Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy.
Anatomia Patologica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Via Conca 71, 60126 Ancona, Italy.
Exp Hematol Oncol. 2015 Jan 14;4(1):3. doi: 10.1186/2162-3619-4-3. eCollection 2015.
Castleman-Kojima disease (TAFRO Syndrome) is characterized by Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly, multiple lymphadenopathy and histopathology pattern of atypical Castleman's disease (CD). Only few cases of this recently identified unique variant of Multicentric CD (MCD) are described in literature, all Japanese. It therefore poses serious diagnostic and therapeutic challenges.
We describe a 21 year old woman with fever, asthenia, bilateral pleural effusion, ascites, hypoalbuminemia, severe thrombocytopenia, anemia, renal failure and proteinuria, whereas microbiological tests, immune serology (except ANA) and bone marrow biopsy were all negative. A CT-scan showed multiple lymphadenopathy and tissue samplings of mediastinal lymph nodes was compatible with a mixed-type CD. The diagnosis of MCD with TAFRO syndrome was made, but after an initial improvement with high dose corticosteroid therapy, clinical and laboratory features worsened. Based upon the high serum IL-6 levels and the high number of CD20-lymphocytes in lymph nodes tissue, we started tocilizumab (partial benefit), followed by rituximab combined with CVP (cyclophosphamide, vincristine and prednisone) chemotherapy, achieving a complete response. A total of six cycles of R-CVP were administered monthly, followed by maintenance with monthly rituximab. A complete remission persists at the 12th month of follow-up.
In patients with massive immune system activation and lymphadenopathy it is mandatory to rule out Castleman-Kojima disease. In our patient a therapy aimed at the prominent pathophysiological abnormalities has been successful so far. However, since the rarity of TAFRO Syndrome, a multicenter registry is strongly desirable for a better understanding of the disease mechanisms, hopefully leading to evidence-based therapeutic choices.
卡斯特曼-科乔玛病（TAFRO 综合征）的特征是血小板减少症、全身性水肿、骨髓纤维化、肾功能障碍、器官肿大、多发性淋巴结病和非典型卡斯特曼病（CD）的组织病理学模式。这种新发现的独特的多中心 CD（MCD）变体在文献中仅描述了少数几个病例，且均为日本人。因此，它带来了严重的诊断和治疗挑战。
我们描述了一名 21 岁女性，她有发热、乏力、双侧胸腔积液、腹水、低白蛋白血症、严重血小板减少症、贫血、肾衰竭和蛋白尿，而微生物学检查、免疫血清学（除 ANA 外）和骨髓活检均为阴性。CT 扫描显示多发性淋巴结病，纵隔淋巴结组织取样符合混合性 CD。诊断为 MCD 伴 TAFRO 综合征，但在大剂量皮质类固醇治疗后最初有所改善，临床和实验室特征恶化。基于高血清 IL-6 水平和淋巴结组织中高数量的 CD20 淋巴细胞，我们开始使用托珠单抗（部分受益），随后联合 CVP（环磷酰胺、长春新碱和泼尼松）化疗，达到完全缓解。共进行了六个周期的 R-CVP 治疗，每月一次，随后每月进行利妥昔单抗维持治疗。在随访的第 12 个月，完全缓解仍在持续。
在存在大量免疫系统激活和淋巴结病的患者中，必须排除卡斯特曼-科乔玛病。在我们的患者中，针对突出的病理生理异常的治疗迄今为止是成功的。然而，由于 TAFRO 综合征的罕见性，强烈需要进行多中心登记，以更好地了解疾病机制，有望为基于证据的治疗选择提供依据。
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