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hemophagocytic syndrome

Juliana Chaves Ruiz Guedes, Karen de Almeida Pinto Fernandes da Cunha, Jorge Ricardo da Silva Machado, Luciana Wernersbach Pinto
Extranodal NK/T-cell lymphoma nasal type is a rare disease that mainly affects the nasal cavity and paranasal sinuses of males in the fifth decade of life. It has aggressive and locally destructive behaviour, and can be complicated by the hemophagocytic syndrome, conferring high lethality to the disease. This article describes a case of NK/T-cell lymphoma nasal type in a previously healthy patient, exemplifying its rapid and fulminant course.
June 2018: Anais Brasileiros de Dermatologia
Jasmine Gowarty, Julie Oda, Christian Cable
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of widespread inflammation due to massive amounts of cytokines released from activated macrophages. The most common trigger for HLH is infection from a virus, most commonly Epstein-Barr virus. Here we report an adult case of this rare and life-threatening syndrome.
July 2018: Proceedings of the Baylor University Medical Center
Masood Sadaat, Sekwon Jang
BACKGROUND: Hemophagocytic Lymphohistiocytosis (HLH), a rare but potentially fatal syndrome of immune hyperactivation, may be an under-recognized immune-related adverse event (irAE). Unlike other irAEs, HLH triggered by immune checkpoint blockade is not well described; no particular diagnostic guidelines and treatment regimens exist. The HLH-2004 criteria remain as the common diagnostic guide. For the treatment of HLH, various combinations of chemotherapeutic, immunosuppressive and glucocorticoid agents are used...
June 5, 2018: Journal for Immunotherapy of Cancer
Shima Yasin, Grant S Schulert
PURPOSE OF REVIEW: The past decade has seen substantial progress in defining the cause and pathogenesis of the chronic childhood arthropathy systemic juvenile idiopathic arthritis (SJIA) and its related complication macrophage activation syndrome (MAS). The purpose of this review is to describe and synthesize advances in this field, particularly since 2016, with the potential to transform clinical practice. RECENT FINDINGS: Newly developed MAS classification criteria have been further studied and validated in other diseases and populations, as well as a recently proposed score to distinguish MAS from familial hemophagocytic lymphohistiocytosis...
June 4, 2018: Current Opinion in Rheumatology
Hao Gu, Jie Ma, Zhenping Chen, Jing Wang, Rui Zhang, Runhui Wu
Autoimmune lymphoproliferative syndrome (ALPS) usually presents in childhood with fever, nonmalignant splenomegaly and lymphadenopathy along with hemocytopenia. This case report describes a 10-year-old boy presenting with signs of autoimmune disease, splenomegaly, hepatomegaly and resistant hemocytopenia. Sirolimus controlled the relapsed thrombocytopenia after splenectomy. Sequencing of the FAS gene identified two spontaneous heterozygous mutations (c.234 T > G, p.D78E) (c.236dupA, p.P80Tfs*26). The boy's homozygous missense variation (c...
June 1, 2018: Gene
Panjarat Sowithayasakul, Phakatip Sinlapamongkolkul, Jitsupa Treetipsatit, Nassawee Vathana, Nattee Narkbunnam, Kleebsabai Sanpakit, Jassada Buaboonnam
Mediastinal germ cell tumor (MGCT), which accounts for 1% to 3% of extragonadal germ cell tumors, has unique manifestations; it is associated with several types of hematologic malignancy, particularly myeloid neoplasm. The aim of this study was to report the 10-year incidence, clinical characteristics, and outcomes of MGCT at Thailand's national pediatric tertiary referral center. This retrospective study included patients diagnosed with MGCT at the Department of Pediatrics, Siriraj Hospital during 2005 to 2014...
June 1, 2018: Journal of Pediatric Hematology/oncology
Rongjiong Zheng, Songsong Xie, Xiaobo Lu, Lihua Sun, Yan Zhou, Yuexin Zhang, Kai Wang
Background . Brucellosis has a wide spectrum of clinical manifestations and it may last several days or even several years; however, it is often misdiagnosed and therefore may cause inadequate therapy and prolonged illness. Previous studies about meta-analysis of manifestations of brucellosis reported in English lacked the data published in Chinese, which did not provide details about the contact history, laboratory tests, and misdiagnosis. We undertake a meta-analysis of clinical manifestations of human brucellosis in China to identify those gaps in the literature...
2018: BioMed Research International
Naoto Sakumura, Masaki Shimizu, Mao Mizuta, Natsumi Inoue, Yasuo Nakagishi, Akihiro Yachie
This study aims to investigate the clinical significance of serum soluble CD163 (sCD163) levels as a predictor of the disease activity of systemic juvenile idiopathic arthritis (s-JIA). In this study, we examined 63 patients with s-JIA, four with Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis (EBV-HLH), and seven with Kawasaki disease (KD), along with 14 healthy controls. We quantified serum cytokine levels (sCD163, neopterin, IL-18, IL-6) by enzyme-linked immunosorbent assay and compared the results with the clinical features of s-JIA...
May 23, 2018: Cytokine
Mixue Xie, Li Li, Lixia Zhu, De Zhou, Xiudi Yang, Jianai Sun, Jingjing Zhu, Mingyu Zhu, Yanlong Zheng, Wanzhuo Xie, Xiujin Ye
Background: Lymphoma-associated hemophagocytic syndrome (LAHS) is a highly fatal immune disorder. Poor prognosis is partly attributed to under diagnosis or delayed diagnosis. Aim: Early identification of LAHS patients based on the laboratory findings could improve the outcomes. Design: Retrospective observational cross-sectional study. Methods: From January 2011 to June 2016, 282 adult patients with hemophagocytosis in bone marrow were enrolled, and 114 hemophagocytic lymphohistiocytosis (HLH) patients with definite underlying cause were finally included for analysis...
May 24, 2018: QJM: Monthly Journal of the Association of Physicians
M Farhan Nasser, Shorabh Sharma, Elizabeth Albers, Sapna Sharma, Anurag Duggal
Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory disorder characterized by the uncontrolled proliferation of lymphocytes and histiocytes with hemophagocytic activity in the bone marrow. To our knowledge, there have been a few reported cases of pregnancy-related HLH. This case highlights the importance of considering HLH in a pregnant woman along with other diagnoses, such as HELLP (which stands for hemolysis, elevated liver enzyme levels, and low platelet levels) syndrome and hemolytic anemias...
March 20, 2018: Curēus
Nophol Leelayuwatanakul, Napplika Kongpolprom
Intravascular large B cell lymphoma (IVLBCL) is a rare and aggressive subtype of diffuse large B cell lymphoma, of which clinical presentations are highly variable among geographical areas. A case series of IVLBCL patients from Asian countries reported the disease to be more aggressive and associated with hemophagocytic syndrome than in cases from Western countries. Although published articles recently revealed hypoxemia as a presentation in IVLBCL patients, orthodeoxia has never been documented. A 71-year-old man presented with prolonged fever, cough, exertional dyspnoea, and orthodeoxia, later developing hypoxemic respiratory failure and refractory septic shock...
April 2018: Respirology Case Reports
Matthew D Taylor, Thomas N Burn, E John Wherry, Edward M Behrens
Familial hemophagocytic lymphohistiocytosis 2 (FHL2) is a cytokine storm syndrome characterized by immune hyperactivation with viral infection due to a CD8 T cell cytotoxic killing defect secondary to a perforin deficiency. As most studies of FHL2 mice have used pathogen naïve animals, the effects of immune memory on FHL2 are understudied. We utilized an immunization model of the perforin-deficient mouse to study the effects of immune memory on FHL2. Prior CD8 T cell specific antigen exposure leads to enhanced HLH disease with increased morbidity and decreased time to mortality...
February 2018: ImmunoHorizons
J H Chen, T T Wang, J Sun, X Y Lyu
No abstract text is available yet for this article.
May 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Hirofumi Toko, Hiroto Tsuboi, Naoto Umeda, Fumika Honda, Ayako Ohyama, Hidenori Takahashi, Saori Abe, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Tomoya Hirota, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond...
May 18, 2018: Internal Medicine
Shinya Daitoku, Tomomi Aoyagi, Shinichiro Takao, Seiya Tada, Mika Kuroiwa
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome that occurs as a complication in many clinical settings. Malignancy-associated HLH develops in patients with hematopoietic neoplasms, particularly in those with lymphoma, and its development in those with myelodysplastic syndrome (MDS) is uncommon. We herein report a case of HLH in a patient with low-risk MDS that was successfully treated with azacitidine. The prevalence of immune abnormalities among MDS patients and the immune effects of azacitidine have recently been elucidated, suggesting that MDS-associated HLH occurs as a result of immune impairment, and azacitidine improves this condition by restoring the immune system...
May 18, 2018: Internal Medicine
Jiří Šrámek, Thomas Karvunidis, Daniel Lysák, Martin Harazim, Michal Karas, Pavel Jindra
Hemophagocytic lymfohistiocytosis (HLH) is rare, life-threatening condition, characterized by excessive activation of immune system with subsequent proinflammatory state resulting in multiorgan failure. Most frequently, it appears in infancy as a primary disorder caused by mutation of immune-regulatory genes. Increasingly, HLH is being diagnosed as a secondary - adult - form, which occurs as a result of aberrant immune response. Viral or bacterial systemic infections, malignancy with a predominance of lymphoproliferative disorders and autoimmune diseases are the most common triggers...
2018: Vnitr̆ní Lékar̆ství
Weihong Shi, Mingyang Duan, Ligang Jie, Weifeng Sun
RATIONALE: A 27-year-old woman with a history of systemic lupus erythaematosus (SLE) developed hemophagocytic syndrome (HPS) secondary due to an unrecognized infection that led to severe SLE with a prolonged recovery. PATIENT CONCERNS: The patient showed a high spiking fever and myalgia. Laboratory data revealed pancytopenia and immunological abnormalities. Pulse methylprednisone plus intravenous immunoglobulin (IVIG) failed to improve the clinical symptoms and laboratory data...
May 2018: Medicine (Baltimore)
Jin-Jun Yang, Xin-Chuan Chen, Yun Tang, Kai Shen, Li-Ping Xie, Ting Liu
OBJECTIVE: To analyze the clinical features,response to therapy and prognosis of intravascular large B-cell lymphoma (IVLBCL). METHODS: The clinical data of 17 cases with IVLBCL were retrospectively reviewed,and survival analysis was conducted. RESULTS: The study involved 10 males and 7 females of IVLBCL with a mean age of 53 years old. The most common symptom of the disease was recurrent fever (76.5%). The lymphoma was mainly observed in bone marrow (64...
January 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
John Jacob Kinross-Wright, Kalyan Chakravarthy Potu, Brandy Pownell, Randall Lamfers, Jonathan S Bleeker
Background: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. Case Summary: A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation...
2018: Case Reports in Hematology
Yusuke Koizumi, Ken-Ichi Imadome, Yasunori Ota, Hitoshi Minamiguchi, Yoshinori Kodama, Dai Watanabe, Hiroshige Mikamo, Tomoko Uehira, Seiji Okada, Takuma Shirasaka
Epstein-Barr virus (EBV) reactivation causes serious diseases in immunocompromised hosts, such as acquired immunodeficiency syndrome (AIDS). We report on a case of plasmablastic lymphoma (PBL) with hemophagocytic lymphohistiocytosis (HLH).A-53-year-old Japanese man was diagnosed with PBL and AIDS. In addition to combined antiretroviral therapy, HyperCVAD (cyclophosphamide, doxorubicin, vincristine, prednisone)/high-dose methotrexate + cytarabine was initiated immediately. Partial remission was attained with chemotherapy...
April 23, 2018: Journal of Clinical Immunology
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