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https://www.readbyqxmd.com/read/28272212/ophthalmic-manifestations-in-igg4-related-disease-clinical-presentation-and-response-to-treatment-in-a-french-case-series
#1
Mikael Ebbo, Matthieu Patient, Aurelie Grados, Matthieu Groh, Julien Desblaches, Eric Hachulla, David Saadoun, Sylvain Audia, Aude Rigolet, Benjamin Terrier, Antoinette Perlat, Constance Guillaud, Frederic Renou, Emmanuelle Bernit, Nathalie Costedoat-Chalumeau, Jean-Robert Harlé, Nicolas Schleinitz
IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28253597/-the-effect-of-long-term-low-dose-prednisone-on-bone-mineral-density-in-patients-with-systemic-lupus-erythematosus
#2
G Wang, X P Li, X M Li, G S Wang, J H Tao, Y Ma
Objective: To investigate the effect of long-term low dose prednisone administration on bone mineral density (BMD) in patients with inactive systemic lupus erythematosus (SLE). Methods: A total of 118 inactive female SLE patients with long-term administration of low dose prednisone were recruited from the Department of Rheumatology and Immunology at An hui Provincial Hospital.All patients were given low dose prednisone for long-term (≤10 mg/d, more than half a year). According to prednisone doses, subjects were divided into two groups, namely group A (≤7...
March 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28249886/case-of-polyostotic-primary-bone-lymphoma-successfully-treated-with-immunochemotherapy-and-consolidation-radiotherapy
#3
Irfan Ahmad, Kundan Singh Chufal, Nidhi Goyal, Chandi Prasad Bhatt
A 47-year-old woman presented with symptoms of sharp pain over the left anterior thigh with radiation from the groin to the knee. She subsequently developed numbness in that region and reduced motor strength in extensors of the left knee. Plain radiography of the spine and knee was normal. An MRI of the spine revealed an irregular extramedullary mass with intradural and extradural components, extending from the L3 to L5 vertebrae. She underwent a laminectomy and posterior spinal decompression based on a working diagnosis of nerve sheath tumour...
March 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28239462/thrombocytopenia-in-patients-with-melanoma-receiving-immune-checkpoint-inhibitor-therapy
#4
Eileen Shiuan, Kathryn E Beckermann, Alpaslan Ozgun, Ciara Kelly, Meredith McKean, Jennifer McQuade, Mary Ann Thompson, Igor Puzanov, John P Greer, Suthee Rapisuwon, Michael Postow, Michael A Davies, Zeynep Eroglu, Douglas Johnson
BACKGROUND: Immune checkpoint inhibitors, including antibodies against programmed death 1 (PD-1) and cytotoxic T-lymphocyte antigen 4 (CTLA-4), are being used with increasing frequency for the treatment of cancer. Immune-related adverse events (irAEs) including colitis, dermatitis, and pneumonitis are well described, but less frequent events are now emerging with larger numbers of patients treated. Herein we describe the incidence and spectrum of thrombocytopenia following immune checkpoint inhibitor therapy and two severe cases of idiopathic thrombocytopenic purpura (ITP)...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28203178/a-patient-with-supraclavicular-lymphadenopathy-and-anterior-mediastinal-mass-presenting-as-a-rare-case-of-composite-lymphoma-a-case-report-and-literature-review
#5
Alex Raufi, James Jerkins, Yung Lyou, Deepa Jeyakumar
Composite lymphoma (CL) is a rare disease with 2 distinct lymphomas concurrently arising in a single patient with an estimated incidence of 1-4.7% of newly diagnosed lymphomas per year. CL most commonly involves 2 B-cell non-Hodgkin lymphomas (NHL) or a B-cell NHL with a Hodgkin lymphoma. Our case is unique in that it was a bilineage CL with both a T-cell and B-cell NHL, which has only been reported in a few case reports. A 49-year-old woman presented with several months of progressive cough, weight loss, dyspnea, and supraclavicular lymphadenopathy...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/28185911/transcriptome-analysis-of-monozygotic-twin-brothers-with-childhood-primary-myelofibrosis
#6
Nan Ding, Zhaojun Zhang, Wenyu Yang, Lan Ren, Yingchi Zhang, Jingliao Zhang, Zhanqi Li, Peihong Zhang, Xiaofan Zhu, Xiaojuan Chen, Xiangdong Fang
Primary myelofibrosis (PMF) is a chronic myeloproliferative disorder in human bone marrow. Over 50% of patients with myelofibrosis have mutations in JAK2, MPL, or CALR. However, these mutations are rarely detected in children, suggesting a difference in the pathogenesis of childhood PMF. In this study, we investigated the response to drug treatment of a monozygotic twin pair with typical childhood PMF. The twin exhibited different clinical outcomes despite following the same treatment regimen. The transcriptomic profiles of patient samples after drug treatment (E2 and Y2) were significantly different between the twin pair, which is consistent with the observation that the drug treatment was effective only in the younger brother, despite the twin being genetically identical...
February 2017: Genomics, Proteomics & Bioinformatics
https://www.readbyqxmd.com/read/28178153/case-report-malignant-transformation-in-cronkhite-canada-syndrome-polyp
#7
Ye Zong, Haiying Zhao, Li Yu, Ming Ji, Yongdong Wu, Shutian Zhang
RATIONALE: Cronkhite-Canada syndrome (CCS) is a rare disease, the etiology of CCS is currently unknown. Although CCS is widely accepted as a benign disorder, the malignant potential of the polyps in CCS patients is yet controversial. PATIENT CONCERNS: A 55-year-old Chinese male was first admitted to Beijing Friendship Hospital with a 3-month history of frequent watery diarrhea (10-15 times/day), loss of taste, and a weight loss of 10 kg in August 2010. The left heel bone fracture in the patient occurred about 2 weeks prior to his diarrhea...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28151917/chronic-diarrhea-associated-with-high-serum-level-of-immunoglobulin-a-and-diffuse-infiltration-of-plasma-cell-in-small-intestine-a-case-report
#8
Junwen Yang, Shuijiao Chen, Linlin Chen, Miao Ouyang, Fujun Li
RATIONALE: Chronic diarrhea in adult patients due to various causes is very common in clinic, but patient suffering with mal-absorption due to immunoproliferative small intestinal disease was rarely reported in China. PATIENT CONCERNS AND DIAGNOSES: A 35-year-old female presented with more than three years history of chronic diarrhea, rickets, high serum value of immunoglobulin A protein, and anemia. Bone marrow aspiration suggested that the patient was in a sideropenic and megalobastic anemia stage...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28122538/upregulation-of-the-proto-oncogene-bmi-1-predicts-a-poor-prognosis-in-pediatric-acute-lymphoblastic-leukemia
#9
Hong-Xia Peng, Xiao-Dan Liu, Zi-Yan Luo, Xiao-Hong Zhang, Xue-Qun Luo, Xiao Chen, Hua Jiang, Ling Xu
BACKGROUND: Bmi-1, the B cell-specific moloney murine leukemia virus insertion site 1, is a member of the Polycomb-group (PcG) family and acts as an oncogene in various tumors; however, its expression related to the prognosis of pediatric patients with acute lymphoblastic leukemia (ALL) has not been well studied. METHODS: The Bmi-1 expression levels in the bone marrow of 104 pediatric ALL patients and 18 normal control subjects were determined by using qRT-PCR. The association between the Bmi-1 expression and the clinicopathological characteristics of pediatric ALL patients was analyzed, and the correlation between Bmi-1 and the prognosis of pediatric ALL was calculated according to the Kaplan-Meier method...
January 25, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28101356/a-case-report-of-primary-cutaneous-natural-killer-t-cell-lymphoma
#10
Chen Tian, Yong Yu, Yizhuo Zhang
Primary cutaneous extranodal natural killer/T-cell lymphoma, nasal type (ENKL-NT) is a relatively rare disease associated with aggressive tumor-cell behavior and poor prognosis. Progress in immunohistochemistry has improved the identification of ENKL-NT. The present case study reported on a 64-year-old female patient presenting with several red nodular lesions on the neck developed over four months. Cutaneous biopsy revealed these cells were positive for CD3, CD56, CD5, CD8 and negative for CD2, CD34, CD7, CD20 and Granzyme B...
December 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28005758/pathological-mandibular-fracture-associated-with-diffuse-large-b-cell-lymphoma-in-hiv-positive-patient
#11
Marina Reis Oliveira, Darcy Fernandes, Marisa Aparecida Cabrini Gabrielli, Cleverton Roberto de Andrade, Cláudia Maria Navarro, Valfrido Antonio Pereira-Filho
This article describes the occurrence of diffuse large B-cell lymphoma in a 39-year-old human immunodeficiency virus-positive patient. The patient sought medical care complaining of increased volume in the right mandibular angle and imaging tests showed an extensive radiolucency with undefined boundaries compromising the mandibular border. After the incisional biopsy, the patient had a pathological fracture in the region, which was properly treated in a second surgical procedure using a 2.4-mm reconstruction plate...
November 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27987516/-non-hodgkin-s-lymphoma-mimicking-mikulicz-disease-a-case-report
#12
A C Liu, Y Chen, J S Jia, S Y Gao, Y Y Liu
IgG4-related disease is a systemic disorder involving a spectrum of multiple indications, and various histopathological features are shared among different IgG4-related disease subtypes, which challenge diagnosis, although certain syndromes have organ-specific involvement. Among them, Mikulicz's disease affecting the salivary and lacrimal glands, distinguished by often elevated levels of serum IgG4, infiltration of IgG4(+) plasma cells into target tissues, and diffuse swelling, mass formation, or fibrosis of affected organs...
December 18, 2016: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/27923552/preleukaemic-clonal-haemopoiesis-and-risk-of-therapy-related-myeloid-neoplasms-a-case-control-study
#13
Koichi Takahashi, Feng Wang, Hagop Kantarjian, Denaha Doss, Kanhav Khanna, Erika Thompson, Li Zhao, Keyur Patel, Sattva Neelapu, Curtis Gumbs, Carlos Bueso-Ramos, Courtney D DiNardo, Simona Colla, Farhad Ravandi, Jianhua Zhang, Xuelin Huang, Xifeng Wu, Felipe Samaniego, Guillermo Garcia-Manero, P Andrew Futreal
BACKGROUND: Therapy-related myeloid neoplasms are secondary malignancies that are often fatal, but their risk factors are not well understood. Evidence suggests that individuals with clonal haemopoiesis have increased risk of developing haematological malignancies. We aimed to identify whether patients with cancer who have clonal haemopoiesis are at an increased risk of developing therapy-related myeloid neoplasms. METHODS: We did this retrospective case-control study to compare the prevalence of clonal haemopoiesis between patients treated for cancer who later developed therapy-related myeloid neoplasms (cases) and patients who did not develop these neoplasms (controls)...
January 2017: Lancet Oncology
https://www.readbyqxmd.com/read/27893685/acute-liver-failure-caused-by-hemophagocytic-lymphohistiocytosis-in-adults-a-case-report-and-review-of-the-literature
#14
REVIEW
Shide Lin, Ying Li, Jun Long, Qichuan Liu, Fangwan Yang, Yihuai He
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as acute liver failure (ALF) has rarely been reported in adults. CASE SUMMARY: A 34-year-old man was admitted to our hospital with nausea and fatigue persisting for 2 weeks and jaundice for 1 week. He had hyperthermia at the onset of disease...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27891588/whole-body-mri-reveals-high-incidence-of-osteonecrosis-in-children-treated-for-hodgkin-lymphoma
#15
Annemieke S Littooij, Thomas C Kwee, Goya Enríquez, Jonathan I M L Verbeke, Claudio Granata, Auke Beishuizen, Charlotte de Lange, Floriana Zennaro, Marrie C A Bruin, Rutger A J Nievelstein
Osteonecrosis is a well-recognized complication in patients treated with corticosteroids. The incidence of osteonecrosis in children treated for Hodgkin lymphoma is unknown because prospective whole-body magnetic resonance imaging (MRI) studies are lacking in this patient population. Paediatric patients with newly diagnosed Hodgkin lymphoma who were treated according to a uniform paediatric Hodgkin protocol were eligible for inclusion in this prospective study. Whole-body MRI was performed in all 24 included patients (mean age 15·1 years, 12 girls) both before treatment and after 2 cycles of chemotherapy, and in 16 patients after completion of chemotherapy...
November 28, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27891382/a-case-of-immune-thrombocytopenic-purpura-secondary-to-pulmonary-tuberculosis
#16
Sameer Panda, Lalit Kumar Meher, Siba Prasad Dalai, Sachidananda Nayak, Sujit Kumar Tripathy
The haematological abnormalities associated with active pulmonary tuberculosis were known to human beings since decades but Immune Thrombocytopenic Purpura (ITP) secondary to pulmonary tuberculosis have been reported only in a couple of instances. We report a 27 year-old male patient who was admitted to our hospital with fever, shortness of breath, haematuria, epistaxis and generalized petechiae. The sputum positivity for Acid Fast Bacilli (AFB) and chest X-ray reports were suggestive of active pulmonary tuberculosis in our patient...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27878318/-corticosteroids-in-the-management-of-advanced-prostate-cancer
#17
H Kübler
Corticosteroids have been widely used for decades in cancer therapy, predominantly due to their anti-inflammatory activity. In the treatment of metastatic castration-resistant prostate cancer (mCRPC), corticosteroids play an important role both in the management of tumor-related symptoms, especially bone metastasis-related pain, and as concomitant treatment to counteract side effects associated with approved active prostatic anticancer agents such as docetaxel, cabazitaxel, and abiraterone acetate. In association with abiraterone acetate, low-dose corticosteroids (prednisone or prednisolone) reduce the mineralocorticoid side effects of abiraterone...
February 2017: Der Urologe. Ausg. A
https://www.readbyqxmd.com/read/27872411/osteoporosis-in-pediatric-liver-transplantation
#18
Walid Faraj, Ghina El Nounou, Abdallah Abou Al Naaj, Nancy Nakhoul, Ali Haydar, Mohammad Khalife
Liver transplantation provides an important, often life-saving treatment for end-stage liver disease. Osteoporosis post-liver transplantation has been described in adults; however, this has not been described in the pediatric population to date. We present a case of a 13-year-old female patient who underwent an orthotopic liver transplant for cryptogenic liver cirrhosis. Her immunosuppressants were tacrolimus and prednisone. Four months posttransplant, she started complaining of bilateral lower limb pain and limping while walking, progressing to a point where she was almost immobile...
December 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27866540/-the-study-of-the-eosinophil-cd34-progenitor-cells-differentiation-mechanism-of-model-rats-with-occupational-asthma-and-the-intervention-of-warm-and-tonifying-kidney-yang-decoction
#19
Z Y Hu, Y M Jia, S J Wang, J Y Han, T Yu
Objective: To investigate the mechanism of CD34(+) progenitor cell differentiation in rat by observing the change relations between the eosinophils (EOS) and the content of Eotaxin and IL-5 in blood and the CD34(+)/CCR3(+), CD34(+)/IL-5Rα+ in bone marrow after occupational asthma (OA) model rats are simulated, and to observe the effect of WTKYD Trraitional Chinese Medicine intervention. Methods: A total of 40 healthy male SD model rats (200~250 g weight) were randomly divided into model contrast Group, prednisone acetate intervention Group, WTKYD+1/2 prednisone acetate intervention Group and WTKYD intervention Group, 10 in each group, and set a Group for blank contrast...
September 20, 2016: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/27847518/adult-onset-still-s-disease-associated-with-mycoplasma-pneumoniae-infection-and-hemophagocytic-lymphohistiocytosis
#20
Abhishek Agnihotri, Allison Ruff, Lauren Gotterer, Addie Walker, Amy H McKenney, Andrei Brateanu
Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated...
2016: Case Reports in Medicine
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