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hemophagocytic syndrome

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https://www.readbyqxmd.com/read/28816794/x-linked-lymphoproliferative-disease-type-1-in-a-patient-with-the-p-gly93asp-sh2d1a-gene-mutation-and-hemophagocytic-lymphohistiocytosis
#1
Raquel de la Varga-Martínez, Francisco Mora-López, Daniel García-Cuesta, M Paz Garrastazul-Sánchez, Sebastián Quintero, Carmen Rodríguez, Almudena Sampalo
Hemophagocytic lymphohistiocytosis is characterized by uncontrolled activation of the immune system that leads to systemic hyperinflammation. Lymphoproliferative syndrome linked to the X chromosome is a hereditary immunodeficiency characterized by an inability to mount an adequate immune response to an Epstein-Barr virus infection. Hemophagocytic lymphohistiocytosis is one of the main clinical features of X-linked lymphoproliferative syndrome. We report the case of a patient who presented with primary hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus infection without a familial history of immunodeficiency...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28815559/characteristic-elevation-of-soluble-tnf-receptor-ii-to-i-ratio-in-macrophage-activation-syndrome-with-systemic-juvenile-idiopathic-arthritis
#2
Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Yasuo Nakagishi, Akihiro Yachie
To investigate the clinical significance of soluble tumor necrosis factor receptor (sTNFR) II/I ratio as an indicator of the diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA), we measured the serum sTNFRI and II levels in 117 patients with s-JIA, including 29 patients with MAS, 15 with Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis (EBVHLH), 15 with Kawasaki disease (KD), and 28 healthy controls (HCs). We determined their correlation with measurements of disease activity and severity...
August 16, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28807602/neutralization-of-interferon-%C3%AE-reverts-clinical-and-laboratory-features-in-a-mouse-model-of-macrophage-activation-syndrome
#3
Giusi Prencipe, Ivan Caiello, Antonia Pascarella, Alexei A Grom, Claudia Bracaglia, Laurence Chatel, Walter G Ferlin, Emiliano Marasco, Raffaele Strippoli, Cristina de Min, Fabrizio De Benedetti
BACKGROUND: The pathogenesis of macrophage activation syndrome (MAS) is not clearly understood: a large body of evidence supports the involvement of mechanisms similar to those implicated in primary hemophagocytic lymphohistiocytosis. OBJECTIVE: To investigate the pathogenic role of interferon-γ (IFNγ) and the therapeutic efficacy of IFNγ neutralization in an animal model of macrophage activation syndrome. METHODS: We used a MAS model established in mice transgenic for human interleukin-6 (IL-6TG) challenged with LPS (MAS mice)...
August 11, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28807357/development-and-initial-validation-of-the-macrophage-activation-syndrome-primary-hemophagocytic-lymphohistiocytosis-score-a-diagnostic-tool-that-differentiates-primary-hemophagocytic-lymphohistiocytosis-from-macrophage-activation-syndrome
#4
Francesca Minoia, Francesca Bovis, Sergio Davì, Antonella Insalaco, Kai Lehmberg, Susan Shenoi, Sheila Weitzman, Graciela Espada, Yi-Jin Gao, Jordi Anton, Toshiyuki Kitoh, Ozgur Kasapcopur, Helga Sanner, Rosa Merino, Itziar Astigarraga, Maria Alessio, Michael Jeng, Vyacheslav Chasnyk, Kim E Nichols, Zeng Huasong, Caifeng Li, Concetta Micalizzi, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli, AnnaCarin Horne
OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample...
August 11, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28806468/an-overview-of-hemophagocytic-lymphohistiocytosis
#5
Ysabella M Esteban, Jill L O de Jong, Melissa S Tesher
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH. Familial HLH is generally an autosomal recessive condition, whereas secondary HLH is usually associated with infectious diseases, autoinflammatory and autoimmune diseases (where it is more commonly known as macrophage activation syndrome), malignancy, immunosuppression, hematopoietic stem cell transplantation, organ transplantation, HIV infection, and metabolic diseases...
August 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28776428/diagnostic-value-of-serum-ferritin-and-cytokine-profiles-of-hemophagocytic-syndrome-following-allogeneic-hematopoietic-cell-transplantation-methodological-issues
#6
Hideo Koh, Satoru Nanno, Masayuki Hino, Hirohisa Nakamae
No abstract text is available yet for this article.
August 4, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28767989/report-of-a-rare-case-of-histiocytic-necrotizing-lymphadenitis-with-bilateral-pleural-effusion-diagnosed-via-cervical-lymph-node-biopsy
#7
Xuchun Liu, Shubin Huang, Guohua Jiang
CONTEXT: Histiocytic necrotizing lymphadenitis (HNL) is a rare disorder that is often benign and self-limiting. There have been reports of co-occurrence of HNL with other diseases, including systemic lupus erythematosus, hemophagocytic syndrome and antiphospholipid syndrome. CASE REPORT: Here, we report a case in which a patient experienced unexplained fever, swelling of the cervical lymph node and bilateral pleural effusion and was ultimately diagnosed with HNL based on results from a lymph node biopsy...
July 31, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28762079/elevated-serum-ferritin-is-not-specific-for-hemophagocytic-lymphohistiocytosis
#8
Zaher K Otrock, Karl G Hock, Sarah B Riley, Theo de Witte, Charles S Eby, Mitchell G Scott
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, syndrome of excessive and ineffective activation of the immune system. The majority of the reported data on HLH is from pediatric patients and lacks specificity. This makes HLH diagnosis challenging especially in adults where HLH is triggered by many conditions and can resemble many disease entities. Elevated ferritin is one of the diagnostic criteria for HLH. We determined the conditions associated with elevated ferritin at our medical center to assess how specific ferritin is for predicting HLH...
July 31, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28760295/diagnostic-challenges-of-hemophagocytic-lymphohistiocytosis
#9
REVIEW
Zaher K Otrock, Naval Daver, Hagop M Kantarjian, Charles S Eby
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by excessive activation of the immune system, resulting in overproduction of inflammatory cytokines. Patients usually present with high fever, cytopenias, hyperferritinemia, and hepatosplenomegaly, and their disease process ranges from mild to fatal multiorgan failure. HLH is a heterogeneous group of disorders that can be triggered by infections, neoplasms, or autoimmune diseases. The HLH diagnosis can be difficult to confidently confirm in critically ill patients while waiting for pathology or reference laboratory results to return, delaying the diagnosis with significantly worsened outcomes...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28732360/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#10
REVIEW
Hongluan Wang, Lixia Xiong, Weiping Tang, Ying Zhou, Fei Li
As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly...
July 14, 2017: Oncotarget
https://www.readbyqxmd.com/read/28723775/the-neutrophil-to-lymphocyte-ratio-could-be-a-good-diagnostic-marker-and-predictor-of-relapse-in-patients-with-adult-onset-still-s-disease-a-strobe-compliant-retrospective-observational-analysis
#11
Ji-Yeoun Seo, Chang-Hee Suh, Ju-Yang Jung, Ar-Reum Kim, Ji Won Yang, Hyoun-Ah Kim
The neutrophil-to-lymphocyte ratio (NLR) is the proportion of absolute neutrophil count to lymphocytes on routine complete blood count (CBC) tests, and has been studied as a simple marker of the systemic inflammatory response. This study was performed to investigate whether the NLR could be used as a tool to diagnose and predict prognosis in cases of adult-onset Still's disease (AOSD).We retrospectively reviewed 164 patients with suspected AOSD. Among 164 patients with suspected AOSD, 37 patients received another diagnosis (such as viral infection) and were compared with the 127 patients who received a diagnosis of AOSD...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28722256/diagnostic-and-prognostic-value-of-low-percentage-of-glycosylated-ferritin-in-acquired-hemophagocytic-lymphohistiocytosis-a-single-center-study
#12
M Nabergoj, M Marinova, G Binotto, L Brugnaro, M Zaninotto, M Plebani, G Semenzato, F Vianello
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome characterized by an excessive immune activation. Glycosylated ferritin (GF) level has been proposed as highly specific of HLH. METHODS: We have studied 12 subjects with HLH according to the HLH-04 trial criteria and 11 patients with a clinical and laboratoristic suspicion of HLH. The percentage of GF was measured by an in-house assay. RESULTS: The only biomarkers that were significantly different in the two groups were fraction of GF (P<...
July 18, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28719435/leukocytoclastic-vasculitis-and-dermal-perivascular-hemophagocytosis-associated-with-adalimumab-therapy-for-rheumatoid-arthritis
#13
Jun Muto, Jun Usami, Daisuke Watanabe
Tumor necrosis factor (TNF)-α inhibitors target TNF-α to effectively treat autoimmune inflammatory conditions, such as rheumatoid arthritis. However, many cases of cutaneous and systemic vasculitis related to TNF-α inhibitors have been reported in the literature. Here, the authors report the first case of a 61-year-old Japanese woman who developed leukocytoclastic vasculitis with cutaneous perivascular hemophagocytosis, which was related to elevated cytokines and immune complexes after initiating adalimumab for rheumatoid arthritis without evidence of hemophagocytic syndrome and rarely encountered in the skin...
July 14, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28717524/anasarca-fever-thrombocytopenia-organomegaly-and-multiorgan-failure-in-a-24-year-old-pregnant-woman
#14
Guillaume Morel, Joy Mootien, Philippe Guiot, Khaldoun Kuteifan
TAFRO syndrome is a distinct idiopathic multicentric Castleman disease characterized by the association of thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We report the first case occurring in a Caucasian pregnant woman. At 34 weeks of gestation, our patient presented with all clinical and biological symptoms compatible with a TAFRO syndrome. Tough quick cesarean section was performed as symptoms got worse with onset of multiorgan failure requiring mechanical ventilation for acute respiratory distress, continuous renal replacement, and vasopressors...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28717082/post-cytokine-release-salt-wasting-as-inverse-tumor-lysis-syndrome-in-a-non-cerebral-natural-killer-cell-neoplasm
#15
Hirotsugu Ariizumi, Yosuke Sasaki, Hiroshi Harada, Yui Uto, Remi Azuma, Tomohide Isobe, Koji Kishimoto, Eisuke Shiozawa, Masafumi Takimoto, Nobuyuki Ohike, Hiraku Mori
The pathogenesis of cerebral/renal salt-wasting syndrome remains unknown. We herein present a case of salt-wasting syndrome with a natural killer-cell neoplasm without cerebral invasion. A 78-year-old man with hemophagocytic syndrome received two cycles of chemotherapy that did not induce tumor lysis syndrome, but repeatedly caused polyuria and natriuresis. The expression of tumor necrosis factor-α in the neoplasm led us to hypothesize that an oncolysis-induced cytokine storm may have caused renal tubular damage and salt wasting...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28700505/sepsis-due-to-streptococcus-pneumoniae-associated-with-secondary-hemophagocytic-lymphohistiocytosis-in-a-splenectomized-patient-for-spherocytosis-a-case-report
#16
Victoria Birlutiu, Rares Mircea Birlutiu
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome that is characterized by an inappropriate hyperinflammatory immune response - primary, as a consequence of a genetic defect of NK cells and cytotoxic T lymphocytes or - secondary, in the progression of infections, rheumatic or autoimmune diseases, malignancies or metabolic diseases. PATIENT CONCERNS: We present the case of a secondary HLH due to Streptococcus pneumoniae infection in a splenectomised patient for spherocytosis, a 37-year-old patient who was splenectomised in childhood for spherocytosis, without immuneprophylaxis induced by antipneumococcal vaccine...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28700492/an-unusual-association-between-hemophagocytic-lymphohistiocytosis-mixed-connective-tissue-disease-and-autoimmune-hemolytic-anemia-a-case-report
#17
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28684198/flow-cytometry-based-diagnosis-of-primary-immunodeficiency-diseases
#18
REVIEW
Hirokazu Kanegane, Akihiro Hoshino, Tsubasa Okano, Takahiro Yasumi, Taizo Wada, Hidetoshi Takada, Satoshi Okada, Motoi Yamashita, Tzu-Wen Yeh, Ryuta Nishikomori, Masatoshi Takagi, Kohsuke Imai, Hans D Ochs, Tomohiro Morio
Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited diseases of the immune system. The definite diagnosis of PID is ascertained by genetic analysis; however, this takes time and is costly. Flow cytometry provides a rapid and highly sensitive tool for diagnosis of PIDs. Flow cytometry can evaluate specific cell populations and subpopulations, cell surface, intracellular and intranuclear proteins, biologic effects associated with specific immune defects, and certain functional immune characteristics, each being useful for the diagnosis and evaluation of PIDs...
July 3, 2017: Allergology International: Official Journal of the Japanese Society of Allergology
https://www.readbyqxmd.com/read/28683778/unusual-acute-lupus-hemophagocytic-syndrome-a-test-of-diagnostic-criteria-a-case-report
#19
Wijetunga Mudalige Udai Akalanka Wijetunga, Ravindra Laxman Satarasinghe, Balasuriya Mudiyanselage Dayananda, Ganhewage Kokila Darshani
BACKGROUND: Hemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon. CASE PRESENTATION: A 57-year-old Sri Lankan woman presented with intermittent fever, weight loss, episodic confusion, and alopecia for 3 months...
July 7, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28669977/an-unusual-triad-of-hemophagocytic-syndrome-lymphoma-and-tuberculosis-in-a-non-hiv-patient
#20
Hafiz Rizwan Talib Hashmi, Rashmi Mishra, Masooma Niazi, Sindhaghatta Venkatram, Gilda Diaz-Fuentes
BACKGROUND Lymphoma complicated with hemophagocytic syndrome and tuberculosis has been rarely reported. The clinical and radiological presentation of these potentially fatal conditions can be easily confused and there is a potential for misdiagnosis. CASE REPORT We present a 58-year-old Hispanic female who was admitted to the hospital with dizziness and fever. Her initial admission diagnosis was severe sepsis secondary to community acquired pneumonia. She was started on intravenous antibiotics. Due to mediastinal lymphadenopathy, lymphoma was considered as a differential diagnosis for which she underwent bronchoscopy and endobronchial ultrasound-guided sampling of her mediastinal lymph nodes...
July 3, 2017: American Journal of Case Reports
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