioli, Gianni Bisi, Sergio Baldari, Alessandro Zanella, Marta Pillon, Pietro Zucchetta, Marta Burei, Alessandra Sala, Luca Guerra, Priscilla Guglielmo, Roberta Burnelli, Stefano Panareo, Federica Scalorbi, Ilaria Rambaldi, Arnoldo Piccardo, Alberto Garaventa, Demetrio Familiari, Maria Concetta Fornito, Egesta Lopci, Maurizio Mascarin, Corinna Altini, Cristina Ferrari, Teresa Perillo, Nicola Santoro, Eugenio Borsatti, Giuseppe Rubini
INTRODUCTION: The present study investigated the utility of fluorine-18 (18 F) fluoro-2-deoxy-d-glucose (18 F-FDG) positron emission tomography/computed tomography (PET/CT) in assessing bone marrow involvement (BMI) compared with bone marrow biopsy (BMB) in newly diagnosed pediatric Hodgkin lymphoma (HL). PATIENTS AND METHODS: A total of 224 pediatric patients with HL underwent 18 F-FDG PET/CT at staging. BMB or follow-up imaging was used as the standard of reference for the evaluation of BMI...
April 14, 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29739109/a-proposed-kinetic-model-for-the-diagnostic-and-prognostic-value-of-wt1-and-p53-in-acute-myeloid-leukemia
#16
Mohammad A Bani-Ahmad, Suleimman A Al-Sweedan, Mohammad A Al-Asseiri, Ahed J Alkhatib
BACKGROUND: Wilms tumor (WT1) and p53 proteins were identified in the pathogenesis of several malignancies, including hematological malignancies. As a result of their interaction and diverse context-specific functions, this study aimed to emphasize the diagnostic and prognostic impacts of WT1 and p53 expression in acute myeloid leukemia (AML). METHODS: Twelve bone marrow (BM) biopsies were obtained from AML patients who were diagnosed in accordance with the French-American-British diagnostic criteria...
March 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29735492/asthmatic-adult-with-marked-blood-eosinophilia-is-it-truly-asthma
#17
Shera Tan, Angela Takano, Aloysius Ho, Keng Leong Tan
A middle-aged woman presented with symptoms suggestive of allergic asthma but with markedly elevated peripheral eosinophilia. She did not respond to inhaled corticosteroids, thereby prompting further investigations. Chest radiograph was normal. CT of the chest revealed bi-apical ground glass opacities. Bronchoalveolar lavage revealed predominantly eosinophilic yield. Autoimmune screen was negative. Bone marrow biopsy showed a normocellular marrow with increased eosinophils. A diagnosis of chronic eosinophilic pneumonia (CEP) was made after exclusion of other causes of eosinophilia...
May 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29732039/jak-2-mutation-frequency-in-patients-with-thrombocytosis
#18
Osman Yokus, Habip Gedik
Background: We aimed to investigate the etiologic causes and the existence of Janus kinase 2 mutation (JAK2) in cases with thrombocytosis. Methods: In this retrospective study, patients who were admitted to hematology clinic with thrombocytosis between 2013 and 2015 were investigated in terms of the etiological causes of thrombocytosis and the existence of JAK2 mutation. Results: We retrospectively evaluated 136 cases that underwent JAK2 mutation analysis due to ET preliminary diagnosis in our hematology clinic...
2018: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/29731799/beware-of-bone-marrow-incidental-detection-and-primary-diagnosis-of-solid-tumours-in-bone-marrow-aspiration-and-biopsies-a-study-of-22-cases
#19
Ruchita Tyagi, Aminder Singh, Bhavna Garg, Neena Sood
Background & objective: Introduction: First detection of any solid tumour as metastatic deposits in bone marrow directs clinicians to start searching for the primary tumour. Detection of bone marrow metastasis determines the stage of the malignancy, prognosis, mode of treatment, chemotherapeutic response and follow-up in case of relapse. The aim of the current study was to analyse the clinico-haematological presentation and morphological pattern of infiltration of solid tumours detected first as metastatic deposits on bone marrow examination...
2018: Iranian Journal of Pathology
https://www.readbyqxmd.com/read/29728434/focal-segmental-glomerulosclerosis-in-a-patient-with-prefibrotic-primary-myelofibrosis
#20
Gopal Krishana Bohra, Durga Shankar Meena, Nitin Bajpai, Abhishek Purohit
We report a case of 56-year-old man presented to us with chief complaints of frothy urine and leg swelling. A urinalysis revealed nephrotic-range proteinuria. Haematological investigations revealed thrombocytosis, leucocytosis and peripheral blood smear showed a leucoerythroblastic picture. JAK 2 mutation was positive. To confirm the diagnosis of myeloproliferative neoplasm, bone marrow biopsy was done, which was suggestive of primary myelofibrosis. The patient underwent kidney biopsy due to rapidly declining renal function and persistent proteinuria, which was suggestive of focal segmental glomerulosclerosis...
May 4, 2018: BMJ Case Reports
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