Saburi Masuho, Kondo Yoshiyuki, Ogata Masao, Soga Yasuhiro, Abe Miyuki, Takano Kuniko, Kohno Kazuhiro, Nagai Takayuki, Nakayama Toshiyuki
Department of Medical Oncology and Hematology, Faculty of Medicine, Oita University, 1-1 Hasama Idaigaoka, Yuhu City, Oita, 879-5593, Japan.
Department of Medical Oncology and Hematology, Faculty of Medicine, Oita University, Oita, Japan.
Int J Hematol. 2020 Nov;112(5):658-665. doi: 10.1007/s12185-020-02957-z. Epub 2020 Aug 1.
The incidence and clinical characteristics of histological transformation (HT) from duodenal type follicular lymphoma (DFL) are unclear. A retrospective analysis was conducted to identify the incidence and clinical features of HT from DFL in 23 cases with DFL. The median follow-up duration was 4.6 years (range, 0.8-20 years). HT to diffuse large B-cell lymphoma was observed in 2 of 23 cases during follow-up (8.7%). One of two cases transformed at 21 months later with increased serum lactate dehydrogenase (LDH; 1655 U/L) and abdominal lymphadenopathy. Partial response was achieved after R-THP (pirarubicin)-COP therapy, but the disease progressed. The other case transformed at 8.3 years with an increase of serum LDH (4022 U/L), abdominal lymphadenopathy, and bone marrow involvement. The disease was refractory to DA-EPOCH-R and a high-dose methotrexate/cytarabine regimen. The patient received allogenic peripheral blood stem cell transplantation and finally achieved complete response. Both cases developed HT at nodal or other intestinal lesions with no progression of the primary duodenal lesion. No significant factors for the occurrence of HT were identified. Although the incidence is low, HT could occur in DFL with aggressive clinical manifestations.
十二指肠型滤泡性淋巴瘤(DFL)组织学转化(HT)的发生率及临床特征尚不清楚。我们进行了一项回顾性分析,以确定23例DFL患者中HT的发生率及临床特征。中位随访时间为4.6年(范围0.8 - 20年)。随访期间,23例中有2例发生了向弥漫性大B细胞淋巴瘤的转化(8.7%)。其中1例在21个月后转化,伴有血清乳酸脱氢酶(LDH)升高(1655 U/L)及腹部淋巴结肿大。R - THP(吡柔比星)- COP治疗后取得部分缓解,但疾病进展。另一例在8.3年时转化,伴有血清LDH升高(4022 U/L)、腹部淋巴结肿大及骨髓受累。该疾病对DA - EPOCH - R和大剂量甲氨蝶呤/阿糖胞苷方案耐药。患者接受了异基因外周血干细胞移植,最终获得完全缓解。两例均在淋巴结或其他肠道病变处发生HT,原发性十二指肠病变无进展。未发现HT发生的显著相关因素。尽管发生率较低,但HT可发生于具有侵袭性临床表现的DFL患者中。