Kawase Kengo, Naiki Taku, Naiki-Ito Aya, Yanada Masamitsu, Ikegami Yosuke, Ota Yuya, Hattori Tatsuya, Matsuyama Nayuka, Hamakawa Takashi, Maruyama Tetsuji, Yasui Takahiro
Department of Nephro-Urology Graduate School of Medical Sciences Nagoya City University Nagoya Aichi Japan.
Department of Urology Nagoya City East Medical Center Nagoya Aichi Japan.
IJU Case Rep. 2019 Jun 17;2(4):232-235. doi: 10.1002/iju5.12096. eCollection 2019 Jul.
Richter syndrome refers to the transformation from chronic lymphocytic leukemia to assaultive lymphoma, often a diffuse large B-cell lymphoma, and has a greatly poor prognosis. Richter syndrome is characterized by rapidly growing lymphadenopathy but rarely presents with extra-nodal involvement, common sites being the digestive tract, lungs, kidneys, and central nervous system. However, Richter syndrome with testicular involvement is extremely rare.
Herein we report a very scare case of a male at the age of 72 with Richter syndrome and testicular involvement, diagnosed by the investigation of bilateral scrotal swellings. The patient had attained disease-free survival for over a year with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, and the intrathecal administration of chemotherapeutic agents after diagnosis by immediate orchiectomy.
An early pathological diagnosis by immediate orchiectomy and the early initiation of induction immunochemotherapy may be good prognostic factors in Richter syndrome involving the testes.
里氏综合征是指慢性淋巴细胞白血病转化为侵袭性淋巴瘤,通常为弥漫性大B细胞淋巴瘤,预后极差。里氏综合征的特征是淋巴结病迅速发展,但很少出现结外受累,常见部位为消化道、肺、肾和中枢神经系统。然而,累及睾丸的里氏综合征极为罕见。
在此,我们报告一例极为罕见的72岁男性里氏综合征伴睾丸受累病例,通过双侧阴囊肿胀检查确诊。患者在接受利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松治疗后,无病生存超过一年,并在诊断后立即行睾丸切除术后鞘内注射化疗药物。
立即行睾丸切除术进行早期病理诊断以及早期开始诱导免疫化疗可能是里氏综合征累及睾丸时的良好预后因素。