keyword
MENU ▼
Read by QxMD icon Read
search

Lymphoproliferative syndromes

keyword
https://www.readbyqxmd.com/read/28404814/nucleotide-binding-oligomerization-domain-nod-signaling-defects-and-cell-death-susceptibility-cannot-be-uncoupled-in-x-linked-inhibitor-of-apoptosis-xiap-driven-inflammatory-disease
#1
Steven M Chirieleison, Rebecca A Marsh, Prathna Kumar, Joseph K Rathkey, George R Dubyak, Derek W Abbott
The X-linked inhibitor of apoptosis (XIAP) protein has been identified as a key genetic driver of two distinct inflammatory disorders, X-linked lymphoproliferative syndrome 2 (XLP-2) and very early onset inflammatory bowel disease (VEO-IBD). Molecularly, the role of XIAP mutations in the pathogenesis of these disorders is unclear. Recent work has consistently shown XIAP to be critical for signaling downstream of the Crohns disease susceptibility protein nucleotide-binding oligomerization domain containing 2 (NOD2); however, the reported effects of XLP-2 and VEO-IBD XIAP mutations on cell death have been inconsistent...
April 12, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28400635/oral-and-cutaneous-lymphomas-other-than-mycosis-fungoides-and-s%C3%A3-zary-syndrome-in-a-mexican-cohort-recategorization-and-evaluation-of-international-geographical-disparities
#2
Amparo Hernández-Salazar, Jorge Andrés García-Vera, Yann Charli-Joseph, Guadalupe Ortiz-Pedroza, Silvia Méndez-Flores, Rocío Orozco-Topete, Ana Lilia Morales-Leyte, Judith Domínguez-Cherit, Carmen Lome-Maldonado
BACKGROUND: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. AIMS: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28400633/new-targeted-treatments-for-cutaneous-t-cell-lymphomas
#3
Martine Bagot
Cutaneous T-cell lymphomas (CTCLs) represent a group of rare and heterogeneous diseases that are very difficult to treat at advanced stages. The development of monoclonal antibodies is a new hope for the treatment of these diseases. Alemtuzumab (Campath) is a humanized IgG1 kappa monoclonal antibody specific for CD52, an antigen expressed by most T and B lymphocytes. Alemtuzumab may frequently induce long-term remissions in patients with Sezary syndrome but high-dose treatments lead to severe cytopenia, immune depletion, and opportunistic infections...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28395106/primary-congenital-immunodeficiency-2015-sh-eahp-workshop-report-part-5
#4
Dita Gratzinger, Elaine S Jaffe, Amy Chadburn, John K C Chan, Daphne de Jong, John R Goodlad, Jonathan Said, Yasodha Natkunam
Objectives: The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review primary immunodeficiency and related lymphoproliferations. Methods: Primary immunodeficiencies were divided into immune dysregulation, DNA repair defects, low immunoglobulins, and combined immunodeficiencies. Results: Autoimmune lymphoproliferative syndrome (ALPS) is a prototypical immune dysregulation-type immunodeficiency, with defects in T-cell signaling or apoptosis, expansion of T-cell subsets, and predisposition to hemophagocytic lymphohistiocytosis...
February 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28388253/lenalidomide-in-the-treatment-of-chronic-lymphocytic-leukemia
#5
Gilad Itchaki, Jennifer R Brown
Lenalidomide is an immunomodulatory drug (IMiD) with a unique mode of action (MOA) that may vary across disease-type. It is currently approved in multiple myeloma (MM), myelodysplastic syndrome (MDS) and mantle cell lymphoma (MCL), yet is also clinically active in a host of lymphoproliferative diseases, including chronic lymphocytic leukemia (CLL). Due to its protean effects on the immune system, lenalidomide may be particularly appealing in CLL, which is distinct in its ability to evade immune recognition and cause immunosuppression...
April 7, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28367854/development-of-allograft-cancer-after-lung-transplantation-a-case-report
#6
Esther I Schwarz, Bart Vrugt, Lars C Huber, Christian Murer, Mace M Schuurmans, Ilhan Inci, Christian Benden
INTRODUCTION: In contrast to skin cancer and lymphoproliferative disorders, de-novo lung allograft cancer is seldom reported after lung transplantation. CASE REPORT: A 19-year-old patient with severe pulmonary hypertension listed urgently for lung transplantation underwent successful bilateral lung transplant procedure receiving lungs from a 55-year-old donor with a smoking history of 30 pack years. After 3.5 years of lung transplantation, a locally advanced squamous cell carcinoma in the left lung allograft was diagnosed...
March 29, 2017: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28367597/salvage-therapy-with-lenalidomide-containing-regimen-for-relapsed-refractory-castleman-disease-a-report-of-three-cases
#7
Xinping Zhou, Juying Wei, Yinjun Lou, Gaixiang Xu, Min Yang, Hui Liu, Liping Mao, Hongyan Tong, Jie Jin
Castleman disease (CD) is uncommon non-clonal lymphoproliferative disorder with unknown etiology. No standard therapy is recommended for relapsed/refractory CD patients, thus requiring development of novel experimental approaches. Our cohort of three adult patients with multicentric CD (MCD) were treated with refractory to traditional chemotherapy lenalidomide-containing regimens (10-25 mg lenalidomide perorally administered on days 1-21 in 28-day cycle) as second- to fourth-line treatment. Partial remission was achieved in first plasma-cell CD patient, who relapsed seven months after autologous hematopoietic stem cell transplantation and then failed to respond to four cycles of chemotherapy...
April 3, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28367350/immune-reconstitution-inflammatory-syndrome-occurring-in-a-kidney-transplant-patient-with-extrapulmonary-tuberculosis
#8
Jose Iglesias, Kandria Jumil Ledesma, Paul J Couto, Jessie Liu
Tuberculosis (TB) occurring in solid organ transplantation (SOT) is associated with significant morbidity and mortality usually due to delays in diagnosis, drug toxicity encountered with antimycobacterial therapy, and drug-drug interactions. TB in SOT patients may mimic other infectious and noninfectious posttransplant complications such as posttransplant lymphoproliferative disorder (PTLD) and systemic cytomegalovirus infection. Immune reconstitution inflammatory syndrome (IRIS) is a host response resulting in paradoxical worsening of an infectious disease which occurs after the employment of effective therapy and reversal of an immunosuppressed state...
2017: Case Reports in Transplantation
https://www.readbyqxmd.com/read/28356696/hiv-associated-large-aggressive-extranodal-lymphoma-of-the-oral-cavity
#9
Rahul Dilip Kamat, Vikas Dhupar, Francis Akkara, Anita Dhupar
Malignant lymphomas form a heterogeneous group of neoplasms of the lymphoid tissue with different clinical courses, depending on the treatment and the prognosis. Human immunodeficiency virus (HIV) infection and acquired immune deficiency syndrome (AIDS) have been associated with an increased risk for the development of lymphoproliferative disorders. The incidence of lymphoma is associated with 60-fold increase in seropositive patients as compared to seronegative patients. The present case describes a diffuse aggressive extranodal lymphoma in a known HIV patient...
July 2016: National Journal of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28349581/tcf1-deficiency-ameliorates-autoimmune-lymphoproliferative-syndrome-alps-like-phenotypes-of-lpr-lpr-mice
#10
Xiaoxie Xu, Bolan Yu, Weibin Cai, Zhaofeng Huang
Autoimmune lymphoproliferative syndrome (ALPS) is an incurable disease, which is characterized by non-malignant autoimmune lymphoproliferation. TCF1 is a key effector in the canonical Wnt/β-catenin pathway, regulating the development, activation and function of T cells. In this study, we aimed to explore the potential role of TCF1 in the development of ALPS-like phenotypes of lpr/lpr mice. We acquired TCF1(-/-) lpr/lpr double mutant mice by crossing TCF1 deficiency mice with lpr/lpr mice. Splenocyte compositions, serum cytokines levels, anti-dsDNA antibody production and kidney pathology were examined in the TCF1(-/-) lpr/lpr mice...
March 28, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28331130/cytopenias-and-clonal-expansion-of-gamma-delta-t-cells-in-a-patient-with-anaplasmosis-a-potential-diagnostic-pitfall
#11
Daniel Marko, Anamarija M Perry, Arjuna Ponnampalam, Michel R Nasr
Human granulocytic anaplasmosis is a rare, tick-borne infectious disease caused by Anaplasma phagocytophilum. Herein, we report a rare case of human granulocytic anaplasmosis associated with cytopenias and clonal expansion of gamma/delta T-cells in the bone marrow. A 77-year old man presented multiple times to the emergency department complaining of muscle weakness. Complete blood count detected cytopenias and peripheral blood smear showed pseudo Pelger-Huet neutrophils. These findings prompted bone marrow evaluation with ancillary studies including flow cytometry, karyotyping and T-cell rearrangement studies...
2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/28293550/dock-8-deficiency-ebv-lymphomatoid-granulomatosis-and-intrafamilial-variation-in-presentation
#12
Victoria R Dimitriades, Vincent Devlin, Stefania Pittaluga, Helen C Su, Steven M Holland, Wyndham Wilson, Kieron Dunleavy, Nirali N Shah, Alexandra F Freeman
Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections. She had normal immunoglobulins with elevated IgE, poor polysaccharide response with low switched memory B cells, low CD4 count, and normal mitogen and antigen responses...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28288231/-primary-immunodeficiencies-in-seriously-ill-children-report-of-3-clinical-cases
#13
Leticia Yáñez, Pamela Lama, Carolina Rivacoba, Juanita Zamorano, María Angélica Marinovic
Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. OBJECTIVE: To present and discuss 3 infants diagnosed with PID. CLINICAL CASES: The cases are presented of three patients with PID diagnosed during their first admission to a Paediatric Intensive Critical Care Unit. The first patient, a 4-month-old infant affected by a severe pneumonia, and was diagnosed as a Severe Combined Immunodeficiency Disease...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28276715/inflammation-in-sj%C3%A3-gren-s-syndrome-cause-or-consequence
#14
Ana Raquel Rodrigues, Raquel Soares
Sjögren's syndrome (SS) is an autoimmune disease most commonly characterized by ocular and oral dryness. Despite the high prevalence of SS, generation and perpetuation of this disease is still unclear in many aspects. Inflammation, nonetheless, seems to play a central role in this pathology especially in the form of Th-1, Th-2 and Th-17 cytokines release within different aspects, concentrations and connections involved in the maintenance of the syndrome. Moreover, the chronically created pro-inflammatory environment appears to promote glandular atrophy and irreparable architectural modifications...
February 14, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28272287/risk-factors-and-outcomes-of-de-novo-cancers-excluding-nonmelanoma-skin-cancer-after-liver-transplantation-for-primary-sclerosing-cholangitis
#15
Mohamad A Mouchli, Siddharth Singh, Edward V Loftus, Lisa Boardman, Jayant Talwalkar, Charles B Rosen, Julie K Heimbach, Russell H Wiesner, Bashar Hasan, John J Poterucha, Kymberly D Watt
BACKGROUND: Patients with primary sclerosing cholangitis (PSC) may be at higher of risk of malignancy after liver transplantation (LT) compared to other LT recipients. We aimed to determine the cumulative incidence of/risk factors for and long-term cancer-related mortality in patients with PSC after LT. METHODS: All adult patients who underwent LT for PSC without cholangiocarcinoma from 1984-2012, with follow-up through June 2015. We estimated cumulative incidence, risk factors and mortality from de novo malignancies after LT RESULTS: 293 patients were identified (mean age, 47±12 years; 63...
March 8, 2017: Transplantation
https://www.readbyqxmd.com/read/28267077/novel-mutations-in-sh2d1a-gene-in-x-linked-lymphoproliferative-syndrome-diagnosed-after-b-cell-non-hodgkin-lymphoma
#16
Svetlana O Sharapova, Alina S Fedorova, Olga E Pashchenko, Svetlana S Vahliarskaya, Irina E Guryanova, Alexandr A Migas, Irina V Kondratenko, Olga V Aleinikova
BACKGROUND: X-linked lymphoproliferative disease type I (XLP I) is caused by mutations in the SH2D1A gene and characterized mainly by hypogammaglobulinemia and abnormal response to Epstein-Barr virus with a high predisposition to B-cell non-Hodgkin lymphoma development. OBSERVATIONS: In this article, we describe the experience of 2 centers in Belarus and in Russia that follow 3 male patients who were diagnosed with XLP I after lymphoma development and treatment...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28260482/late-diagnosis-of-e148q-mutation-positive-familial-mediterranean-fever-in-a-kidney-transplant-patient-with-fever-of-unknown-origin-a-case-report
#17
Erhan Tatar, Adam Uslu, Cenk Simsek, Ahmet Aykas, Giray Bozkaya, Cetin Imamoglu
Fever of unknown origin is a rare condition after solid organ transplant and is generally associated with atypical infections (eg, tuberculosis, fungal infections) and/or lymphoproliferative disorders. Here, we present a kidney transplant patient with a late diagnosis of E148Q mutation-positive familial Mediterranean fever as the cause of fever of unknown origin. A 22-year-old female patient with a previous history of 4 years of hemodialysis and unknown primary renal disease received a deceased-donor kidney transplant at our center 5 years previously...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28210942/how-we-treat-chronic-active-epstein-barr-virus-infection
#18
REVIEW
Akihisa Sawada, Masami Inoue, Keisei Kawa
Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years...
February 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28205564/elevated-serum-interferon-%C3%AE-induced-protein-10-kda-is-associated-with-tafro-syndrome
#19
Noriko Iwaki, Yuka Gion, Eisei Kondo, Mitsuhiro Kawano, Taro Masunari, Hiroshi Moro, Koji Nikkuni, Kazue Takai, Masao Hagihara, Yuko Hashimoto, Kenji Yokota, Masataka Okamoto, Shinji Nakao, Tadashi Yoshino, Yasuharu Sato
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28190860/activated-phosphoinositide-3-kinase-%C3%AE-syndrome-presenting-with-gut-associated-t-cell-lymphoproliferative-disease
#20
Hideto Teranishi, Masataka Ishimura, Yuuki Koga, Katsuhide Eguchi, Motoshi Sonoda, Tetsuko Kobayashi, Satoru Shiraishi, Kentaro Nakashima, Kouji Ikegami, Murasaki Aman, Hidetaka Yamamoto, Hidetoshi Takada, Shouichi Ohga
A 13-year-old boy was admitted to our hospital because of persistent diarrhea, abdominal pain, and bloody stools. The patient had experienced repeated hospitalizations for the treatment of respiratory infections since early childhood. Colonoscopic and pathological studies led to a diagnosis of gut-associated T-cell lymphoproliferative disease (T-cell LPD). Laboratory data showed T-lymphocytopenia (492/µl), increased serum IgG levels (1,984 mg/dl), and low serum antibody titers for specific pathogens. Combined immunodeficiency accompanied by T-LPD suggested the diagnosis of activated PI3Kδ syndrome (APDS)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
keyword
keyword
119112
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"