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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28222313/cytokine-profiles-as-novel-diagnostic-markers-of-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-in-children
#1
Xiu-Cui Han, Qing Ye, Wei-Ying Zhang, Yong-Min Tang, Xiao-Jun Xu, Ting Zhang
PURPOSE: The aim of this study was to identify specific laboratory indices to distinguish Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children. MATERIALS AND METHODS: In this prospective study, Th1/Th2 cytokines, including IL-2, IL-4, IL-6, IL-10, TNF-α, and IFN-γ, were analyzed in patients with EBV-HLH or sepsis at the onset of disease by flow cytometry. RESULTS: IL-10, IFN-γ, IL-10/IL-6, and IFN-γ/IL-6 were higher and IL-6 was lower in EBV-HLH patients compared to sepsis patient levels...
February 10, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28219834/incidence-and-outcomes-of-central-nervous-system-hemophagocytic-lymphohistiocytosis-relapse-after-reduced-intensity-conditioning-hematopoietic-stem-cell-transplant
#2
Dana T Lounder, Pooja Khandelwal, Sharat Chandra, Michael B Jordan, Ashish R Kumar, Michael Grimley, Stella M Davies, Jack J Bleesing, Rebecca A Marsh
Hemophagocytic Lymphohistiocytosis (HLH) is an immune regulatory disorder that commonly presents with CNS involvement. The only cure for genetic HLH is hematopoietic stem cell transplantation (HSCT), typically treated with reduced intensity conditioning (RIC) regimens. We sought to estimate the incidence of CNS relapse after RIC HSCT, determine risk factors, and evaluate outcomes. We performed a retrospective chart review of 94 consecutive children and young adults with primary HLH who received RIC HSCT. CNS relapse within 1 year after transplant was diagnosed by review of clinical symptoms, cerebral spinal fluid (CSF), and radiologic findings...
February 17, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28219570/living-donor-lung-transplantation-after-bone-marrow-transplantation-for-chediak-higashi-syndrome
#3
Tetsu Yamada, Toyofumi F Chen-Yoshikawa, Shigeharu Oh, Rieko Ito-Taniguchi, Fumiaki Gochi, Masaaki Sato, Akihiro Aoyama, Hiroshi Date
An 8-year-old girl with Chediak-Higashi syndrome (CHS) had pulmonary complications after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) and eventually underwent single living-donor lobar lung transplantation (LDLLT). Electron micrographic findings showed vagus nerve tissue in extracted lung having granular inclusions, which are pathognomonic for CHS. Because her mother was the donor for both hematopoietic stem cell and lung transplantations, she was weaned from immunosuppression and is doing well 3 years after lung transplantation...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28210636/hemophagocytic-lymphohistiocytosis-in-a-patient-with-hodgkin-lymphoma-and-concurrent-ebv-cmv-and-candida-infections
#4
Moaath Mustafa Ali, Ana Lucia Ruano Mendez, Hetty E Carraway
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by immune activation and subsequent widespread organ damage. Patients affected by HLH commonly develop fever, cytopenias, liver damage, neurologic manifestations, and hypercytokinemia. In this case, we describe a 60-year-old male who presented with HLH and concurrent Epstein-Barr virus, cytomegalovirus, and Candida infections and was subsequently diagnosed with a Hodgkin lymphoma. This case highlights the importance of considering a cancer diagnosis in the differential diagnosis of patients presenting with HLH...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28202022/hematologic-manifestations-of-babesiosis
#5
REVIEW
Tamer Akel, Neville Mobarakai
BACKGROUND: Babesiosis, a zoonotic parasitic infection transmitted by the Ixodes tick, has become an emerging health problem in humans that is attracting attention worldwide. Most cases of human babesiosis are reported in the United States and Europe. The disease is caused by the protozoa of the genus Babesia, which invade human erythrocytes and lyse them causing a febrile hemolytic anemia. The infection is usually asymptomatic or self-limited in the immunocompetent host, or follows a persistent, relapsing, and/or life threatening course with multi-organ failure, mainly in the splenectomized or immunosuppressed patients...
February 15, 2017: Annals of Clinical Microbiology and Antimicrobials
https://www.readbyqxmd.com/read/28193515/infliximab-not-associated-with-increased-risk-of-malignancy-or-hemophagocytic-lymphohistiocytosis-in-pediatric-patients-with-inflammatory-bowel-disease
#6
Jeffrey S Hyams, Marla C Dubinsky, Robert N Baldassano, Richard B Colletti, Salvatore Cucchiara, Johanna Escher, William Faubion, John Fell, Benjamin D Gold, Anne Griffiths, Sibylle Koletzko, Subra Kugathasan, James Markowitz, Frank M Ruemmele, Gigi Veereman, Harland Winter, Nicholas Masel, Chu Ri Shin, Kezhen L Tang, Meena Thayu
BACKGROUND AND AIMS: Immunosuppressive therapy for inflammatory bowel disease (IBD) in pediatric patients is thought to increase risk of malignancy and lymphoproliferative disorders, including hemophagocytic lymphohistiocytosis (HLH). We compared unadjusted incidence rates and of malignancy and HLH in pediatric patients with IBD exposed to infliximab compared with patients not exposed to biologics and calculated standardized incidence ratios (SIRs). METHODS: We collected and analyzed data from 5766 participants in a prospective study of long-term outcomes of pediatric patients with IBD (NCT00606346), from 2007 through 30 June 2016...
February 10, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28191297/leishmania-infantum-and-epstein-barr-virus-co-infection-in-a-patient-with-hemophagocytosis
#7
Zied Gaifer, Mohamed-Rachid Boulassel
The authors describe a rare case of a 27- year old previously healthy male presenting with high grade fever, pancytopenia, hepatosplenomegaly, high levels of ferritin and triglyceride, suggesting a diagnosis of hemophagocytic lymphohistiocytosis (HLH) syndrome. Other investigations showed a positive Leishmania infantum serology and high Epstein-Barr virus (EBV) viremia. The diagnosis of a visceral leishmaniasis was confirmed by bone morrow biopsy, which showed Leishman-Donovan bodies and evidence of HLH. The patient received liposomal amphotericin B and he had a complete resolution of his symptoms and clearance of EBV viremia...
December 31, 2016: Infectious Disease Reports
https://www.readbyqxmd.com/read/28188950/hemophagocytic-lymphohistiocytosis-in-a-neonate-case-report
#8
Pari Zarrini, Ziba Mosayebi, Asghar Ramyar, Hosein Dalili
 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This article will introduce a neonate with HLH in Iran. We report a case of HLH presenting with respiratory distress and fever, hepatosplenomegaly, jaundice and pancytopenia on the second day of life. Typical clinical and laboratory findings were detected in the neonate. HLH was diagnosed according to HLH-2004 guidelines...
January 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/28185252/diagnostic-dilemmas-in-hlh-can-t-cell-phenotyping-help
#9
Rebecca A Marsh
The diagnosis of hemophagocytic lymphohistiocytosis (HLH) can be a difficult one, and the distinction between primary versus secondary HLH can be particularly challenging during the early stages of diagnosis. This distinction is important to make as primary HLH requires allogeneic hematopoietic cell transplantation for a definitive cure. Flow cytometric screening tests for many of the genetic forms of HLH are available. However, not all patients with primary HLH are captured by these screening tests, due to the fact that no screening test is 100% sensitive, and additionally, some patients with "primary" forms of HLH may have mutations in genes which are yet to be discovered...
February 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28185204/application-of-an-improved-flow-cytometry-based-nk-cell-activity-assay-in-adult-hemophagocytic-lymphohistiocytosis
#10
Jia Zhang, Yini Wang, Lin Wu, Jingshi Wang, Ran Tang, Shuo Li, Jianhang Chen, Zhuo Gao, Ruijun Pei, Zhao Wang
Low or absent natural killer (NK) cell activity is included as one of the HLH-2004 diagnostic criteria. To improve the diagnosis of HLH, we aimed to establish a rapid and reliable NK cell activity assay that avoids the use of radioactivity. The K562 cell line, as standard NK target cells, was engineered to stably express enhanced green fluorescent protein (EGFP), which can be quantified by flow cytometry. The EGFP-flow cytometry method for measuring NK cell activity was improved by double staining of early and late apoptotic target cells...
February 9, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28180067/-hemophagocytic-lymphohistiocytosis-after-ebv-reactivation-and-ibrutinib-treatment-in-relapsed-refractory-chronic-lymphocytic-leukemia
#11
Maurizio Cavallari, Maria Ciccone, Simonetta Falzoni, Francesco Cavazzini, Luca Formigaro, Francesco Di Virgilio, Antonella Rotola, Gian Matteo Rigolin, Antonio Cuneo
Hemophagocytic Lymphohistiocytosis (HLH) is a rare syndrome characterized by ineffective T-cell and NK response. We report the clinical course of a patient with relapsed CLL who developed acute symptoms soon after starting ibrutinib. Hyperpyrexia, splenomegaly, hyperferritinemia, hypertriglyceridemia, cytopenias, and a typical cytokine pattern, i.e. high interleukin (IL)-6, IL10 and IL18, were consistent with a diagnosis of HLH. Coexistent Epstein Barr virus reactivation was documented. Ibrutinib-induced impairment of NK degranulation, associated with EBV reactivation and CLL-related immunodeficiency may have contributed to the development of HLH in our patient...
2017: Leukemia Research Reports
https://www.readbyqxmd.com/read/28178007/role-of-skin-biopsies-in-the-diagnosis-of-hemophagocytic-lymphohistiocytosis
#12
Aileen Santos-Arroyo, Julián Barrera-Llaurador, Julio E Sánchez, Rafael Martín-García, Jorge L Sánchez
This is a report of a 15-year-old female who presented with a skin eruption and clinical features consistent with severe sepsis and septic shock. A diagnosis of acquired hemophagocytic lymphohistiocytosis (HLH) was made and confirmed by bone marrow analysis. Skin biopsy showed hemophagocytosis and CD163-positive staining of macrophages. We briefly review the clinical, histologic, and laboratory findings of hemophagocytic lymphohistiocytosis in addition to the potential role of skin biopsies in this condition...
January 26, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28161232/-correctly-address-the-cause-of-hemophagocytic-lymphohistiocytosis
#13
M Penel-Page, B Ben Said, A Phan, L Hees, C Hartmann-Merlin, S Girard, Y Gillet, A Belot
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe syndrome usually associated with a cytotoxicity deficiency, which leads to an excess of immune response driven by activated macrophages and cytotoxic T cells. In children, HLH can be genetic, as part of a familial lymphohistiocytosis, or secondary: the most frequent causes are systemic-onset juvenile idiopathic arthritis, hematological malignancies, and severe infections, especially with Ebstein-Barr virus or leishmaniosis. We report on the case of a 3-year-old girl with no past medical history, who presented inaugural Pseudomonas aeruginosa maxillary osteitis, with secondary HLH...
February 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28155064/how-to-treat-involvement-of-the-central-nervous-system-in-hemophagocytic-lymphohistiocytosis
#14
REVIEW
AnnaCarin Horne, Ronny Wickström, Michael B Jordan, E Ann Yeh, Ahmed Naqvi, Jan-Inge Henter, Gritta Janka
Central nervous system (CNS)-hemophagocytic lymphohistiocytosis (HLH) is not a disease in itself, but it is part of a systemic immune response. The vast majority of patients with CNS-HLH also have systemic HLH and a large number of patients with primary and secondary HLH have CNS involvement. Reactivations within the CNS are frequent during the course of HLH treatment and may occur concomitant with or independent of systemic relapses. It is also important to consider primary HLH as an underlying cause of "unknown CNS inflammation" as these patients may present with only CNS disease...
January 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28153549/a-rare-life-threatening-kodamaea-ohmeri-endocarditis-associated-with-hemophagocytic-lymphohistiocytosis
#15
Buqing Ni, Weidong Gu, Yaning Mei, Kourong Miao, Shijiang Zhang, Yongfeng Shao
No abstract text is available yet for this article.
January 30, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28153057/unusual-presentation-of-a-severely-ill-patient-having-severe-fever-with-thrombocytopenia-syndrome-a-case-report
#16
Masahiko Kaneko, Masaki Maruta, Hisaharu Shikata, Kengo Asou, Hiroto Shinomiya, Tadaki Suzuki, Hideki Hasegawa, Masayuki Shimojima, Masayuki Saijo
BACKGROUND: Severe fever with thrombocytopenia syndrome is an emerging infectious disease caused by a novel phlebovirus belonging to the family Bunyaviridate. Emergence of encephalitis/encephalopathy during severe fever with thrombocytopenia syndrome progression has been identified as a major risk factor associated with a poor prognosis. Here we report the case of a severely ill patient with severe fever with thrombocytopenia syndrome virus-associated encephalitis/encephalopathy characterized by a lesion of the splenium, which resolved later...
February 3, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28120605/hemophagocytic-lymphohistiocytosis-mimics-many-common-conditions-case-series-and-review-of-literature
#17
A T Akenroye, N Madan, F Mohammadi, J Leider
Introduction. Hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease, is characterized by excessive immune activation and cytokine release which stimulates bone marrow macrophages to engulf hematopoietic cells. HLH could be secondary to infections: viral, fungal, and bacterial; malignancies and autoimmune diseases. The diagnosis of HLH is usually delayed due to the presence of non-specific symptoms at presentation. This delay contributes to increased mortality. Cases and review. We present the case of 4 patients who presented with subjective fevers and extreme fatigue...
January 2017: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28108590/human-immunity-against-ebv-lessons-from-the-clinic
#18
REVIEW
Stuart G Tangye, Umaimainthan Palendira, Emily S J Edwards
The mammalian immune system has evolved over many millennia to be best equipped to protect the host from pathogen infection. In many cases, host and pathogen have coevolved, each acquiring sophisticated ways of inducing or protecting from disease. Epstein-Barr virus (EBV) is a human herpes virus that infects >90% of individuals. Despite its ubiquity, infection by EBV is often subclinical; this invariably reflects the necessity of the virus to preserve its host, balanced with sophisticated host immune mechanisms that maintain viral latency...
February 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28105088/clinical-significance-of-lymphocyte-subset-changes-in-hemophagocytic-lymphohistiocytosis-of-children
#19
Qi An, Yi Wang, Shaoyan Hu, Daihua Fang, Chengmin Xuan, Shumei Xu, Mingwei Jin, Qiang Ji
In order to examine the role of peripheral blood lymphocyte subsets on the diagnosis, treatment and prognosis of hemophagocytic lymphohistiocytosis (HLH), 30 affected children during the acute period of the disease and 30 healthy children within the same age range were selected to test their peripheral blood lymphocyte subsets using flow cytometry and compare these subsets. At the same time, the peripheral blood lymphocyte subsets of 20 children with complete remission from HLH were compared to those of 10 cases who succumbed to the disease...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28095017/spectrum-of-xanthogranulomatous-processes-in-the-abdomen-and-pelvis-a-pictorial-review-of-infectious-inflammatory-and-proliferative-responses
#20
Kelsey S Bourm, Christine O Menias, Kamran Ali, Kinan Alhalabi, Khaled M Elsayes
OBJECTIVE: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. XG infectious processes are mainly seen in cholecystitis and pyelonephritis, but several other organs can also be involved. Histiocytic processes can be divided into Langerhans and non-Langerhans cell histiocytosis...
March 2017: AJR. American Journal of Roentgenology
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