keyword
MENU ▼
Read by QxMD icon Read
search

Lymphoproliferative syndromes

keyword
https://www.readbyqxmd.com/read/27918349/soft-palate-ulcer-an-unusual-presentation-of-a-posttransplant-lymphoproliferative-disorder
#1
Maryam Bahmanyar, Nader Shakibazad, Mohammad Hadi Imanieh, Seyed Mohsen Dehghani
Posttransplant lymphoproliferative disorder (PTLD) is a serious complication of solid organ transplantation. It has several clinical manifestations, including infectious mononucleosis-like syndrome, lymphadenopathy, and other organ involvement. Soft palate ulcer is a rare manifestation of PTLD. The aim of this study is to present a 17-year-old girl with liver transplantation due to metastatic liver disease. After transplantation, she presented with a painful whitish ulcerative lesion accompanied by central necrosis in the right soft palate area measuring about 3 cm×3...
December 1, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27913462/pure-red-cell-aplasia
#2
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27902471/autophagy-autophagy-associated-adaptive-immune-responses-and-its-role-in-hematologic-malignancies
#3
REVIEW
Liangshun You, Shenhe Jin, Li Zhu, Wenbin Qian
Autophagy is a tightly regulated catabolic process that leads to the degradation of cytoplasmatic components such as aggregated/misfolded proteins and organelles through the lysosomal machinery. Recent studies suggest that autophagy plays such a role in the context of the anti-tumor immune response, make it an attractive target for cancer immunotherapy. Defective autophagy in hematopoietic stem cells may contribute to the development of hematologic malignancies, including leukemia, myelodysplastic syndrome, and lymphoproliferative disorder...
November 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27888883/transformed-lymphoma
#4
REVIEW
Mary Ann Anderson, Piers Blombery, John F Seymour
Transformed lymphoma is a complex syndrome that encompasses an array of different underlying low-grade lymphoproliferative conditions transforming into more aggressive disease as manifest by morphologic, clinical, and genetic features. Over the last decade, advances in chemoimmunotherapy have led to new options. Knowledge surrounding the genetic changes driving the process of transformation is leading to novel targeted therapies. This article focuses on the transformation of chronic lymphocytic leukemia and follicular lymphoma into diffuse large B-cell lymphoma...
December 2016: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/27885340/acute-kidney-injury-in-hematopoietic-stem-cell-transplantation-a-review
#5
REVIEW
Vinod Krishnappa, Mohit Gupta, Gurusidda Manu, Shivani Kwatra, Osei-Tutu Owusu, Rupesh Raina
Hematopoietic stem cell transplantation (HSCT) is a highly effective treatment strategy for lymphoproliferative disorders and bone marrow failure states including aplastic anemia and thalassemia. However, its use has been limited by the increased treatment related complications, including acute kidney injury (AKI) with an incidence ranging from 20% to 73%. AKI after HSCT has been associated with an increased risk of mortality. The incidence of AKI reported in recipients of myeloablative allogeneic transplant is considerably higher in comparison to other subclasses mainly due to use of cyclosporine and development of graft-versus-host disease (GVHD) in allogeneic groups...
2016: International Journal of Nephrology
https://www.readbyqxmd.com/read/27881371/pure-red-cell-aplasia
#6
REVIEW
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#7
REVIEW
Saritha Kartan, Vivian Y Shi, Ashley K Clark, Lawrence S Chan
Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm...
November 22, 2016: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27873163/an-update-on-the-use-of-immunomodulators-in-primary-immunodeficiencies
#8
REVIEW
Pandiarajan Vignesh, Amit Rawat, Surjit Singh
The genomic revolution in the past decade fuelled by breathtaking advances in sequencing technologies has defined several new genetic diseases of the immune system. Many of these newly characterized diseases are a result of defects in genes involved in immune regulation. The discovery of these diseases has opened a vista of new therapeutic possibilities. Immunomodulatory agents, a hitherto unexplored therapeutic option in primary immunodeficiency diseases have been tried in a host of these newly described maladies...
November 21, 2016: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/27856754/mouse-model-for-acute-epstein-barr-virus-infection
#9
Tristan Wirtz, Timm Weber, Sven Kracker, Thomas Sommermann, Klaus Rajewsky, Tomoharu Yasuda
Epstein-Barr Virus (EBV) infects human B cells and drives them into continuous proliferation. Two key viral factors in this process are the latent membrane proteins LMP1 and LMP2A, which mimic constitutively activated CD40 receptor and B-cell receptor signaling, respectively. EBV-infected B cells elicit a powerful T-cell response that clears the infected B cells and leads to life-long immunity. Insufficient immune surveillance of EBV-infected B cells causes life-threatening lymphoproliferative disorders, including mostly germinal center (GC)-derived B-cell lymphomas...
November 29, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27852053/therapy-related-acute-myeloid-leukemia-following-treatment-of-lymphoid-malignancies
#10
Sarah Bertoli, Arthur Sterin, Suzanne Tavitian, Lucie Oberic, Loïc Ysebaert, Reda Bouabdallah, François Vergez, Audrey Sarry, Emilie Bérard, Françoise Huguet, Guy Laurent, Thomas Prébet, Norbert Vey, Christian Récher
Therapy-related acute myeloid leukemia (t-AML) is a heterogeneous entity most frequently related to breast cancer or lymphoproliferative diseases (LD). Population-based studies have reported an increased risk of t-AML after treatment of lymphomas. The aim of this study was to describe the characteristics and outcome of 80 consecutive cases of t-AML following treatment of LD. t-AML accounted for 2.3% of all AML cases, occurred 60 months after LD diagnosis, and were characterized by a high frequency of FAB M6 AML and poor-risk cytogenetic abnormalities...
November 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27848183/an-atypical-case-of-late-onset-systemic-lupus-erythematosus-with-systemic-lymphadenopathy-and-severe-autoimmune-thrombocytopenia-neutropenia-mimicking-malignant-lymphoma
#11
Keita Tamaki, Satoko Morishima, Sawako Nakachi, Sakiko Kitamura, Sachie Uchibori, Shouhei Tomori, Taeko Hanashiro, Natsuki Shimabukuro, Iori Tedokon, Kazuho Morichika, Yukiko Nishi, Takeaki Tomoyose, Kennosuke Karube, Takuya Fukushima, Hiroaki Masuzaki
Here, we report a rare case of systemic lupus erythematosus (SLE) with conspicuous manifestation of hematological abnormalities. At onset, the 52-year-old male patient showed systemic lymphadenopathy and splenomegaly, severe autoimmune thrombocytopenia, and autoimmune neutropenia. Bone marrow examination and lymph node biopsy excluded the possibility of malignant lymphoma. Based on laboratory findings, he was finally diagnosed with combined autoimmune cytopenia coupled with SLE. Atypical clinical manifestations of SLE prompted us to explore the possibility of autoimmune lymphoproliferative syndrome (ALPS)...
November 15, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27846610/bone-marrow-findings-in-autoimmune-lymphoproliferative-syndrome-with-germline-fas-mutation
#12
Yi Xie, Stefania Pittaluga, Susan Price, Mark Raffeld, Jamie Hahn, Elaine S Jaffe, V Koneti Rao, Irina Maric
Autoimmune lymphoproliferative syndrome is rare genetic disorder characterized by defective FAS-mediated apoptosis, autoimmune disease, accumulation of mature T-cell receptor alpha/beta positive, CD4 and CD8 double-negative T cells and increased risk for lymphoma. Despite frequent hematological abnormalities, literature is scarce regarding the bone marrow pathology in autoimmune lymphoproliferative syndrome. We retrospectively reviewed 3l bone marrow biopsies from a cohort of 240 patients with germline FAS mutation...
October 20, 2016: Haematologica
https://www.readbyqxmd.com/read/27845235/haploinsufficiency-of-tnfaip3-a20-by-germline-mutation-is-involved-in-autoimmune-lymphoproliferative-syndrome
#13
Masatoshi Takagi, Shohei Ogata, Hiroo Ueno, Kenichi Yoshida, Tzuwen Yeh, Akihiro Hoshino, Jinhua Piao, Motoy Yamashita, Mai Nanya, Tsubasa Okano, Michiko Kajiwara, Hirokazu Kanegane, Hideki Muramatsu, Yusuke Okuno, Yuichi Shiraishi, Kenichi Chiba, Hiroko Tanaka, Yuki Bando, Motohiro Kato, Yasuhide Hayashi, Satoru Miyano, Kohsuke Imai, Seishi Ogawa, Seiji Kojima, Tomohiro Morio
BACKGROUND: Autoimmune diseases in children are rare and can be difficult to diagnose. Autoimmune lymphoproliferative syndrome (ALPS) is a well-characterized pediatric autoimmune disease caused by mutations in genes associated with the FAS-dependent apoptosis pathway. In addition, various genetic alterations are associated with the ALPS-like phenotype. OBJECTIVE: The aim of the present study was to elucidate the genetic etiology of the ALPS-like phenotype. METHODS: Candidate genes associated with the ALPS-like phenotype were screened by whole exome sequencing...
November 11, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27837196/abnormal-thymic-maturation-and-lymphoproliferation-in-mrl-faslpr-lpr-mice-can-be-partially-reversed-by-synthetic-oligonucleotides-implications-for-systemic-lupus-erythematosus-and-autoimmune-lymphoproliferative-syndrome
#14
R F Ashman, N Singh, P S Lenert
MRL-Fas (lpr/lpr) mice represent an excellent animal model for studying non-malignant lymphoproliferation, regeneration and systemic autoimmunity. Retro-transposon insertion into the second intron of the pro-apoptotic Fas gene appears to be responsible for both lymphoproliferation and autoimmunity, while other genes are more likely to contribute to the regenerative healing characteristic of this mouse strain. Previous studies have shown that neonatal thymectomy can halt the development of abnormal lymphoproliferation...
November 10, 2016: Lupus
https://www.readbyqxmd.com/read/27829440/wrn-targeted-therapy-using-inhibitors-nsc-19630-and-nsc-617145-induce-apoptosis-in-htlv-1-transformed-adult-t-cell-leukemia-cells
#15
R Moles, X T Bai, H Chaib-Mezrag, C Nicot
BACKGROUND: Human T-cell leukemia virus type 1 (HTLV-1) infection is associated with adult T-cell leukemia/lymphoma (ATLL), a lymphoproliferative malignancy with a dismal prognosis and limited therapeutic options. Recent evidence shows that HTLV-1-transformed cells present defects in both DNA replication and DNA repair, suggesting that these cells might be particularly sensitive to treatment with a small helicase inhibitor. Because the "Werner syndrome ATP-dependent helicase" encoded by the WRN gene plays important roles in both cellular proliferation and DNA repair, we hypothesized that inhibition of WRN activity could be used as a new strategy to target ATLL cells...
November 9, 2016: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/27818812/cns-vasculitis-associated-with-waldenstr%C3%A3-m-macroglobulinemia
#16
Tanawan Riangwiwat, Chris Y Wu, Alberto S Santos-Ocampo, Randal J Liu, Aaron M McMurtray, Beau K Nakamoto
Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27812262/membranoproliferative-glomerulonephritis-secondary-to-a-low-grade-lymphoproliferative-disorder-a-rare-cause-of-renal-dysfunction
#17
Tushar Sehgal, Nidhi Jain, Gaurav Prakash, Ritambhra Duseja, Neelam Varma
Chronic lymphocytic leukemia is a B-cell neoplasm with an indolent clinical course. Most patients are asymptomatic and are diagnosed incidentally on a routine blood count. The malignant cells of this low-grade neoplasm infiltrate various organs and tissues. However, the resultant end organ damage is a rare phenomenon. Here we describe a case of chronic lymphocytic leukemia that presented with an unusual cause of acute renal dysfunction. The patient had deranged renal parameters with a nephrotic range proteinuria...
December 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27799292/sumo-modified-fadd-recruits-cytosolic-drp1-and-caspase-10-to-mitochondria-for-regulated-necrosis
#18
Seon-Guk Choi, Hyunjoo Kim, Eun Il Jeong, Sungwoo Park, Song-Yi Lee, Hyeon-Jeong Lee, Seong Won Lee, Chin Ha Chung, Yong-Keun Jung
Fas-associated protein with Death Domain (FADD) plays a key role in extrinsic apoptosis. Herein, we show that FADD is SUMOylated as an essential step during intrinsic necrosis. FADD was modified at multiple lysine residues (K120/125/149) by Small Ubiquitin-related Modifier 2 (SUMO2) during necrosis caused by calcium ionophore A23187 and by ischemic damage. SUMOylated FADD bound to dynamin-related protein 1 (Drp1) in cells both in vitro and in ischemic tissue damage cores, thus promoting Drp1 recruitment by Mitochondrial fission factor (Mff) to accomplish mitochondrial fragmentation...
October 31, 2016: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/27796825/complete-response-to-post-transplant-lymphoproliferative-disorder-by-surgical-resection-and-rituximab-after-living-donor-liver-re-transplantation-for-recurrent-primary-sclerosing-cholangitis
#19
Koichiro Haruki, Hiroaki Shiba, Junichi Shimada, Norimitsu Okui, Tomonori Iida, Katsuhiko Yanaga
Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication of solid organ transplantation. We herein report a case of PTLD after living-donor liver re-transplantation (reLDLT) for recurrent primary sclerosing cholangitis (PSC), for which complete response was achieved by surgical resection and rituximab. A 47-year-old man, who had undergone living-donor liver transplantation (LDLT) twice at age of 43 and 45 years for end-stage liver disease firstly for PSC and secondary for recurrent PSC, suffered liver dysfunction due to an acute cellular rejection (ACR) 17 months after reLDLT...
October 31, 2016: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/27790959/igg4-related-syndrome-another-multiorgan-disease-in-the-interest-field-of-internal-medicine
#20
Antonino Tuttolomondo, Irene Simonetta, Domenico Di Raimondo, Tiziana Di Chiara, Roberta Conigliaro, Francesca Corpora, Valerio Vassallo, Antonio Pinto
BACKGROUND: IgG4-related disease is a rare, clinical and pathologic disease entity of unknown etiology. Its main features are increased serum concentrations of IgG4 > 1,35 g/l, lymphocyte and IgG4+plasma-cell infiltration within tissues, fibrosis or sclerosis. The classical presentation of IgG4-RSD is pancreatitis which is combined with the involvement of biliary ducts in 74 percent of patients. Extrapancreatic manifestations include: abdominal or mediastinal lymphadenopathy; the involvement of salivary glands and lacrimal glands, kidneys, lung, retroperitoneum...
2016: Current Pharmaceutical Design
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"