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https://www.readbyqxmd.com/read/28543448/successful-treatment-of-pure-red-cell-aplasia-because-of-abo-major-mismatched-stem-cell-transplant
#1
Katie Sackett, Claudia S Cohn, Kayla Fahey-Ahrndt, Angela R Smith, Andrew D Johnson
BACKGROUND: Pure red cell aplasia (PRCA) is a well-documented potential side effect of ABO major mismatched allogeneic hematopoietic stem cell transplants. This side effect may be self-limiting, but is sometimes treated using modalities such as steroids, antithymocyte globulin, donor lymphocyte infusions, rituximab, or plasma exchanges. Another well-documented cause of pure red cell aplasia is a chronic parvovirus B19 infection, which may be seen in immunocompromised hosts. The treatment of this cause of PRCA includes removal of immunosuppression, intravenous immunoglobulin (IVIg), or rituximab; however, this condition may also be self-limiting...
May 24, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28541003/effect-of-early-plasma-exchange-on-survival-in-patients-with-severe-fever-with-thrombocytopenia-syndrome-a-multicenter-study
#2
Won Sup Oh, Jeong Rae Yoo, Ki Tae Kwon, Hye In Kim, Su Jin Lee, Jae Bum Jun, Seong Yeol Ryu, Hyun Ah Kim, Jian Hur, Yu Mi Wi, Min Hee Lim, Sang Taek Heo
Despite a high mortality rate, no specific treatment for severe fever with thrombocytopenia syndrome (SFTS) has been established. This study compared the clinical outcomes of SFTS patients treated with plasma exchange (PE group) with those who were not treated (non-PE group) at nine Korean hospitals between May 2013 and August 2015. A total of 53 SFTS patients were included: 24 (45.3%) PE cases and 29 (54.7%) non-PE cases. The overall in-hospital mortality rate was 32.1% (17/53). The in-hospital mortality rate of the PE group did not differ from that of the non-PE group (29...
July 2017: Yonsei Medical Journal
https://www.readbyqxmd.com/read/28538511/successful-treatment-of-transplantation-associated-atypical-hemolytic-uremic-syndrome-with-eculizumab
#3
Daiichiro Hasegawa, Atsuro Saito, Nanako Nino, Suguru Uemura, Satoru Takafuji, Takehito Yokoi, Aiko Kozaki, Toshiaki Ishida, Keiichiro Kawasaki, Takahiro Yasumi, Naoki Sakata, Yasufumi Ohtsuka, Satoshi Hirase, Takeshi Mori, Noriyuki Nishimira, Mayumi Kusumoto, Yoshiharu Ogawa, Kenta Tominaga, Taku Nakagawa, Kyoko Kanda, Ryojiro Tanaka, Yoshiyuki Kosaka
We herein reported a 4-month-old boy with transplantation-associated atypical hemolytic uremic syndrome (TA-aHUS) who was successfully treated with eculizumab. The patient diagnosed with type 3 of familial hemophagocytic lymphohistiocytosis underwent cord blood transplantation. After transplantation, he developed TA-aHUS, but plasma exchanges were unsuccessful. We identified deletions in CFH-related gene 1 (del-CFHR1) by the multiplex ligation-dependent probe amplification testing procedure and CFH autoantibodies...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28535517/coupled-plasma-filtration-adsorption-application-for-liver-and-thyroid-toxins
#4
Gabriele Donati, Irene Capelli, Anna Laura Croci Chiocchini, Nicolò Natali, Anna Scrivo, Gaetano La Manna
Coupled plasma filtration and adsorption (CPFA) is a detoxification system that combines a plasma adsorption circuit and a continuous renal replacement therapy circuit. Its main application is for sepsis and septic shock with or without acute renal failure. Several recent studies have suggested that CPFA can reduce the mortality when the volume of plasma absorbed on the styrenic resin is at least >0.18 L/kg/day. At present, new applications for CPFA are under investigation, also in patients without significant kidney failure...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#5
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28516871/diagnosis-of-catastrophic-anti-phospholipid-syndrome-in-a-patient-tested-negative-for-conventional-tests
#6
Fabrizio Conti, Roberta Priori, Cristiano Alessandri, Roberta Misasi, Antonella Capozzi, Monica Pendolino, Simona Truglia, Silvia Frisenda, Maurizio Sorice, Guido Valesini
Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of aPL (lupus anticoagulant and/or anticardiolipin antibodies and/or anti-Beta2-glcyoprotein I antibodies). Here we report a case of a 39-year-old woman patient who developed a CAPS which was negative to the conventional aPL but positive for aPL in thin layer chromatography immunostaining and vimentin/cardiolipin antibodies by ELISA test...
May 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28515608/thrombotic-thrombocytopenic-purpura-in-a-case-of-dengue-fever-a-rare-presentation
#7
Abhijit S Gavali, Jayant Shelgaonkar, Sandip Bartakke
Here, we present an unusual occurrence of thrombotic thrombocytopenic purpura (TTP) in a case of dengue fever. Both the conditions are fatal and can result in significant mortality and morbidity if left untreated. In this case, as soon as, we diagnosed the patient as having TTP, we treated her with plasma exchange therapy, steroids, and monoclonal antibodies such as rituximab. The patient responded very well to the treatment and completely recovered from neurological symptoms and laboratory parameters also normalized...
April 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28515156/anti-glomerular-basement-membrane-disease
#8
Stephen P McAdoo, Charles D Pusey
Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals...
May 17, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28514704/guillain-barr%C3%A3-syndrome-variants-forms-fruste-reclassification-with-new-criteria
#9
Fu Liong Hiew, Rahmansah Ramlan, Shanthi Viswanathan, Santhi Puvanarajah
OBJECTIVES: This study aimed to evaluate the clinical and electrophysiological characteristics of various distinctive classical and localised Guillain-Barré syndrome (GBS) subtypes. PATIENTS AND METHODS: Clinical characteristics and electrophysiological data of sixty-one consecutive patients admitted between 2012 and 2015 were systematically analysed and reclassified according to the new GBS clinical classification. Neurophysiology was evaluated with Hadden et al...
May 4, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28512098/a-rare-case-of-thrombotic-microangiopathy-triggered-by-acute-pancreatitis
#10
Kevin Singh, Ahmed Jamal Nadeem, Behzad Doratotaj
Thrombotic microangiopathy (TMA) occurring after acute pancreatitis is rarely described. Without prompt intervention, TMA can be, and often is, lethal, so prompt recognition is important. Here, we present a case of a 61-year-old woman with a history of alcohol misuse who presented with epigastric pain, nausea and vomiting after binge drinking. Elevated serum lipase and imaging were suggestive of acute-on-chronic pancreatitis. Although the patient's symptoms of acute pancreatitis subsided, her anaemia, thrombocytopenia and acute kidney injury worsened...
May 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#11
Noriaki Sato, Hideki Yokoi, Hirotaka Imamaki, Eiichiro Uchino, Kaoru Sakai, Takeshi Matsubara, Tatsuo Tsukamoto, Sachiko Minamiguchi, Motoko Yanagita
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509118/anti-glomerular-basement-membrane-disease-accompanied-by-systemic-lupus-erythematosus-presenting-central-nervous-system-involvement
#12
Hirohito Sugawara, Hideki Takizawa, Yoshinosuke Shimamura, Norihito Moniwa, Koichi Hasegawa, Yayoi Ogawa
We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (GBM) disease accompanied by systemic lupus erythematosus (SLE) presenting central nervous system involvement in a 32-year-old Japanese male. He was admitted to our hospital because of a 3-week history of fever and rapidly failing renal function requiring hemodialysis (HD). Laboratory tests showed anti-GBM antibody elevation with a value of 16,385 units/ml. On day 85, he had generalized tonic-clonic seizure...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508984/successful-treatment-of-myeloma-cast-nephropathy-using-bortezomib-based-chemotherapy-plus-selective-plasma-exchange
#13
Mayuko Kawabe, Izumi Yamamoto, Ai Katsuma, Naomi Hayashi, Yo Komatsuzaki, Yasuyuki Nakada, Akihiro Shimizu, Yudo Tanno, Ichiro Ohkido, Nobuo Tsuboi, Kazuhito Suzuki, Takaki Shimada, Yoji Ogasawara, Katsuki Sugiyama, Keisuke Aiba, Takashi Yokoo
Myeloma cast nephropathy is a major complication of multiple myeloma. Recent evidence has demonstrated that the earlier induction of bortezomib-based chemotherapy with plasma exchange (PE) provides better results for kidney function and patient survival. Due to its non-selectivity, PE with albumin replacement carries the risk of fibrinogen loss, leading to bleeding. We herein report a case of successful treatment of myeloma cast nephropathy using bortezomib-based chemotherapy and selective PE. A 61-year-old woman who had a 20-year history of type II diabetes mellitus was admitted to our hospital for the evaluation of hypercalcemia, severe kidney dysfunction, and anemia...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508980/an-adult-case-of-severe-steroid-resistant-henoch-sch%C3%A3-nlein-purpura-nephritis-treated-with-intravenous-cyclophosphamide-and-tonsillectomy
#14
Emi Sasaki, Maki Shibata, Asami Kato, Naoto Hamano, Takashi Katsuki, Manami Tada, Fumihiko Hinoshita
A previously healthy 37-year-old Canadian man living in Japan visited a hospital in Thailand while traveling because of edematous legs, purpura, arthralgia, bloody stool, and fever after an insect bite. Henoch-Schönlein purpura (HSP) was suspected. His creatinine level was 5.2 mg/dL. He was treated with oral prednisolone (PSL) and oral cyclophosphamide (CPA); after treatment, his creatinine level improved to 2.4 mg/dL. Upon returning to Japan, he was admitted to the National Center for Global Health and Medicine Hospital in Tokyo...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#15
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#16
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28500622/implementation-of-a-rapid-assay-of-adamts13-activity-was-associated-with-improved-30-day-survival-rate-in-patients-with-acquired-primary-thrombotic-thrombocytopenic-purpura-who-received-platelet-transfusions
#17
Yumi Yoshii, Yoshihiro Fujimura, Charles L Bennett, Ayami Isonishi, Norio Kurumatani, Masanori Matsumoto
BACKGROUND: Platelet (PLT) transfusions are probably harmful in patients with acquired idiopathic thrombotic thrombocytopenic purpura (aTTP). Introduction of a rapid assay for ADAMTS13 activity should reduce the time to definite diagnosis of aTTP, reduce the amount of inappropriately transfused PLT concentrates, and improve mortality and morbidity. STUDY DESIGN AND METHODS: We selected 265 aTTP patients with severe ADAMTS13 deficiency. Of these, 91 patients were diagnosed by March 2005 (Period 1), when ADAMTS13 activity was measured by von Willebrand factor multimer assay, which took 4 to 7 days until the result was reported...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28499586/-gemcitabine-induced-thrombotic-microangiopathy-can-we%C3%A2-improve-screening-and%C3%A2-treatment
#18
Xavier Charmetant, Anne Jolivot, Thomas Fournier, Jean-Charles Puthet, Philippe Cassier, Sandrine Lemoine, Laurent Juillard
Thrombotic microangiopathy is a rare but severe complication of treatment with gemcitabine. Its prevalence increases because gemcitabine's indications are growing. We report four cases, which presented with common clinical and biological manifestations, i.e. high blood pressure, proteinuria and increasing plasmatic creatinine level. However, severity was not similar, hemodialysis was inconstant. There is no consensus on treatment for this condition. Stopping gemcitabine is essential. Treatment was dispensed considering the severity of the presentation: plasma exchange therapy of variable outcome, and eculizumab, which was efficient when used...
May 9, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28495143/central-and-peripheral-nervous-system-immune-mediated-demyelinating-disease-after-allogeneic-hematopoietic-stem-cell-transplantation
#19
M I Stefanou, F Bischof
OBJECTIVE: We aimed to evaluate clinical and diagnostic features of central and peripheral immune-mediated demyelinating disease (CPID) in allogeneic hematopoietic stem cell transplantation (aHSCT) recipients. BACKGROUND: CPID refers to the late-onset, immune-mediated neurological complications following aHSCT, when other frequent differential diagnoses have been ruled out, and when symptoms and signs of systemic GvHD manifestations are absent. METHODS: Case records at the University of Tuebingen, between 2001 and 2015, were screened to identify patients with CPID after aHSCT...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28495137/early-predictors-of-rapidly-evolving-multiple-sclerosis-a-case-report
#20
Kryshani T M Fernando, Michael James
Objective The aim of this case report is to highlight some important features of rapidly evolving Multiple Sclerosis. BACKGROUND: In a small proportion of patients, Multiple Sclerosis (MS) can present as a fulminant disease characterised by severe and frequent relapses. This form of rapidly evolving MS is associated with significant morbidity and mortality. It is therefore important to identify these patients as early as possible, so that they can be managed effectively. However, due to the rarity of fulminant forms of MS, there is limited data on the natural history and management of this condition...
June 15, 2017: Journal of Neuroimmunology
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