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https://www.readbyqxmd.com/read/28210942/how-we-treat-chronic-active-epstein-barr-virus-infection
#1
REVIEW
Akihisa Sawada, Masami Inoue, Keisei Kawa
Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years...
February 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28210636/hemophagocytic-lymphohistiocytosis-in-a-patient-with-hodgkin-lymphoma-and-concurrent-ebv-cmv-and-candida-infections
#2
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/28202118/-clinical-and-laboratory-features-of-macrophage-activation-syndrome
#3
Li Guo, Mei-Ping Lu, Gui-Juan Dong, Li-Ping Teng, Yi-Ping Xu, Li-Xia Zou, Qi Zheng
OBJECTIVE: To study the clinical and laboratory features of macrophage activation syndrome (MAS) at the early stage of diagnosis, and to explore a method for early identification of MAS. METHODS: A retrospective analysis was performed for the demographic data, clinical and laboratory features, and treatment outcomes of 21 MAS patients. RESULTS: Of the 21 MAS patients, 14 had systemic juvenile idiopathic arthritis, 5 had Kawasaki disease (KD), and 2 had connective tissue disease (CTD) as primary diseases...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28196537/characterization-of-a-novel-disease-causing-mutation-in-exon-1-of-sh2d1a-gene-through-amplicon-sequencing-a-case-report-on-hlh
#4
Shiyuan Zhou, Hongyu Ma, Bo Gao, Guangming Fang, Yi Zeng, Qing Zhang, GaoFu Qi
BACKGROUND: Hemophagocytic lymphohistocytosis (HLH) is a rare but fatal hyperinflammatory syndrome caused by uncontrolled proliferation of activated macrophages and T lymphocytes secreting high amounts of inflammatory cytokines. Genetic defect is a common cause of HLH. HLH is complicated to be diagnosed as there are many common symptoms with other disorders. CASE PRESENTATION: Here we report on an HLH case caused by 1 bp deletion in gene SH2D1A. Patient was a 3-years-old boy and had fever for more than 8 days...
February 14, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28193996/a-case-report-of-primary-nasal-natural-killer-nk-t-cell-lymphoma-in-an-african-american-patient-presenting-with-hemophagocytic-syndrome
#5
Bowei Tan, Cherif Abdelmalek, James E O'Donnell, Thomas Toltaku, Rashid Chaudhry, Jen C Wang, Vladimir Gotlieb
BACKGROUND Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. CASE REPORT A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1...
February 14, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28191297/leishmania-infantum-and-epstein-barr-virus-co-infection-in-a-patient-with-hemophagocytosis
#6
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
#7
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/28180067/-hemophagocytic-lymphohistiocytosis-after-ebv-reactivation-and-ibrutinib-treatment-in-relapsed-refractory-chronic-lymphocytic-leukemia
#8
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/28163944/immune-reconstitution-inflammatory-syndrome-opening-pandora-s-box
#9
Mariana Meireles, Conceição Souto Moura, Margarida França
One of the purposes of antiretroviral therapy (ART) is to restore the immune system. However, it can sometimes lead to an aberrant inflammatory response and paradoxical clinical worsening known as the immune reconstitution inflammatory syndrome (IRIS). We describe a 23-year-old male, HIV1 infected with a rapid progression phenotype, who started ART with TCD4+ of 53 cells/mm(3) (3,3%) and HIV RNA = 890000 copies/mL (6 log). Four weeks later he was admitted to the intensive care unit with severe sepsis...
2017: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/28161232/-correctly-address-the-cause-of-hemophagocytic-lymphohistiocytosis
#10
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/28153057/unusual-presentation-of-a-severely-ill-patient-having-severe-fever-with-thrombocytopenia-syndrome-a-case-report
#11
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/28149024/extremely-high-ferritinemia-associated-with-haemophagocytic-lympho-histiocytosis-hlh
#12
Neelima Verma, Jyoti Chakraverty, Pankaj Baweja, Alka Girotra, Leena Chatterjee, Manish Chugh
Hyperferritinemia (>10,000 ng/ml) is an important hallmark used as an indicator of infection triggered macrophage activation syndrome leading to hemophagocytic lympho histiocytosis (HLH). Measurement of serum ferritin can be used in diagnosis as well as disease monitoring indicator and prognosis related to HLH, cAPS, sepsis, neoplasm and inflammatory conditions. It is a major contributor to manage critically ill patients as predicting and monitoring indicator. It can be used as acute phase response in conditions of MAS, AOSD, cAPS etc...
March 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28120605/hemophagocytic-lymphohistiocytosis-mimics-many-common-conditions-case-series-and-review-of-literature
#13
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/28100334/-a-case-report-of-childhood-primary-hemophagocytic-syndrome
#14
Ying-Chao Wang, Gui-Ying Zhu, Chu-Yun Yin, Lan-Jun Jiang, Xiao-Ming Zhao, Guang-Yao Sheng
No abstract text is available yet for this article.
January 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#15
Julius Koifman, John Granton, John Thenganatt
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28090281/successful-treatment-of-immune-reconstitution-inflammatory-syndrome-related-hemophagocytic-syndrome-in-an-hiv-patient-with-primary-effusion-lymphoma
#16
Markela-P Zorzou, Maria Chini, Athina Lioni, Georgios Tsekes, Thomas Nitsotolis, Ioannis Tierris, Nicolaos Panagiotou, Dimitra Rontogianni, Nicolaos Harhalakis, Marios Lazanas
Although the connection of [secondary hemophagocytic syndrome (sHS)] with HIV has been well documented, optimal treatment regimen is not well established. This is due not only to the rarity of the syndrome, but also to the heterogeneity of the involved population. Most cases are related to opportunistic infections or malignancies in advanced stage, but many cases are also related to seroconversion, in the primary infection setting. Moreover, in the [antiretroviral treatment (ART)] era, rare cases of ART-related sHS have been reported...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/28088756/super-refractory-status-epilepticus-with-hemophagocytic-syndrome-in-a-child-with-hiv-infection
#17
Neeraj Gupta, Puneet Jain, Kuldeep Singh, Shilajit Bhattacharya
No abstract text is available yet for this article.
January 14, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28070498/hemophagocytic-lymphohistiocytosis-in-adults-an-under-recognized-entity
#18
Abdul Rashid Shah, Tariq Muzzafar, Rita Assi, Dawid Schellingerhout, Zeev Estrov, Gevorg Tamamyan, Hagop Kantarjian, Naval Daver
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28060133/secondary-hemophagocytic-lymphohistiocytosis-do-we-really-need-chemotherapeutics-for-all-patients
#19
Zeliha Haytoglu, Nalan Yazici, Ayse Erbay
Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28052298/the-maze-of-diagnosing-hemophagocytic-lymphohistiocytosis-single-center-experience-of-a-series-of-6-clinical-cases
#20
Maher Hanoun, Ulrich Dührsen
Clinical symptoms of hemophagocytic lymphohistiocytosis (HLH) are based on an excessive inflammatory response. Not only the diversity of the putative underlying etiologies of this rare syndrome but also the subsequent large variety of symptoms complicate the diagnosis of HLH in adult patients. However, early diagnosis and immediate treatment initiation are imperative for clinical outcome. In this article, we will review the diagnostic criteria of HLH and, in this context, discuss 6 cases, each of whom presented with a different clinical appearance causally associated with distinct malignant and nonmalignant diseases, exemplifying the spectrum of associations and manifestations of HLH...
January 5, 2017: Oncology
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