Nogués-Castell Javier, Feu-Basilio Silvia, Felguera García Óscar, Fernández de Larrea Carlos, Oliver-Caldés Aina, Balagué Ponz Olga, Fassi Jessica Matas
Institut Clínic d'Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain.
Institut D' Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain.
Front Oncol. 2023 Aug 10;13:1217714. doi: 10.3389/fonc.2023.1217714. eCollection 2023.
Plasma cell leukemia (PCL) is an aggressive and rare form of plasma cell dyscrasia characterized by peripheral blood expression, poor prognosis, and high relapse rates. Extramedullary plasmacytomas are common in this entity and can affect various organs and soft tissues. Chimeric antigen receptor-T-cell (CAR-T) therapy is a novel immunotherapy for hematological malignancies with promising results. However, it is not indicated for PCL, and experience in this condition is limited. This case is a rare presentation of bilateral orbital plasmacytomas after CAR-T therapy in a patient with PCL history.
We present the case of a 51-year-old female patient with a history of previous primary PCL treated with CAR-T therapy achieving complete response and without evidence of systemic progression. Six months after the treatment, she developed subacute proptosis and ptosis on the left eye.An orbital CT scan was performed and showed an orbital tumor in both eyes. A surgical biopsy with histological examination revealed plasma cells, consistent with a plasmacytoma. PET-CT and MRI confirmed the presence of tumors in both orbits. The patient was treated with dexamethasone and chemotherapy along with palliative radiation therapy to the left orbit which had a good response.
Orbital involvement in multiple myeloma and PCL is rare, with plasmacytomas being more common in other parts of the body. In this report, we present a case of a patient with PCL history, treated with multiple therapeutic lines including CAR-T therapy, who presented bilateral orbital plasmacytomas as the first sign of extramedullary progression after the treatment. This case should be considered by specialist to be aware that the orbits are a possible location of extramedullary progression.
浆细胞白血病（PCL）是一种侵袭性且罕见的浆细胞发育异常形式，其特征为外周血表达、预后不良及高复发率。髓外浆细胞瘤在该疾病实体中较为常见，可累及各种器官和软组织。嵌合抗原受体T细胞（CAR-T）疗法是一种用于血液系统恶性肿瘤的新型免疫疗法，取得了令人鼓舞的结果。然而，它并不适用于PCL，且在这种情况下的经验有限。本病例是一名有PCL病史的患者在接受CAR-T治疗后出现双侧眼眶浆细胞瘤的罕见表现。
我们报告一例51岁女性患者，既往有原发性PCL病史，接受CAR-T治疗后达到完全缓解且无全身进展证据。治疗6个月后，她出现左眼亚急性眼球突出和上睑下垂。进行了眼眶CT扫描，显示双眼均有眼眶肿瘤。手术活检及组织学检查显示为浆细胞，符合浆细胞瘤。PET-CT和MRI证实双侧眼眶均有肿瘤。患者接受了地塞米松和化疗，并对左眼进行了姑息性放射治疗，反应良好。
眼眶受累在多发性骨髓瘤和PCL中罕见，浆细胞瘤在身体其他部位更为常见。在本报告中，我们呈现了一例有PCL病史的患者，接受了包括CAR-T治疗在内的多种治疗方案，在治疗后出现双侧眼眶浆细胞瘤作为髓外进展的首发体征。专科医生应考虑到这种情况，意识到眼眶是髓外进展的一个可能部位。
