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Taizo Wada
CD5 is a cell surface molecule that is expressed on most circulating T cells and a small population of B cells and is involved in modulation of antigen-specific receptor-mediated activation. Down-regulation of CD5 on CD8+ T cells is a poorly understood but increasingly recognized phenomenon that may be associated with dysregulated T-cell activation. An increased subpopulation of activated CD8+ T cells with down-regulation of CD5 has recently been described in patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (HLH) and familial HLH caused by perforin deficiency and Munc 13-4 deficiency...
June 19, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
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
Eric Gars, Natasha Purington, Gregory Scott, Karen Chisholm, Dita Gratzinger, Beth A Martin, Robert S Ohgami
Hemophagocytic lymphohistiocytosis is a rare multi-system inflammatory disorder with diagnostic criteria based on the HLH-2004 trial. Hemophagocytosis is the only histomorphologic criterion, but in isolation is neither specific nor sensitive for the diagnosis of hemophagocytic lymphohistiocytosis. While objective thresholds for clinical and laboratory criteria have been established, specific criteria for histomorphologic evidence of hemophagocytosis in hemophagocytic lymphohistiocytosis have not been rigorously evaluated or established...
June 14, 2018: Haematologica
Awais Abbas, Mohammad Raza, Aamina Majid, Yumna Khalid, Syed Hamza Bin Waqar
Hemophagocytic lymphohistiocytosis (HLH) secondary to an infection is a great impersonator. It is caused by hyperimmune activation, which leads to a wide array of hematological abnormalities. If the disease is untreated, it is usually fatal. We report the case of a four-year-old girl who presented to our tertiary care hospital with high-grade fever, frequent loose stools, and bleeding from the lips and gums. Investigations showed pancytopenia, hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia whereas the bone marrow biopsy revealed hemophagocytosis with trilineage suppression...
April 12, 2018: Curēus
Amitabh Singh, Lesa Dawman, Rachna Seth
Aim of Study: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of immune dysregulation resulting in abnormal T-cell activation and inflammatory cytokine production which produces a constellation of clinical features unique to HLH. Pediatric secondary HLH is usually triggered by infection, malignancy, or rheumatological disorders. The diagnosis of malignancy-associated HLH (MA-HLH) poses a difficult challenge as clinical features may be attributed to the underlying disease or chemotherapy...
April 2018: Journal of Cancer Research and Therapeutics
Richard H Row, Amy Pegg, Brian Kinney, Gist H Farr, Lisa Maves, Sally Lowell, Valerie Wilson, Benjamin Louis Martin
The mesodermal germ layer is patterned into mediolateral subtypes by signaling factors including BMP and FGF. How these pathways are integrated to induce specific mediolateral cell fates is not well understood. We used mesoderm derived from post-gastrulation neuromesodermal progenitors (NMPs), which undergo a binary mediolateral patterning decision, as a simplified model to understand how FGF acts together with BMP to impart mediolateral fate. Using zebrafish and mouse NMPs, we identify an evolutionarily conserved mechanism of BMP and FGF mediated mediolateral mesodermal patterning that occurs through modulation of basic helix-loop-helix (bHLH) transcription factor activity...
June 7, 2018: ELife
Zakia Sefsafi, Brahim El Hasbaoui, Amina Kili, Aomar Agadr, Mohammed Khattab
Macrophage activation syndrome (MAS) is a severe and potentially fatal life-threatening condition associated with excessive activation and expansion of T cells with macrophages and a high expression of cytokines, resulting in an uncontrolled inflammatory response, with high levels of macrophage colony-stimulating factor and causing multiorgan damage. This syndrome is classified into primary (genetic/familial) or secondary forms to several etiologies, such as infections, neoplasias mainly hemopathies or autoimmune diseases...
2018: Pan African Medical Journal
Dylan E Lee, M Estela Martinez-Escala, Linda M Serrano, Xiaolong A Zhou, Jason B Kaplan, Barbara Pro, Jaehyuk Choi, Joan Guitart
Importance: Hemophagocytic lymphohistiocytosis (HLH) has been reported as a serious complication of cutaneous T-cell lymphoma (CTCL). Despite available diagnostic guidelines, it remains a diagnostic and therapeutic challenge in this patient population. Objectives: To examine the characteristics of CTCL associated with HLH and analyze the presenting signs and symptoms, therapeutic options, and outcome. Design, Setting, and Participants: In this case series, patients diagnosed with CTCL and HLH who were treated at a single institution from January 1, 2014, through December 31, 2017, were studied...
June 6, 2018: JAMA Dermatology
Itziar Astigarraga, Luis I Gonzalez-Granado, Luis M Allende, Laia Alsina
Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a disorder with high mortality, typically recognised at paediatric age. Without proper treatment, HLH can be fatal. The risk of a rapid progression to multi-organ failure and central nervous system involvement leading to long-term sequelae, are the most feared consequences of a diagnostic delay. Therefore, HLH is a medical emergency that paediatricians should be able to identify in a patient with fever and progressive worsening of general condition...
June 2, 2018: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
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
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
Uroosa Ibrahim, Amina Saqib, Maryam Rehan, Jean Paul Atallah
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes...
2018: Case Reports in Hematology
Fei Li, Xiaojie Zhang, Yunyun Wang, Ailin Yang, Zhanglin Zhang, Weiping Tang, Nan Zhong, Huidong Shi
BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) is considered to be a large challenge for clinicians due to the variable overlaps of symptoms with other severe diseases and a high rate of mortality. Prompt diagnosis and treatment are crucial to avoid a fatal outcome. However, very limited reports have focused on HLH during chemotherapy (Ch-HLH) due to a low incidence and insufficient knowledge. CASE PRESENTATION: A 22-year-old male was diagnosed with acute monocytic leukemia with FLT3-ITD and DNMT3A mutations and pulmonary infection...
May 29, 2018: BMC Cancer
A L Booth, P Osehobo, D Rodgers-Soriano, A Lalarukh, M A Eltorky, H L Stevenson
Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disease that often presents with nonspecific findings. A high index of suspicion is necessary to make a prompt diagnosis and prevent fatal disease. A 45-year-old man presented with fever, hypotension, abdominal pain, nausea, and vomiting. Imaging showed hepatosplenomegaly and laboratory tests revealed pancytopenia, increased ferritin, and a cholestatic pattern of injury with elevated alkaline phosphatase and total bilirubin. Due to a history of Crohn disease, systemic lupus erythematous, and rheumatoid arthritis, the patient was on immunosuppressants, including infliximab...
January 2018: Case Reports in Gastroenterology
Marek Trybus, Beata Stepańczak, Mateusz Koziej, Maksymilian Gniadek, Małgorzata Kołodziej, Mateusz Hołda
BACKGROUND: The aim of this study was to perform anthropometrical measurements of patients' hands with carpal tunnel syndrome (CTS) in order to evaluate if there is a correlation between CTS occurrence and hand features regarding sexual dimorphism, age and physical activity. MATERIALS AND METHODS: Study sample consisted of 48 patients (33 females) and control group included 80 healthy volunteers (56 females) with no history of CTS. Following measurements were performed: the wrist circumference, length of the hand, the hand's width, width of the wrist, thickness of the wrist, height of the hypothenar and thenar, length of the arm and forearm, circumference of the proximal phalanges and width of the digits; as well as several indexes were calculated i...
May 26, 2018: Folia Morphologica (Warsz)
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
Hiroki Miyabayashi, Satoru Kumaki, Atsushi Sato, Ryoichi Onuma, Rie Noguchi, Taiki Sato, Takaya Metoki, Yohei Watanabe, Yusaku Tazawa, Masue Imaizumi, Setsuko Kitaoka
Hemophagoytic lymphohistiocytosis (HLH) is a rare life-threatening disorder caused by overactivation of the immune system, associated with infections, autoimmune disorders, and malignancies. The pathological hallmark of HLH is phagocytosis of blood cells and platelets by activated macrophages and histiocytes. In this report, we describe the onset of HLH in three children, aged 2, 5 and 7 years old, during the treatment of acute focal bacterial nephritis (AFBN) with an antibiotic, piperacillin-tazobactam (PIPC-TAZ)...
2018: Tohoku Journal of Experimental Medicine
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
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
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