A Multicenter Study of Patients with Multisystem Langerhans Cell Histiocytosis Who Develop Secondary Hemophagocytic Lymphohistiocytosis
Por:
Chellapandian D, Hines MR, Zhang R, Jeng M, van den Bos C, Salvador-Marcos N, Lehmberg K, Sieni E, Wang Y, Nakano T, Williams JA, Fustino NJ, Astigarraga I, Dunkel IJ, Abla O, van Halteren AGS, Pei D, Cheng C, Weitzman S, Sung L and Nichols KE
Publicada:
15 mar 2019
Ahead of Print:
6 dic 2018
Resumen:
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the presence of abnormal CD1a-positive (CD1a(+))/CD207(+) histiocytes. Hemophagocytic lymphohistiocytosis (HLH) represents a spectrum of hyperinflammatory syndromes typified by the dysregulated activation of the innate and adaptive immune systems. Patients with LCH, particularly those with multisystem (MS) involvement, can develop severe hyperinflammation mimicking that observed in HLH. Nevertheless, to the authors' knowledge, little is known regarding the prevalence, timing, risk factors for development, and outcomes of children and young adults who develop HLH within the context of MS-LCH (hereafter referred to LCH-associated HLH). METHODS: To gain further insights, the authors conducted a retrospective, multicenter study and collected data regarding all patients diagnosed with MS-LCH between 2000 and 2015. RESULTS: Of 384 patients with MS-LCH, 32 were reported by their primary providers to have met the diagnostic criteria for HLH, yielding an estimated 2-year cumulative incidence of 9.3% +/- 1.6%. The majority of patients developed HLH at or after the diagnosis of MS-LCH, and nearly one-third (31%) had evidence of an intercurrent infection. Patient age <2 years at the time of diagnosis of LCH; female sex; LCH involvement of the liver, spleen, and hematopoietic system; and a lack of bone involvement each were found to be independently associated with an increased risk of LCH-associated HLH. Patients with MS-LCH who met the criteria for HLH had significantly poorer 5-year survival compared with patients with MS-LCH who did not meet the criteria for HLH (69% vs 97%; P < .0001). CONCLUSIONS: Given its inferior prognosis, further efforts are warranted to enhance the recognition and optimize the treatment of patients with LCH-associated HLH. (C) 2018 American Cancer Society.
Filiaciones:
Chellapandian D:
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada
Blood and Marrow Transplant Program, Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St. Petersburg, Florida
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee
Hines MR:
Division of Critical Care, Department of Pediatric Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee
Zhang R:
Hematology and Oncology Center, Beijing Children's Hospital, Beijing, China
Jeng M:
Division of Pediatric Hematology/Oncology, Lucile Packard Children's Hospital Stanford, Palo Alto, California
van den Bos C:
Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands
Salvador-Marcos N:
Department of Pediatric Hematology/Oncology, Sant Joan de Deu Hospital, Barcelona, Spain
Lehmberg K:
Department of Paediatric Haematology/Oncology, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
Sieni E:
Department of Paediatric Haematology/Oncology, Meyer Children's University Hospital, Florence, Italy
Wang Y:
Department of Medical Oncology, Beijing Friendship Hospital, Beijing, China
Nakano T:
Pediatric Hematology/Oncology, Children's Hospital Colorado, University of Colorado School of Medicine, Denver, Colorado
Williams JA:
Division of Pediatric Hematology/Oncology, Phoenix Children's Hospital, Phoenix, Arizona
Fustino NJ:
Pediatric Hematology/Oncology, Blank Children's Hospital, Des Moines, Iowa
Astigarraga I:
Department of Paediatric Haematology/Oncology, BioCruces Health Research Institute, Cruces University Hospital, Bizkaia, Spain
Dunkel IJ:
Pediatric Oncology, Memorial Sloan Kettering Cancer Center, New York, New York
Abla O:
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada
van Halteren AGS:
Immunology Laboratory, Willem-Alexander Children's Hospital, Leiden University Medical Center, Leiden, The Netherlands
Pei D:
Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, Tennessee
Cheng C:
Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, Tennessee
Weitzman S:
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada
Sung L:
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada
Nichols KE:
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee
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