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Hemophagocytosis

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https://www.readbyqxmd.com/read/28733045/induction-of-live-cell-phagocytosis-by-a-specific-combination-of-inflammatory-stimuli
#1
Takamasa Ishidome, Takeshi Yoshida, Rikinari Hanayama
Conditions of severe hyper-inflammation can lead to uncontrolled activation of macrophages, and the ensuing phagocytosis of live cells. However, relationships between inflammatory stimuli and uncontrolled phagocytosis of live cells by macrophages are poorly understood. To identify mediators of this process, we established phagocytosis assays of live cells by stimulating macrophages with CpG DNA, interferon-γ, and anti-interleukin-10 receptor antibody. In this model, various cell surface receptors were upregulated on macrophages, and phagocytosis of live cells was induced in a Rac1-dependent manner...
July 13, 2017: EBioMedicine
https://www.readbyqxmd.com/read/28732360/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#2
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/28722256/diagnostic-and-prognostic-value-of-low-percentage-of-glycosylated-ferritin-in-acquired-hemophagocytic-lymphohistiocytosis-a-single-center-study
#3
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
#4
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/28683778/unusual-acute-lupus-hemophagocytic-syndrome-a-test-of-diagnostic-criteria-a-case-report
#5
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
#6
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
https://www.readbyqxmd.com/read/28668113/a-28-year-old-woman-with-fever-rash-and-pancytopenia
#7
Sebastian Ochoa, Kyle Cheng, Christine M Fleury, Stefano Luccioli, Joseph A Bellanti
A 28-year-old Hispanic woman was admitted to the hospital with fever, sore throat, arthralgia, and a generalized rash of 2 weeks' duration. Her medical history was significant for various food and medication allergies. Multiple antibiotics were given for suspected infection, and she subsequently developed a new skin rash, acute liver injury, eosinophilia, and pancytopenia. Additional studies showed hypertriglyceridemia; elevated interleukin-2 receptor levels; absent natural killer cell activity; and hemophagocytosis in skin, liver, and bone marrow biopsy specimens...
July 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28647558/durable-chimerism-and-long-term-survival-after-unrelated-umbilical-cord-blood-transplantation-for-pediatric-hemophagocytic-lymphohistiocytosis-a-single-center-experience
#8
Sachit A Patel, Heather A Allewelt, Jesse D Troy, Paul L Martin, Timothy A Driscoll, Vinod K Prasad, Joanne Kurtzberg, Kristin M Page, Suhag H Parikh
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immune dysregulation characterized by fever, hepatosplenomegaly, cytopenias, central nervous system disease, increased inflammatory markers, and hemophagocytosis. Currently, allogeneic hematopoietic stem cell transplantation is the only curative approach for patients with HLH, with reported survival ranging from 50% to 70% with myeloablative conditioning (MAC) regimens. However, donor availability and transplantation-related mortality associated with conventional MAC are major barriers to success...
June 21, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28629255/hemophagocytic-lymphohistiocytosis-associated-to-haemophilus-parainfluenzae-endocarditis-a-case-report
#9
D I Costescu Strachinaru, M Chaumont, D Gobin, L Sattar, M Strachinaru, E Karakike, A Roman, D Konopnicki
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal syndrome that is characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. This syndrome is classified as primary (genetic) or secondary when acquired in the context of infections (usually viral), malignancies, rheumatologic and metabolic diseases. CASE SUMMARY: We report a case of HLH complicating a Haemophilus parainfluenzae mitral valve endocarditis and resolving under antibiotic and surgical treatment alone...
June 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28626191/hemophagocytic-lymphohistiocytosis-in-a-fatal-case-of-severe-fever-with-thrombocytopenia-syndrome
#10
Ayako Nakano, Hirohisa Ogawa, Yoshinori Nakanishi, Hiromi Fujita, Fumihiko Mahara, Kazuya Shiogama, Yutaka Tsutsumi, Toshiaki Takeichi
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging disease caused by a novel Bunyavirus with a high mortality rate. We herein report a fatal case of an 86-year-old woman with SFTS complaining of a fever, fatigue, and bicytopenia. Her condition deteriorated with rapid progression of bleeding tendency, disturbance of consciousness, and multiple organ failure leading to death on Day 6 of her illness. The histopathological findings in the autopsy revealed marked infiltration of macrophages with hemophagocytosis in the bone marrow, liver, and spleen leading to a diagnosis of hemophagocytic lymphohistiocytosis (HLH)...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28626190/miliary-tuberculosis-in-a-young-woman-with-hemophagocytic-syndrome-a-case-report-and-literature-review
#11
Mina Asaji, Kazunori Tobino, Koujin Murakami, Yuki Goto, Takuto Sueyasu, Saori Nishizawa, Kohei Yoshimine, Miyuki Munechika, Yuki Ko, Yuki Yoshimatsu, Kosuke Tsuruno, Hiromi Ide, Hiroyuki Miyajima, Noriyuki Ebi
We herein report a rare case of miliary tuberculosis-associated hemophagocytic syndrome (HPS) complicated with respiratory failure. A 19-year-old Japanese woman with a fever, general malaise, and chest radiograph abnormalities was referred to our hospital. After admission, she developed respiratory failure with pancytopenia. A histological examination of lung and bone marrow biopsy samples revealed noncaseating granulomas without evidence of acid-fast bacilli or lymphoma. In addition, a bone marrow biopsy showed marked histiocyte hyperplasia with hemophagocytosis, and a bronchoalveolar lavage fluid culture grew Mycobacterium tuberculosis...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28596829/an-unusual-case-of-primary-hepatic-lymphoma-with-dramatic-but-unsustained-response-to-bendamustine-plus-rituximab-and-literature-review
#12
Sih-Han Liao, Yin-Kai Chen, Shan-Chi Yu, Ming-Shiang Wu, Hsiu-Po Wang, Ping-Huei Tseng
OBJECTIVES: Primary hepatic lymphoma is an uncommon cause of hepatic space-occupying lesions. METHODS: We describe the case of a 73-year-old man with primary hepatic lymphoma, who presented with a low-grade fever and lower limb weakness which had progressed in the past 2 months. RESULTS: Abdominal ultrasound and computed tomography showed multiple small hepatic tumors. Echo-guided biopsy of the hepatic tumor demonstrated primary hepatic diffuse large B cell lymphoma...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28583781/-macrophage-activation-syndrome-and-kawasaki-disease-four-new-cases
#13
B Dumont, P Jeannoel, L Trapes, E Rolland, C Gay, J-L Stephan
Macrophage activation syndrome (MAS) is a rarely reported complication of Kawasaki disease (KD). It must be sought during KD with unusual clinical signs, such as enlargement of the liver or spleen, cytopenia (including thrombocytopenia), and elevated serum triglycerides. Here, we report four cases from a single center. The first is the description of a case of KD occurring in an 11-month-old child 15 days after the occurrence of infectious spondylitis. For the second, acute myocarditis heralded KD in a 5-year-old child...
July 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28562515/secondary-hemophagocytic-lymphohistocytosis-in-a-child-with-brucellosis
#14
Esra Pekpak, Benhur Sirvan Cetin
Hemophagocytic lymphohistocytosis (HLH) is a potentially fatal hyperinflammatory syndrome that is characterized by proliferation of histiocytes and hemophagocytosis in different organs. The diagnostic criteria include fever, hepatosplenomegaly, bicytopenia, high serum ferritin level, decreased natural killer cell activity, elevated soluble CD25 level, high serum fasting triglyceride level or low fibrinogen level, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. HLH can be classified as primary and secondary...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28521652/case-report-patient-with-unexplained-high-fever-and-pancytopenia
#15
Kelly De Schuyter, Tom Lodewyck
OBJECTIVE AND IMPORTANCE: We present a case of hemophagocytic lymphohistiocytosis (HLH), giving insight in how to establish diagnosis and start appropiate treatment. CLINICAL PRESENTATION: A 45-year-old male presented at the emergency ward with high fever and pancytopenia. Repeat bone marrow aspirates showed hemophagocytosis. Extensive work-up with exclusion of other infectious and malignant diseases, eventually lead us to the diagnosis of hemophagocytic lymphohistiocytosis...
May 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28494931/hemophagocytic-lymphohistiocytosis-in-acute-african-swine-fever-clinic
#16
Z R Karalyan, Z R Ter-Pogossyan, N Yu Karalyan, Z B Semerjyan, M R Tatoyan, S A Karapetyan, E M Karalova
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) usually has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, lymph nodes, etc. in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia. HLH is often triggered by viral infection. The aim of this study was to ascertain the features of HLH involvement in African swine fever virus (ASFV) (genotype II) pathogenesis. METHODS: The serum levels of macrophage colony-stimulating factor (MCSF) and granulocyte-macrophage colony-stimulating factor (GMCSF), as well as the histological constitution (for hemophagocytic macrophages detection) of various organs of pigs infected with ASFV genotype II were investigated...
May 2017: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/28489771/chronic-active-epstein-barr-virus-infection-associated-with-hemophagocytic-syndrome-and-extra-nodal-natural-killer-t-cell-lymphoma-in-an-18-year-old-girl-a-case-report
#17
Yawei Xing, Junwen Yang, Guanghui Lian, Shuijiao Chen, Linlin Chen, Fujun Li
RATIONALE: Chronic active Epstein-Barr virus infection (CAEBV) associated with hemophagocytic syndrome (HPS) and extra-nodal natural killer (NK)/T-cell lymphoma (ENKL) is a rare life-threatening disorder. This disease is easily misdiagnosed because of its varied presentations. PATIENT CONCERNS: An 18-year-old girl was admitted to our hospital with a history of edema in the lower limbs and intermittent fever lasting for more than 1 month. At admission, she had severe liver injury of unknown etiology...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28459018/visceral-leishmaniasis-associated-hemophagocytosis-a-tale-of-two-unexpected-diagnoses-from-a-nonendemic-region
#18
Rashmi Kaul Raina, Sujeet Raina, Manupriya Sharma
A case of visceral leishmaniasis (VL)-associated hemophagocytic lymphohistiocytosis (HLH) in an immunocompetent native from a nonendemic area was reported. The patient belonged to Ravi river valley area (altitude 996 meters above the mean sea level) of Chamba, Himachal Pradesh, India. VL and HLH were not a differential diagnosis. Identification of the Leishman-Donovan bodies and hemophagocytosis in bone marrow aspirate and biopsy provided the diagnosis. The patient recovered to the treatment with amphotericin B...
January 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/28403691/novel-nlrc4-mutation-causes-a-syndrome-of-perinatal-autoinflammation-with-hemophagocytic-lymphohistiocytosis-hepatosplenomegaly-fetal-thrombotic-vasculopathy-and-congenital-anemia-and-ascites
#19
Jiancong Liang, Danielle N Alfano, James E Squires, Melissa M Riley, W Tony Parks, Julia Kofler, Areeg El-Gharbawy, Suneeta Madan-Kheterpal, Roxanne Acquaro, Jennifer Picarsic
Autoinflammatory diseases are caused by pathologic activation of the innate immune system. Primary hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation caused by monogenic mutations resulting in cytotoxic cell defects and subsequent failure to eliminate activated macrophages. Secondary HLH is often diagnosed in cases without a known Mendelian inheritance. However, some cases of "secondary" HLH have been shown to harbor mutations with partial dysfunction of the cytotoxic system...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28376538/-hepatic-manifestation-of-a-macrophage-activation-syndrome-mas
#20
Michael Nagel, Andreas Schwarting, Beate K Straub, Peter R Galle, Tim Zimmermann
Background Elevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure...
May 2017: Zeitschrift Für Gastroenterologie
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