Hadjadj Jérôme, Nielly Hubert, Piekarski Eve, Cuccuini Wendy, Deau-Fischer Bénedicte, Hourseau Muriel, Benali Khadija, Fieschi Claire, Aletti Marc, Papo Thomas, Oksenhendler Eric, Galicier Lionel, Boutboul David
Clinical Immunology Department, Hôpital Saint Louis, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, 1 Avenue Claude Vellefaux, Paris, France.
Internal Medicine and Rheumatology Department, Percy Military Teaching Hospital, 101 Avenue Henri Barbusse, Clamart, France.
Ann Hematol. 2017 Nov;96(11):1891-1896. doi: 10.1007/s00277-017-3117-4. Epub 2017 Aug 29.
Primary intravascular large B cell lymphoma (IVL) remains a diagnostic challenge because of non-specific clinical, laboratory and imaging findings. The aim of the study was to analyse the major characteristics of IVL with uterine involvement. We retrospectively collected features of IVL with uterine involvement that was proven histologically or demonstrated by significant FDG uptake on FDG-PET/CT. Findings were compared to a comprehensive literature review. Five patients were identified. All of them were admitted for fever of unknown origin (FUO), with haemophagocytic lymphohistiocytosis in three cases. None had gynaecological symptom, contrasting with the literature data. Structural imaging (including whole-body CT scan and pelvic RMI) failed to yield any diagnosis. FDG-PET/CT showed intense uterine uptake in all cases. Endometrial biopsy was performed in three cases and was positive in one. Diagnosis was obtained from coelioscopic iliac adenopathy biopsy in one case and from total hysterectomy in another. Punch biopsy of skin lesions led to diagnosis in the two remaining cases. Bone marrow biopsy was normal in all cases. Clinicians should be aware of potential isolated uterine involvement in IVL, especially in elderly women with FUO. Normal structural imaging does not rule out the diagnosis and FDG-TEP/CT should be performed to guide high-yielding biopsy.
原发性血管内大B细胞淋巴瘤（IVL）由于其非特异性的临床、实验室和影像学表现，仍然是一个诊断难题。本研究的目的是分析累及子宫的IVL的主要特征。我们回顾性收集了经组织学证实或在FDG-PET/CT上表现为显著FDG摄取的累及子宫的IVL的特征。将研究结果与全面的文献综述进行比较。共确定了5例患者。所有患者均因不明原因发热（FUO）入院，其中3例伴有噬血细胞性淋巴组织细胞增生症。与文献数据不同的是，所有患者均无妇科症状。结构成像（包括全身CT扫描和盆腔磁共振成像）均未能确诊。FDG-PET/CT显示所有病例子宫均有强烈摄取。3例行子宫内膜活检，1例阳性。1例通过腹腔镜髂淋巴结活检确诊，另1例通过全子宫切除术确诊。其余2例通过皮肤病变穿刺活检确诊。所有病例骨髓活检均正常。临床医生应意识到IVL可能存在孤立的子宫受累情况，尤其是在患有FUO的老年女性中。正常的结构成像不能排除诊断，应进行FDG-TEP/CT以指导高阳性率的活检。
