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hemophagocytic syndrome

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https://www.readbyqxmd.com/read/27905287/hemophagocytic-syndrome-due-to-leishmania-infection-diagnosed-with-immunofluorescence-antibody-test
#1
Hakan Sarbay, Yasemin Işık Balcı, Selin Güler, Meral Türk, Mehmet Akın, Aziz Polat
Leishmaniasis is a reticuloendothelial system disease that mostly observed before the age of 5. Visceral infection causes long-standing fever, weight loss, weakness, pancytopenia, and hepatosplenomegaly. Leishmania infantum is responsible for visceral leishmaniasis (VL) in Turkey. We present a case of hemophagocytic syndrome due to Leishmania infection diagnosed with an immunofluorescence antibody test (IFAT). Leishmania amastigotes were not observed on bone marrow aspiration. We consider that IFAT is very important for parasite detection in the diagnosis of VL in children, particularly when amastigotes are not obtained on bone marrow aspiration...
September 2016: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/27883371/severe-fever-with-thrombocytopenia-syndrome-presenting-with-hemophagocytic-lymphohistiocytosis
#2
Jongmin Lee, Gyeongmin Jeong, Ji Hun Lim, Hawk Kim, Sun Whan Park, Won Ja Lee, Jae Bum Jun
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by the newly discovered SFTS Bunyavirus, and there have been no case reports of SFTS patients presenting with hemophagocytic lymphohistiocytosis (HLH) in the English literature. We report a case of SFTS presenting with HLH in a 73-year-old immunocompetent male farmer. Although the patient had poor prognostic factors for SFTS, such as old age and central nervous system symptoms, he recovered fully with supportive care...
November 11, 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27883370/epstein-barr-virus-associated-hemophagocytic-syndrome-after-scrub-typhus-infection
#3
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/27862115/pediatric-acute-liver-failure-of-undetermined-cause-a-research-workshop
#4
Estella M Alonso, Simon P Horslen, Edward M Behrens, Edward Doo
: Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials...
November 14, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27853859/hemophagocytic-lymphohistiocytosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-case-report-and-review
#5
REVIEW
L García-Montoya, C N Sáenz-Tenorio, I Janta, J Menárguez, F J López-Longo, I Monteagudo, E Naredo
Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin...
November 16, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27830097/histoplasmosis-induced-hemophagocytic-lymphohistiocytosis-in-an-adult-patient-a-case-report-and-review-of-the-literature
#6
Ramona Vesna Untanu, Syed Akbar, Stephen Graziano, Neerja Vajpayee
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymphocytes lead to production of large amounts of cytokines, also called cytokine storm. Hematopoietic and lymphoid tissues are directly involved while other organs are damaged by circulating cytokines. Primary HLH is attributed to genetic defects of the immune system and secondary HLH is usually seen in adults secondary to malignancy, infection, or autoimmune diseases...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27826329/recurrent-macrophage-activation-syndrome-since-toddler-age-in-an-adolescent-boy-with-hla-b27-positive-juvenile-ankylosing-spondylitis
#7
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/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/27800096/systemic-lupus-erythematous-revealed-by-cytomegalovirus-infection
#9
Rezgui Amel, Karmani Monia, Mzabi Anis, Ben Fredj Fatma, Laouani Chadia
Cytomegalovirus (CMV) infection have been described as exacerbing systemic lupus erythematous (SLE). The role of CMV in starting off SLE remains object of debate. We report a severe presentation of SLE revealed by CMV infection with hemophogocytic syndrome. A 22 old women without a history of systemic disease developed a cutaneous eruption with fever and myalgia persistant for 2 weeks. Laboratory studies revealed a CMV serology supporting acute CMV infection, with positive antinuclear antidody, anti ds DNA, elevated liver functions tests, pancytopenia...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27799439/systemic-lupus-erythematosus-in-spanish-males-a-study-of-the-spanish-rheumatology-society-lupus-registry-relesser-cohort
#10
A Riveros Frutos, I Casas, I Rúa-Figueroa, F J López-Longo, J Calvo-Alén, M Galindo, A Fernández-Nebro, J M Pego-Reigosa, A Olivé Marqués
OBJECTIVE: The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. METHODS: A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). RESULTS: Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women)...
October 31, 2016: Lupus
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
#11
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/27795515/diagnosis-and-treatment-of-hlh-in-adults
#12
Rebecca Porter, Nancy Berliner
Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening syndrome of uncontrolled immune activation. It was initially recognized in children, where it occurs primarily as an inherited syndrome related to homozygous null mutations in immune response genes involved in cytotoxic T cell and NK cell function. A minority of pediatric patients develop "secondary" HLH as a consequence of infection or autoimmune disease. In the last 10-15 years, secondary HLH has been increasingly recognized in adults, where it is frequently associated with lymphoid malignancy, infection, or autoimmune disease...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27793107/acute-hiv-infection-presenting-as-hemophagocytic-syndrome-with-an-unusual-serological-and-virological-response-to-art
#13
Rita Veiga Ferraz, Ana Cláudia Carvalho, Fernando Araújo, Carmo Koch, Cândida Abreu, António Sarmento
BACKGROUND: HIV clinical presentation in the acute stage is variable and some of its virological and immunological aspects are not completely understood. Most cases of HIV- associated reactive hemophagocytic syndrome have been reported in patients with advanced stages of HIV and to our knowledge, there are only 8 cases in the English literature presenting during acute HIV infection, most in East Asia, being this the first case in a European patient. CASE PRESENTATION: We report a case of a European Caucasian 27- year old woman with a primary HIV- infection presenting with extremely low CD4+ T cell count who developed a haemophagocytic syndrome after starting ART and in whom we documented a very unusual serological and virological response, characterized by an impaired HIV- antibody production and a 12 month time frame to reach an undetectable viral load, despite no evidence of resistance...
October 28, 2016: BMC Infectious Diseases
https://www.readbyqxmd.com/read/27785117/a-retrospective-analysis-of-56-children-with-hemophagocytic-lymphohistiocytosis
#14
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/27769081/an-unusual-case-of-lchad-deficiency-presenting-with-a-clinical-picture-of-hemophagocytic-lymphohistiocytosis-secondary-hlh-or-coincidence
#15
Sahin Erdol, Mehmet Ture, Birol Baytan, Tahsin Yakut, Halil Saglam
There are published reports stating that some of the congenital metabolic diseases, such as lysinuric protein intolerance, multiple sulphatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis, might lead to secondary hemophagocytic lymphohistiocytosis (HLH). However, to date, to our knowledge, the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency has never been investigated among patients with HLH. Here, we report on a patient who was referred to our institution for a differential diagnosis of pancytopenia, liver failure, and rhabdomyolysis...
November 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27766802/infection-associated-secondary-hemophagocytic-lymphohistiocytosis-in-sepsis-syndromes-a-tip-of-an-iceberg
#16
Arun Agarwal, Aakanksha Agarwal
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare, underdiagnosed, fatal and devastating hyperinflammatory syndrome that has gained increasing recognition over the past decade. Patients with HLH present with clinical and laboratory evidence of uncontrolled inflammation. Delay in diagnosis and management inevitably leads to a rapidly progressive and fatal course. In this case series, we present 7 cases of secondary HLH (sHLH) in adults with their presentation, course, and outcomes...
October 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27760897/a-term-infant-of-neonatal-toxic-shock-syndrome-like-exanthematous-disease-complicated-with-hemophagocytic-syndrome
#17
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/27759358/symptomatic-primary-selective-igm-immunodeficiency-b-lymphoid-cell-defect-in-adult-man-with-secondary-hlh-syndrome
#18
Arun Agarwal, Samiksha Sharma, Mala Airun
Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations...
July 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27751970/varicella-zoster-virus-infections-in-pediatric-malignancy-patients-a-7-year-analysis
#19
Mine Düzgöl, Gülcihan Özek, Nuri Bayram, Yeşim Oymak, Ahu Kara, Bengü Demirağ, Tuba Hilkay Karapınar, Yılmaz Ay, Canan Vergin, İlker Devrim
Primary Varicella Zoster Virus infection is a benign self-limited disease. In this study, we aimed to review our experience with focusing on the outcome and treatment of the varicella zoster virus (VZV) infection in pediatric malignancy patients. During the study period; a total of 41 patients with pediatric malignancy had been hospitalized with the diagnosis of VZV infection. All the patients were treated with intravenous acyclovir for a median of 7 days (ranging from 5 to 21 days). The calculated attributable delay of chemotherapy due to VZV infections was 8 days (ranging from 2 to 60 days)...
October 18, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/27743708/therapeutic-plasma-exchange-in-primary-hemophagocytic-lymphohistiocytosis-reports-of-two-cases-and-a-review-of-the-literature
#20
Mehmet Bosnak, Seher Erdogan, E Habibe Aktekin, Ali Bay
We report two children who were diagnosed as having primary hemophagocytic syndrome and who successfully underwent therapeutic plasma exchange (TPE). The first patient was a 6-month-old girl diagnosed with HLH who was admitted to the pediatric intensive care unit. The patient's clinical condition worsened on the 9th day of the HLH-2004 treatment protocol. Her ferritin level was found 50.000 ng/mL, and TPE was performed for 9 sessions, after which her clinical condition and laboratory findings improved. The patient is still on the HLH-2004 protocol and waits for a suitable stem cell transplantation donor...
September 28, 2016: Transfusion and Apheresis Science
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