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https://www.readbyqxmd.com/read/28509137/successful-treatment-by-tocilizumab-without-steroid-in-a-very-severe-case-of-tafro-syndrome
#1
Tamami Fujiki, Suguru Hirasawa, Seishi Watanabe, Shunsuke Iwamoto, Ryoichi Ando
Successful use of tocilizumab (TCZ) to treat TAFRO syndrome has recently been reported. In those cases, TCZ was used with steroid. We present herein the case of a 59-year-old man with very severe TAFRO syndrome who was successfully treated using TCZ without steroid. He showed rapidly progressive anasarca, acute renal failure and very severe thrombocytopenia. We initially used steroid, but its efficacy was limited. Moreover, steroid use had to be stopped as soon as possible, because hemorrhagic shock developed due to severe duodenal ulcer...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28502946/two-cases-of-thrombocytopenia-anasarca-fever-reticulin-fibrosis-renal-failure-and-organomegaly-tafro-syndrome-with-high-serum-procalcitonin-levels-including-the-first-case-complicated-with-adrenal-hemorrhaging
#2
Mizuho Nara, Atsushi Komatsuda, Fumiko Itoh, Hajime Kaga, Masaya Saitoh, Masaru Togashi, Yoshihiro Kameoka, Hideki Wakui, Naoto Takahashi
Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28491220/a-primary-intestinal-lymphangiectasia-hiding-the-diagnosis-of-pleural-and-pericardial-tuberculosis-a-clinical-observation
#3
Sanaa Hammi, Hajar Berrani, Thami Benouchen, Naima Lamlami, Imane Elkhiyat, Jamal Eddine Bourkadi
Primary intestinal lymphangiectasia (Waldmann's disease) is an exudative enteropathy characterized by lymph leakage into the small bowel lumen leading to hypoalbuminemia, hypogammaglobulinemia and lymphopenia (particularly T-cell). The diagnosis is based on viewing the duodenal lymphangiectasia. A 20 years old female patient, treated for a primary intestinal lymphangiectasia, has consulted for anasarca. Etiological work-up reveals pleural and pericardial tuberculosis. The clinical aggravation of an enteropathy, particularly in adulthood, requires a search for a secondary etiology...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28465984/uncommon-cardiac-manifestations-of-left-sided-pseudomonas-endocarditis-in-an-intravenous-drug-abuser-with-an-undiagnosed-atrial-septal-defect
#4
Prashanth Panduranga, Seif Al-Abri, Mamatha Punjee Rajarao
A 56-year-old male, who is an active intravenous drug abuser (IVDA) (heroin) with a history of diabetes, hypertension, chronic kidney disease, and hepatitis C-related liver cirrhosis, presented with generalized anasarca, bilateral pneumonic infiltrations, and heart failure. His blood cultures were positive for Pseudomonas aeruginosa and were treated with antibiotics. Echocardiogram showed multiple uncommon manifestations of left-sided endocarditis. Surprisingly, he did not have right-sided involvement. Furthermore, echocardiogram revealed undiagnosed large atrial septal defect suggesting a paradoxical seeding of infective vegetation...
January 2017: Journal of Cardiovascular Echography
https://www.readbyqxmd.com/read/28353560/a-life-threatening-case-of-tafro-syndrome-with-dramatic-response-to-tocilizumab-rituximab-and-pulse-steroids-the-first-case-report-in-latin-america
#5
Fabio Freire José, Lucila Nassif Kerbauy, Guilherme Fleury Perini, Danielle Isadora Blumenschein, Denise da Cunha Pasqualin, Denise Maria Avancini Costa Malheiros, Guilherme de Carvalho Campos Neto, Fabio Pires de Souza Santos, Ronaldo Piovesan, Nelson Hamerschlak
RATIONALE: This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America. PATIENT CONCERNS: The patient was a 61-year-old white woman of Ashkenazi Jewish descent, who presented with a history of 8 days of nausea, vomiting, and fever; severe pitting edema in both legs, ascites, splenomegaly, and palpable axillary lymph nodes. DIAGNOSES: Abdominal computed tomography (CT) showed bilateral pleural effusion and retroperitoneal lymph node enlargement...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28274535/two-patients-with-tafro-syndrome-exhibiting-strikingly-similar-anterior-mediastinal-lesions-with-predominantly-fat-attenuation-on-chest-computed-tomography
#6
Yoko Ozawa, Hiroshi Yamamoto, Masanori Yasuo, Hidekazu Takahashi, Kazunari Tateishi, Atsuhito Ushiki, Satoshi Kawakami, Yasunari Fujinaga, Shiho Asaka, Kenji Sano, Hiroshi Takayama, Hiroshi Imamura, Masayuki Hanaoka
We herein report on two middle-aged men with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis or renal failure, and organomegaly) syndrome, a unique clinicopathological variant of multicentric Castleman׳s disease recently proposed in Japan. Strikingly similar anterior mediastinal fat swellings with soft tissue density were observed in the patients on chest computed tomography. In TAFRO syndrome, bilateral pleural effusion and slight lymph node swelling are common in the thoracic region; however, anterior mediastinal lesions have not been previously observed...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28205564/elevated-serum-interferon-%C3%AE-induced-protein-10-kda-is-associated-with-tafro-syndrome
#7
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/28135567/tafro-syndrome-new-subtype-of-idiopathic-multicentric-castleman-disease
#8
REVIEW
Gordan Srkalovic, Inga Marijanovic, Maya B Srkalovic, David C Fajgenbaum
Castleman disease (CD) describes a group of three rare and poorly understood lymphoproliferative disorders that have heterogeneous clinical symptoms and common lymph node histopathological features. Unicentric CD (UCD) involves a single region of enlarged nodes. Multicentric CD (MCD) involves multiple regions of enlarged lymph nodes, constitutional symptoms, and organ dysfunction due to a cytokine storm often including interleukin 6. MCD is further divided into Human Herpes Virus-8 (HHV-8)-associated MCD, which occurs in immunocompromised individuals, and HHV-8-negative/idiopathic MCD (iMCD)...
January 26, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28097080/immunoglobulin-m-nephropathy-in-a-patient-with-wilson-s-disease
#9
Zain Ul Abideen, Zoya Sajjad, Asna Haroon Khan, Nadira Mamoon, Muhammad Bilal, Khaja Hameeduddin Mujtaba Quadri
Immunoglobulin M nephropathy (IgMN) is characterized by the deposition of immunoglobulin M in a dominant distribution in the renal glomeruli. Primary immunoglobulin M nephropathy is diagnosed after consistent light microscopy (LM), immunofluorescence (IF), electron microscopy (EM) results, and exclusion of known systemic disorders causing immunoglobulin M deposition in the glomeruli. The secondary disease has been reported with a few conditions though it has never been reported with any primary disease of the liver...
December 13, 2016: Curēus
https://www.readbyqxmd.com/read/28012935/idiopathic-systemic-capillary-leak-syndrome-clarkson-disease
#10
Kirk M Druey, Samir M Parikh
In 1960, Dr Bayard Clarkson described a woman experiencing sporadic recurrent episodes of shock and anasarca. Plasma from an acute attack induced a shock-like syndrome when injected into rats. The enigmatic systemic capillary leak syndrome (SCLS) named for Dr Clarkson is characterized by transient and severe but reversible hemoconcentration and hypoalbuminemia caused by leakage of fluids and macromolecules into tissues. Although less than 500 cases of SCLS have been reported in the literature since 1960, the condition is probably underdiagnosed because of a lack of awareness and a high mortality without treatment...
December 22, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28001175/nodular-glomerulosclerosis-in-a-non-diabetic-hypertensive-dyslipidemic-smoker-patient-a-case-report
#11
Liliane Silvano Araújo, Alessandro Alves Queiroz, Maria Luíza Reis Monteiro, Crislaine Aparecida Silva, Lívia Helena de Morais Pereira, Mariana Molinar Mauad Cintra, Claudia Renata Bibiano Borges, Juliana Reis Machado, Marlene Antônia Dos Reis
INTRODUCTION: This is a case report of a patient with idiopathic nodular glomerulosclerosis whose pathogenesis and morphology are similar to diabetic nephropathy. CASE PRESENTATION: A 64-year-old Brazilian man, leukoderma, dyslipidemic, obese with chronic obstructive pulmonary disease secondary to tobacco smoking, known to be hypertensive for five years and he had no history of diabetes. He was admitted with sudden anasarca, rapid loss of renal function and needed to start hemodialysis immediately...
December 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/27864846/monochorial-diamniotic-dizygotic-twins-in-a-german-shepherd-dog-a-case-report
#12
C Urhausen, K Wolf, A Beineke, C Dierks, M Schmicke, A Einspanier, A R Günzel-Apel
A 6.5-year-old clinically healthy German Shepherd Dog with regular oestrous cycles of 6 months was presented for pregnancy diagnosis on day 38 after ovulation (p.ov.). Ultrasonography revealed three individual placental sites in progressed resorption and two vital adequately developed foetuses sharing a joint placenta. On days 41 and 48 p.ov., sonographic signs indicated normal development of both foetuses, but on day 52 p.ov., both foetuses were found to be dead. A caesarean section was performed the same day...
November 18, 2016: Reproduction in Domestic Animals, Zuchthygiene
https://www.readbyqxmd.com/read/27825275/magnetic-resonance-imaging-of-bone-marrow-for-tafro-syndrome
#13
Gen Nakamura, Noriyuki Homma, Akio Kasai, Takuya Kasami, Kunihiko Makino, Youhei Aoki, Kunihiko Wakaki, Norihito Nakagawa
We report two cases of TAFRO syndrome, which is characterized by thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly. Magnetic resonance imaging (MRI) of the spine showed a dark medullary pattern in the bone marrow on the T1- and T2-weighted images of both patients. One patient showed complete resolution after treatment. Serial MRIs of the improved patient revealed a transition to a normal marrow pattern on both images, which might represent resolution of the disease.
November 9, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27795511/proposed-diagnostic-criteria-disease-severity-classification-and-treatment-strategy-for-a-novel-disorder-tafro-syndrome
#14
Yasufumi Masaki, Hiroshi Kawabata, Kazue Takai, Norifumi Tsukamoto, Shino Fujimoto, Yasuhito Ishigaki, Nozomu Kurose, Masaru Kojima, Shigeo Nakamura, Tomohiro Kinoshita, Sadao Aoki
TAFRO syndrome is a systemic inflammatory disorder manifesting as thrombocytopenia; anasarca including pleural effusion and ascites; fever; renal insufficiency; and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology remains unknown. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of Castleman's disease, other specific features can differentiate between the two. Some patients have been successfully treated with glucocorticoids and/or immunosuppressants including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, eventually succumbing to the disease...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27795506/tafro-syndrome-with-primary-sjogren-s-syndrome
#15
Nozomi Iwanaga, Kohei Harada, Yoshika Tsuji, Chieko Kawahara, Kazuhiro Kurohama, Yasumori Izumi, Shinichiro Yoshida, Keita Fujikawa, Masahiro Ito, Atsushi Kawakami, Kiyoshi Migita
  A 25-year-old woman diagnosed 1 year earlier with Primary Sjogren's syndrome was admitted to a nearby hospital with fever of unknown origin. Examination revealed anasarca, systemic lymphadenopathy, hepatosplenomegaly and high C-reactive protein level. The patient's symptoms were initially suspected to be caused by severe bacterial infection with Sjogren's syndrome flare. She was given antibiotics and prednisolone (PSL) at 50 mg/day. However, the patient developed anemia and thrombocytopenia and was transferred to our hospital for further care...
2016: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27777802/tafro-syndrome-associated-with-ebv-and-successful-triple-therapy-treatment-case-report-and-review-of-the-literature
#16
Malorie Simons, Emmanuel Apor, James N Butera, Diana O Treaba
TAFRO syndrome is a rare constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly. Its pathogenesis involves an excessive and inappropriate cytokine storm, most notably from IL-6, causing multiorgan failure; however, its etiology is undetermined. Starting in 2012, TAFRO syndrome was first identified in Japan as an atypical variant of Castleman's disease. Previous reports include various different treatment protocols with inconsistent survival outcomes...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27746224/efficacy-of-a-russian-backbone-live-attenuated-influenza-vaccine-among-children-in-senegal-a-randomised-double-blind-placebo-controlled-trial
#17
John C Victor, Kristen D C Lewis, Aldiouma Diallo, Mbayame N Niang, Bou Diarra, Ndongo Dia, Justin R Ortiz, Marc-Alain Widdowson, Jodi Feser, Rebecca Hoagland, Shannon L Emery, Kathryn E Lafond, Kathleen M Neuzil
BACKGROUND: Live attenuated influenza vaccines have been shown to significantly reduce influenza in diverse populations of children, but no efficacy studies have been done in resource-poor tropical settings. In Senegal, we assessed the efficacy and safety of a live attenuated influenza vaccine based on Russian-derived master donor viruses and licensed as a single dose. METHODS: In this double-blind, placebo-controlled, parallel group, single-centre trial done near Niakhar, Senegal, generally healthy children aged 2-5 years were randomly allocated (2:1) to receive a single intranasal dose of masked trivalent live attenuated influenza vaccine or placebo...
December 2016: Lancet Global Health
https://www.readbyqxmd.com/read/27741115/case-report-and-literature-review-glomerular-and-neurologic-thrombotic-microangiopathy-as-a-primary-manifestation-of-multicentric-castleman-disease
#18
REVIEW
Adrien Flahault, Marguerite Vignon, Marion Rabant, Aurélie Hummel, Laure-Hélène Noël, Danielle Canioni, Bertrand Knebelmann, Felipe Suarez, Khalil El Karoui
INTRODUCTION: We report the case of a multicentric Castleman disease (MCD) with initial renal involvement. Although the renal involvement in this case was typical of MCD, it constitutes a rare presentation of the disease, and in our case the renal manifestations led to the haematological diagnosis. CLINICAL FINDINGS/PATIENT CONCERNS: The patient was admitted for fever, diarrhea, anasarca, lymphadenopathies and acute renal failure. Despite intravenous rehydration using saline and albumin, renal function worsened and the patient required dialysis...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27725549/diffuse-large-b-cell-lymphoma-during-corticosteroid-therapy-for-tafro-syndrome
#19
Eiko Ohya, Minoru Mizutani, Haruna Sakaguchi, Takao Sekine
Thrombocytopenia, anasarca, myelofibrosis, renal dysfunction and organomegaly (TAFRO) syndrome is a variant of Castleman's disease recently identified in Japan. A 73-year-old man was diagnosed with TAFRO syndrome according to clinical findings, and his symptoms improved after corticosteroid therapy. Ten months later, lymphadenopathy worsened during tapering of corticosteroids. Histological findings of abdominal lymph nodes showed diffuse large B-cell lymphoma. After 6 cycles of R-CHOP therapy, he has remained in sustained complete remission...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27721728/spontaneous-bacterial-peritonitis-and-anasarca-in-a-female-patient-with-ovarian-hyperstimulation-syndrome-complicated-by-respiratory-and-kidney-failure
#20
Muhammad Abdul Mabood Khalil, Muhammad Salman Ghazni, Jackson Tan, Nazish Naseer, Muhammad Ashhad Ullah Khalil
Ovarian hyperstimulation syndrome (OHSS) was first described in 1960. It may occur as a complication of gonadotropin hormone therapy during assisted pregnancy or for primary infertility. A 26-year-old female patient with polycystic ovarian syndrome and primary infertility was treated to conceive. She received intravenous gonadotropin-releasing hormone (GnRH) along with follicle-stimulating hormone in an outside private clinic. She presented to the emergency department with abdominal and chest pain, loose stool, vomiting, shortness of breath and decreasing urine output...
May 2016: Case Reports in Gastroenterology
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