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Hemophagocytosis

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https://www.readbyqxmd.com/read/27883370/epstein-barr-virus-associated-hemophagocytic-syndrome-after-scrub-typhus-infection
#1
Jeong Woo Hong, Hyun Seon You, Tae Won Lee, Won Yong Jo, Bo Ra Kim, Young Sun Suh, In Gyu Bae, Oh Hyun Cho
There have been a small number of cases of scrub typhus-associated hemophagocytic syndrome (HPS), most of which were treated successfully using adequate antibiotics. Here, we report a case of Epstein-Barr virus (EBV)-associated HPS after scrub typhus infection that was not improved using antirickettsial treatment. A 73-year-old male who had been diagnosed with scrub typhus according to an eschar and a positive serology was transferred to our institution because of a persistent fever despite 7-day doxycycline therapy...
November 8, 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27863755/identification-of-the-myst3-crebbp-fusion-gene-in-infants-with-acute-myeloid-leukemia-and-hemophagocytosis
#2
Francianne Gomes Andrade, Elda Pereira Noronha, Rosania Maria Baseggio, Teresa Cristina Cardoso Fonseca, Bruno Marcelo Rocha Freire, Isis M Quezado Magalhaes, Ilana R Zalcberg, Maria S Pombo-de-Oliveira
BACKGROUND: Acute myeloid leukemia presenting the MYST3-CREBBP fusion gene is a rare subgroup associated with hemophagocytosis in early infancy and monocytic differentiation. The aim of this study was to define the relevant molecular cytogenetic characteristics of a unique series of early infancy acute myeloid leukemia cases (≤24months old), based on the presence of hemophagocytosis by blast cells at diagnosis. METHODS: A series of 266 infant cases of acute myeloid leukemia was the reference cohort for the present analysis...
October 2016: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/27847518/adult-onset-still-s-disease-associated-with-mycoplasma-pneumoniae-infection-and-hemophagocytic-lymphohistiocytosis
#3
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
https://www.readbyqxmd.com/read/27846765/adult-onset-still-s-disease-like-manifestation-accompanied-by-the-cancer-recurrence-after-long-term-resting-state
#4
Kazuhito Fukuoka, Ayako Miyamoto, Yuko Ozawa, Noriko Ikegaya, Tomohiro Maesono, Yoshinori Komagata, Shinya Kaname, Yoshihiro Arimura
A 72-year-old woman presented nine months ago with skin rash on her bilateral forearms, which was followed by intermittent high fever, and stiffness and swelling of her bilateral fingers. She was diagnosed with seronegative rheumatoid arthritis (RA). She had a past history of breast cancer and had undergone breast preservation surgery 13 years previously. During admission in our hospital, she developed high fever and leukocytosis with a relapsing skin rash, sore throat, polyarthralgia and increased levels of serum ALT/AST and ferritin, all of which fulfilled Yamaguchi's criteria for adult-onset Still's disease (AOSD)...
November 16, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27826329/recurrent-macrophage-activation-syndrome-since-toddler-age-in-an-adolescent-boy-with-hla-b27-positive-juvenile-ankylosing-spondylitis
#5
Joon Hyeong Park, Yu Mi Seo, Seung Beom Han, Ki Hwan Kim, Jung Woo Rhim, Nack Gyun Chung, Myung Shin Kim, Jin Han Kang, Dae Chul Jeong
Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3...
October 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27822583/prevalence-severity-and-pathogeneses-of-anemia-in-visceral-leishmaniasis
#6
REVIEW
Yasuyuki Goto, Jingjie Cheng, Satoko Omachi, Ayako Morimoto
Anemia is a typical symptom during visceral leishmaniasis (VL). We performed a systematic analysis of the literature on anemia in VL to understand the prevalence, severity, and possible mechanisms. Anemia is very common in VL patients with an overall prevalence higher than 90 %. The degree of anemia in VL is moderate to severe (hemoglobin level ∼7.5 g/dl), and the status can be recovered by treatment with antileishmanial drugs within a certain period of time. Possible pathogeneses of anemia in VL based on clinical observations included anti-RBC antibodies, dysfunction in erythropoiesis, and hemophagocytosis in the bone marrow or spleen, while hemolysis is a more likely cause than dyserythropoiesis...
November 7, 2016: Parasitology Research
https://www.readbyqxmd.com/read/27818826/macrophage-activation-syndrome-associated-with-adult-onset-still-s-disease-successfully-treated-with-anakinra
#7
Aswini Kumar, Hiroshi Kato
Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still's disease (Still's disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still's disease, whether it is therapeutic for MAS associated with Still's disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27803821/hemophagocytic-lymphohistiocytosis-in-a-patient-with-classical-hodgkin-lymphoma
#8
G Hyun, K J Robbins, N Wilgus, L Grosso, S D Goyal
Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can be associated with inherited genetic mutations, malignancy, autoimmune disorders, and viral infections. Though the pathogenesis is not fully known, HLH is understood to be a reactive process in the setting of uncontrolled activation of macrophages, CD8+ cytotoxic lymphocytes, and other immune cells. Hallmark clinicopathological features of HLH include fevers, cytopenias, hepatosplenomegaly, and hemophagocytosis in the bone marrow...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27801337/-the-clinical-characteristics-of-macrophage-activation-syndrome-secondary-to-systemic-lupus-erythematosus
#9
N Jiang, M T Li, D Wu, X F Zeng
Objective: To investigate the clinical features of macrophage activation syndrome (MAS) associated with systemic lupus erythematosus (SLE). Method: The clinical data of 15 patients with SLE-induced MAS diagnosed in Peking Union Medical College Hospital from July 2011 to December 2014 were retrospectively analyzed. Results: Fourteen patients were female. The average age was 28.07. When MAS occurred, the average duration of SLE was 20.47 months, and the average SLE disease activity index (SLEDAI) was 18.4. All 15 patients developed fever, hematocytopenia and impaired liver function in the course of MAS, while patients with splenomegaly, coagulation disorders and neuropsychiatric symptoms were 11, 14 and 8, respectively...
November 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27795864/histiocytic-sarcoma-in-a-kidney-transplant-patient-a-case-report-and-review-of-the-literature
#10
Maressa Pollen, Siraj El Jamal, Jack Lewin, Varsha Manucha
Objective. Histiocytic sarcoma (HS) is an aggressive neoplasm with only limited number of reported series of cases and rare case reports of occurrence as a posttransplant neoplastic disorder. The etiology and pathogenesis of the disease is unknown and the optimal treatment is still under investigation. We describe an unusual case of HS in a patient with a remote history of kidney transplant. Method and Results. A 54-year-old male with a remote history of renal transplantation under maintenance immunosuppression presented with features of sepsis...
2016: Case Reports in Pathology
https://www.readbyqxmd.com/read/27795540/primary-immunodeficiency-disease-and-hematology
#11
Hirokazu Kanegane
Primary immunodeficiency disease (PID) is an inborn error of the immune system, and is characterized by not only susceptibility to infection but also frequent combination with autoimmune diseases and malignancies. PID is principally caused by a germline mutation, and some PID patients develop hematological abnormalities. In some patients, PID is associated with hemophagocytosis-induced cytopenia, neutropenia, thrombocytopenia, and autoimmune cytopenia. In addition, a subset of PID patients presented with myeloid dysplasia...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27785117/a-retrospective-analysis-of-56-children-with-hemophagocytic-lymphohistiocytosis
#12
Yan-Rong Wang, Yi-Ning Qiu, Yan Bai, Xian-Feng Wang
AIM: The aim of this study was to investigate the etiological factors, clinical features, and prognostic factors in children with hemophagocytic lymphohistiocytosis (HLH). METHODS: Fifty-six children with HLH in Wuhan Union Hospital, People's Republic of China, were retrospectively analyzed in recent years. We reviewed the medical records of 56 HLH children hospitalized from 2000 to 2013 to identify the possible prognostic factors. RESULTS: In more than half of the cases (64...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27776187/epidemiological-and-clinical-features-of-severe-fever-with-thrombocytopenia-syndrome-in-japan-2013-2014
#13
Hirofumi Kato, Takuya Yamagishi, Tomoe Shimada, Tamano Matsui, Masayuki Shimojima, Masayuki Saijo, Kazunori Oishi
Although severe fever with thrombocytopenia syndrome (SFTS) was first reported from Japan in 2013, the precise clinical features and the risk factors for SFTS have not been fully investigated in Japan. Ninety-six cases of severe fever with thrombocytopenia syndrome (SFTS) were notified through the national surveillance system between April 2013 and September 2014 in Japan. All cases were from western Japan, and 82 cases (85%) had an onset between April and August. A retrospective observational study of the notified SFTS cases was conducted to identify the clinical features and laboratory findings during the same period...
2016: PloS One
https://www.readbyqxmd.com/read/27760897/a-term-infant-of-neonatal-toxic-shock-syndrome-like-exanthematous-disease-complicated-with-hemophagocytic-syndrome
#14
Akimune Kaga, Hiroshi Watanabe, Hiroki Miyabayashi, Takaya Metoki, Setsuko Kitaoka, Satoru Kumaki
Neonatal toxic shock syndrome-like exanthematous disease (NTED) is a newly recognized neonatal infectious disease, caused by the superantigen toxic shock syndrome toxin-1 (TSST-1). TSST-1 is mainly produced by methicillin-resistant Staphylococcus aureus, and the immune responses to TSST-1 are known to cause toxic shock syndrome, a life-threatening infectious disease. The clinical symptoms of NTED are skin rash, fever, and thrombocytopenia, but severe thrombocytopenia is rare in term infants with NTED. Although the cause of NTED is the same as that of toxic shock syndrome, the clinical symptoms of NTED are milder than toxic shock syndrome...
2016: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27706506/hemophagocytic-lymphohistiocytosis-and-bone-marrow-hemophagocytosis-a-5-year-institutional-experience-at-a-tertiary-care-hospital
#15
Kriselle Lao, Namita Sharma, Ajeet Gajra, Neerja Vajpayee
OBJECTIVE: The purpose of this study was to correlate the significance of bone marrow hemophagocytosis and analyze outcome data in patients with suspected hemophagocytic lymphohistiocytosis (HLH) at a tertiary care hospital during the course of 5 years. METHODS: The pathology database of State University of New York Upstate Medical University, Syracuse, was searched for the terms "hemophagocytosis," "hemophagocytic syndrome," and "hemophagocytic lymphohistiocytosis" encompassing the period January 2009-December 2014...
October 2016: Southern Medical Journal
https://www.readbyqxmd.com/read/27699249/a-xenograft-model-of-macrophage-activation-syndrome-amenable-to-anti-cd33-and-anti-il-6r-treatment
#16
Mark Wunderlich, Courtney Stockman, Mahima Devarajan, Navin Ravishankar, Christina Sexton, Ashish R Kumar, Benjamin Mizukawa, James C Mulloy
Transgenic expression of key myelosupportive human cytokines in immune-deficient mice corrects for the lack of cross-species activities of stem cell factor (SCF), IL-3, and GM-CSF. When engrafted with human umbilical cord blood (UCB), these triple-transgenic mice produce BM and spleen grafts with much higher myeloid composition, relative to nontransgenic controls. Shortly after engraftment with UCB, these mice develop a severe, fatal macrophage activation syndrome (MAS) characterized by a progressive drop in rbc numbers, increased reticulocyte counts, decreased rbc half-life, progressive cytopenias, and evidence of chronic inflammation, including elevated human IL-6...
September 22, 2016: JCI Insight
https://www.readbyqxmd.com/read/27658824/hemophagocytic-lymphohistiocytosis-responding-to-withdrawal-of-gluten-a-case-report
#17
Nicholas J Fordham, Richa Ajitsaria, Leena Karnik, Subarna Chakravorty
BACKGROUND: This is the first documented case of a patient with hemophagocytic lymphohistiocytosis in association with coeliac disease. There was complete clinical and biochemical remission of hemophagocytic lymphohistiocytosis following the introduction of a gluten-free diet. CASE PRESENTATION: A 7-year-old white girl presented with fevers and maculopapular rash with a recent history of tonsillitis. Blood tests revealed thrombocytopenia (64×10(9)/L), anemia (80 g/L), hypofibrinogenemia (1 g/L), and hyperferritinemia (71,378 μg/L)...
2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27633921/use-of-extracorporeal-support-in-hemophagocytic-lymphohistiocytosis-secondary-to-ehrlichiosis
#18
Amy Cheng, Feifei Williams, James Fortenberry, Catherine Preissig, Steven Salinas, Pradip Kamat
Ehrlichiosis, caused by transmission of Ehrlichia chaffeensis to humans through the bite of an infected lone star tick, can lead to secondary hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition caused by uncontrolled activation of the cellular immune system. We describe a child with HLH secondary to ehrlichiosis who developed multiorgan failure and was successfully managed with extracorporeal membrane oxygenation (ECMO). A 9-year-old boy developed headaches, fever, and sore throat after suspected tick exposure...
September 15, 2016: Pediatrics
https://www.readbyqxmd.com/read/27631517/ebv-negative-aggressive-nk-cell-leukemia-lymphoma-clinical-pathologic-and-genetic-features
#19
Alina Nicolae, Karthik A Ganapathi, Trinh Hoc-Tran Pham, Liqiang Xi, Carlos A Torres-Cabala, Nahid M Nanaji, Hongbin D Zha, Zhen Fan, Sybil Irwin, Stefania Pittaluga, Mark Raffeld, Elaine S Jaffe
Aggressive natural killer cell leukemia (ANKL) is a systemic NK-cell neoplasm, almost always associated with Epstein-Barr virus (EBV). Rare cases of EBV-negative ANKL have been described, and some reports suggested more indolent behavior. We report the clinicopathologic, immunophenotypic, and molecular characteristics of 7 EBV-negative ANKL. All patients were adults, with a median age of 63 years (range 22 to 83 y) and an M:F ratio of 2.5:1. Five patients were White, 1 Black, and 1 Asian. All patients presented acutely, with fever (6/7), cytopenias (6/7), and splenomegaly (4/7)...
September 14, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27610283/correlation-of-antiglobulin-reactivity-and-severity-of-pancytopenia-in-a-patient-with-hemophagocytic-lymphohistiocytosis-a-case-report-and-review-of-literature
#20
Uroosa Ibrahim, Muhammad N Siddique, Gautam Valecha, Masoud Asgari, Edhan Isaac, Meekoo Dhar
A 46‑year‑old obese male with a medical history of thalassemia minor presented to the emergency room with complaints of severe fatigue and jaundice worsening over two weeks. On further evaluation, the patient was found to have significant hyperbilirubinemia and transaminitis. The hospital course was further complicated by pancytopenia requiring multiple transfusions, worsening hyperbilirubinemia, severe hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia. He was also found to have splenomegaly and evidence of hemophagocytosis on bone marrow biopsy...
2016: Curēus
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