Research Papers:
Prognostic factors for histiocytic and dendritic cell neoplasms
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Abstract
Joji Shimono1,3, Hiroaki Miyoshi1, Fumiko Arakawa1, Kensaku Sato1, Takuya Furuta1, Reiji Muto1, Eriko Yanagida1, Yuya Sasaki1, Daisuke Kurita1, Keisuke Kawamoto1, Koji Nagafuji2 and Koichi Ohshima1
1Department of Pathology, Kurume University, School of Medicine, Kurume, Japan
2Department of Hematology, Kurume University, School of Medicine, Kurume, Japan
3Department of Hematology, Hokkaido University Graduate School of Medicine, Sapporo, Japan
Correspondence to:
Hiroaki Miyoshi, email: [email protected]
Keywords: dendritic cell neoplasm; histiocytic sarcoma; survival; prognostic factor; BRAF
Received: June 05, 2017 Accepted: September 21, 2017 Published: October 19, 2017
ABSTRACT
Histiocytic and dendritic cell neoplasms are rare and poorly studied. We report the clinical characteristics and prognostic factors in such cases in Japan. We investigated the clinical characteristics and survival in 87 adult patients with histiocytic and dendritic cell neoplasms. Fifty patients had histiocytic sarcoma, 12 had Langerhans cell histiocytosis, 11 had follicular dendritic cell sarcoma, 8 had Langerhans cell sarcoma, 6 had interdigitating cell sarcoma and 1 had indeterminate dendritic cell sarcoma. The median follow-up period was 18.0 (range: 9.6-71.8) months, and median overall survival (OS) was 23.5 months. The 2-year OS rate was 49.2%. In the multivariate analysis, elevated lactate dehydrogenase (LDH) (p =.004), ECOG performance status (PS) 2-4 (p =.006), and Ann Arbor stage III-IV (p =.008) affected OS. Stratification by elevated LDH, ECOG PS 2-4, and Ann Arbor stage III-IV allowed classification of patients into low risk, intermediate risk, and high risk groups. The same classification was applicable for HS and non-HS categories. In the rare neoplasms of histiocytic and dendritic cell sarcoma, ECOG PS, Ann Arbor stage, and LDH are important prognostic factors for predicting survival.
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