Journal ArticleDOI
Nationwide survey of hemophagocytic lymphohistiocytosis in Japan.
Eiichi Ishii,Shouichi Ohga,Shinsaku Imashuku,Masaki Yasukawa,Hiroyuki Tsuda,Ikuo Miura,Ken Yamamoto,Hisanori Horiuchi,Kenzo Takada,Koichi Ohshima,Shigeo Nakamura,Naoko Kinukawa,Kazuo Oshimi,Keisei Kawa +13 more
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TLDR
Although this nationwide survey establishes the heterogeneous characteristics of HLH, the results should be useful in planning prospective studies to identify the most effective therapy for each HLH subtype.Abstract:
Hemophagocytic lymphohistiocytosis (HLH), a disorder of the mononuclear phagocyte system, can be classified into two distinct forms: primary HLH (FHL) and secondary HLH. To clarify the epidemiology and clinical outcome for each HLH subtype, we conducted a nationwide survey of HLH in Japan. Since 799 patients were diagnosed in 292 institutions of Japan between 2001 and 2005, the annual incidence of HLH was estimated as 1 in 800,000 per year. Among them, 567 cases were actually analyzed in this study. The most frequent subtype was Epstein-Barr virus (EBV)-associated HLH, followed by other infection- or lymphoma-associated HLH. Age distribution showed a peak of autoimmune disease- and infection-associated HLH in children, while FHL and lymphoma-associated HLH occurred almost exclusively in infants and the elderly, respectively. The 5-year overall survival rate exceeded 80% for patients with EBV- or other infection-associated HLH, was intermediate for those with FHL or B-cell lymphoma-associated HLH, and poor for those with T/NK cell lymphoma-associated HLH (<15%). Although this nationwide survey establishes the heterogeneous characteristics of HLH, the results should be useful in planning prospective studies to identify the most effective therapy for each HLH subtype.read more
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Adult haemophagocytic syndrome
Manuel Ramos-Casals,Pilar Brito-Zerón,Armando López-Guillermo,Munther A. Khamashta,Xavier Bosch +4 more
TL;DR: Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms, and it is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder.
Journal ArticleDOI
Recommendations for the management of hemophagocytic lymphohistiocytosis in adults
Paul La Rosée,AnnaCarin Horne,Melissa Hines,Tatiana von Bahr Greenwood,Rafał Machowicz,Nancy Berliner,Sebastian Birndt,Juana Gil-Herrera,Michael Girschikofsky,Michael B. Jordan,Ashok Kumar,Jan A M van Laar,Gunnar Lachmann,Kim E. Nichols,Athimalaipet V Ramanan,Yini Wang,Zhao Wang,Gritta Janka,Jan-Inge Henter +18 more
TL;DR: In this article, the authors present expert opinions derived from an interdisciplinary working group on adult HLH, sponsored by the Histiocyte Society, to facilitate knowledge transfer between physicians caring for pediatric and adult patients with HLH with the aim to improve the outcome for adult patients affected by HLH.
Journal ArticleDOI
EBV-associated T/NK–cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases
Hiroshi Kimura,Yoshinori Ito,Shinji Kawabe,Kensei Gotoh,Yoshiyuki Takahashi,Seiji Kojima,Tomoki Naoe,Shinichi Esaki,Atsushi Kikuta,Akihisa Sawada,Keisei Kawa,Koichi Ohshima,Shigeo Nakamura +12 more
TL;DR: Age at onset of disease and liver dysfunction were risk factors for mortality, whereas patients who received transplantation had a better prognosis, and insight into the diagnostic and therapeutic approaches for distinct disease is provided.
Journal ArticleDOI
Hemophagocytic syndromes — An update
Gritta Janka,Kai Lehmberg +1 more
TL;DR: Treatment aims at suppressing hypercytokinemia and eliminating activated and infected cells, including immunomodulatory and immunosuppressive agents, cytostatics, T-cell and cytokine antibodies.
Journal ArticleDOI
Hemophagocytic lymphohistiocytosis: an update on diagnosis and pathogenesis.
Flavia G. Rosado,Annette S. Kim +1 more
TL;DR: This article summarizes new advances in the diagnosis of HLH and includes a review of clinical findings, updated understanding of the pathogenesis, and promising new testing methods.
References
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Book
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
TL;DR: Thank you very much for reading who classification of tumours of haematopoietic and lymphoid tissues, and maybe you have knowledge that, people have look hundreds of times for their chosen readings like this, but end up in malicious downloads.
Journal ArticleDOI
Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis
Susan E. Stepp,Dufourcq-Lagelouse R,Françoise Le Deist,Françoise Le Deist,Sadhna Bhawan,Stéphanie Certain,Porunelloor A. Mathew,Jan-Inge Henter,Michael J. Bennett,Alain Fischer,Alain Fischer,Geneviève de Saint Basile,Vinay Kumar +12 more
TL;DR: In this paper, the coding regions of the perforin gene of eight unrelated 10q21-22-linked FHL patients revealed homozygous nonsense mutations in four patients and missense mutations in the other four patients.
Journal ArticleDOI
Munc13-4 Is Essential for Cytolytic Granules Fusion and Is Mutated in a Form of Familial Hemophagocytic Lymphohistiocytosis (FHL3)
Jérôme Feldmann,Isabelle Callebaut,Graça Raposo,Stéphanie Certain,Delphine Bacq,Cécile Dumont,Nathalie Lambert,Marie Ouachée-Chardin,Gaëlle Chédeville,Hannah Tamary,Véronique Minard-Colin,Etienne Vilmer,Stéphane Blanche,Françoise Le Deist,Alain Fischer,Geneviève de Saint Basile +15 more
TL;DR: In this article, the locus of a perforin (+) FHL subtype (FHL3), observed in 10 patients, was mapped to 17q25. This region contains hMunc13-4, a member of the Munc13 family of proteins involved in vesicle priming.
Journal ArticleDOI
Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation.
Jan-Inge Henter,AnnaCarin Samuelsson-Horne,Maurizio Aricò,R. Maarten Egeler,Göran Elinder,Alexandra H. Filipovich,Helmut Gadner,Shinsaku Imashuku,Diane M. Komp,Stephan Ladisch,David Webb,Gritta Janka +11 more
TL;DR: HLH-94 is very effective, allowing BMT in most patients, and survival of children with HLH has been greatly improved.
Journal ArticleDOI
Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society.
TL;DR: A general picture of the disease is presented that includes the clinical, laboratory, and histological findings and reflects the difficulties in making the diagnosis of this frequently misunderstood disorder.