A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis
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A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis. / Chellapandian, Deepak; Hines, Melissa R; Zhang, Rui; Jeng, Michael; van den Bos, Cor; Santa-María López, Vicente; Lehmberg, Kai; Sieni, Elena; Wang, Yini; Nakano, Taizo; Williams, James A; Fustino, Nicholas J; Astigarraga, Itziar; Dunkel, Ira J; Abla, Oussama; van Halteren, Astrid G S; Pei, Deqing; Cheng, Cheng; Weitzman, Sheila; Sung, Lillian; Nichols, Kim E.
In: CANCER-AM CANCER SOC, Vol. 125, No. 6, 15.03.2019, p. 963-971.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis
AU - Chellapandian, Deepak
AU - Hines, Melissa R
AU - Zhang, Rui
AU - Jeng, Michael
AU - van den Bos, Cor
AU - Santa-María López, Vicente
AU - Lehmberg, Kai
AU - Sieni, Elena
AU - Wang, Yini
AU - Nakano, Taizo
AU - Williams, James A
AU - Fustino, Nicholas J
AU - Astigarraga, Itziar
AU - Dunkel, Ira J
AU - Abla, Oussama
AU - van Halteren, Astrid G S
AU - Pei, Deqing
AU - Cheng, Cheng
AU - Weitzman, Sheila
AU - Sung, Lillian
AU - Nichols, Kim E
N1 - © 2018 American Cancer Society.
PY - 2019/3/15
Y1 - 2019/3/15
N2 - 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.
AB - 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.
KW - Journal Article
U2 - 10.1002/cncr.31893
DO - 10.1002/cncr.31893
M3 - SCORING: Journal article
C2 - 30521100
VL - 125
SP - 963
EP - 971
JO - CANCER-AM CANCER SOC
JF - CANCER-AM CANCER SOC
SN - 0008-543X
IS - 6
ER -
