Primary synovial non-Hodgkin's lymphoma in association with ankylosing spondylitis.
Primary synovial non-Hodgkin's lymphoma in association with ankylosing spondylitis.
复制标题
原发性滑膜非霍奇金淋巴瘤与强直性脊柱炎相关。
DOI：
10.1093/rheumatology/keh015
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发表时间：
2003
期刊：
影响因子：
5.5
通讯作者：
D. Hutchinson
中科院分区：
文献类型：
--
作者：
S. Khan;D. Hutchinson
先生，一位65岁的男士，有3个月的左膝疼痛、肿胀和左腹股沟淋巴结肿大的病史。没有体重减轻或发烧的症状。他有长期强直性脊柱炎的背景，伴有左膝炎症，1984年需要关节镜检查。这显示了广泛的滑膜炎，但活检没有更多的特异性。膝关节症状在数月内消退。左膝平片显示一些骨质破坏，伴有明显的大量积液。临床表现为大量积液，但液体抽吸困难，滑膜呈木质，滑液带血，未见恶性细胞。总白色血细胞计数为1.3 × 10 9 /l（多形核细胞26%，单核细胞3%）。炎症标志物仅轻度升高，红细胞沉降率为21 mm/h，C反应蛋白浓度为25 mg/l。全血细胞计数正常，乳酸脱氢酶361 U/l（正常范围211-423）。MRI扫描显示骨侵蚀伴骨髓水肿，影响股骨远端前部以及股骨内侧髁和髁间区域的胫骨（图1）。存在影响髌上囊的严重滑膜肥大。股骨后方可见一3cm肿块，信号特征与滑膜相似，但与关节无关。这被认为是一个扩大的腘淋巴结。进行关节镜检查，并采取滑膜活检，这表明弥漫性浸润的细胞与不规则和肾形核。渗透似乎是炎症。网硬蛋白染色显示纤维围绕细胞群而不是单个细胞。肿瘤对LCA（白细胞共同抗原）、L26和CD 79 a呈强阳性。有一些CD 68和点状CD 3阳性。VS 38也是阳性。免疫化学显示细胞主要为B细胞，所有T细胞标记均为阴性。组织学表现为弥漫性大B细胞非霍奇金淋巴瘤。对左腹股沟淋巴结进行活检。外观再次与大B细胞淋巴瘤一致。CD 10阳性表明，这可能最初是卵泡中心细胞起源。骨髓活检显示没有证据表明涉及非霍奇金淋巴瘤（NHL）。分期CT扫描，他的胸部，腹部和骨盆没有发现异常以外的髂外淋巴结肿块测量2.7 - 2.5厘米。六个疗程的化疗（CHOP）和五个疗程的抗CD 20治疗（利妥昔单抗）产生了戏剧性的反应，他的膝盖恢复正常。在NHL中，高达25%的病例可累及骨骼。滑膜受累较少见，通常是由于骨的直接延伸[1]。在这种情况下，滑膜被认为是肿瘤的原发部位，因为滑膜肥大的程度与骨受累的数量相比。文献中报告的原发性滑膜结节性NHL病例很少，仅报告了2例与强直性脊柱炎相关的淋巴瘤病例，均不影响膝关节[2，3]。NHL滑膜受累的MRI表现在文献中仅报告过一次[4]。
SIR, A 65-yr-old gentleman presented with a 3-month history of a painful, swollen left knee associated with left inguinal lymphadenopathy. There had been no symptoms of weight loss or fevers. There was a background of longstanding ankylosing spondylitis associated with inflammation of his left knee, requiring arthroscopy in 1984. This had shown extensive synovitis but nothing more specific on biopsy. The knee symptoms resolved over a period of months. A plain film of the left knee had shown some bony destruction associated with an apparent large effusion. Clinically there was a large effusion but aspiration of fluid was difficult and the synovium had a wooden quality to it. The synovial fluid was bloodstained and no malignant cells were seen. The total white blood cell count was 1.3 10 9 /l (polymorphonuclear cells 26%, monocytes 3%). Markers of inflammation were raised only modestly, erythrocyte sedimentation rate was 21 mm/h and C-reactive protein concentration 25 mg/l. The full blood count was normal and lactate dehydrogenase 361 U/l (normal range 211–423). An MRI scan demonstrated bony erosion with bone marrow oedema affecting the distal femur anteriorly and the medial femoral condyle and the tibia in the intercondylar region (Fig. 1). There was gross synovial hypertrophy affecting the suprapatellar pouch. A 3cm mass was seen posterior to the femur; it had similar signal characteristics to synovium but no connection with the joint could be seen. This was thought to be an enlarged popliteal lymph node. Arthroscopy was undertaken and synovial biopsies were taken, which revealed a diffuse infiltrate of cells with irregular and kidneyshaped nuclei. The infiltrate appeared to be inflammatory. Reticulin stain showed fibres around groups of cells rather than around individual cells. The tumour was strongly positive for LCA (leucocyte common antigen), L26 and CD79a. There was some CD68 and spotty CD3 positivity. VS38 was also positive. Immunochemistry showed the cells to be predominantly B cells, all T-cell markers being negative. The histological appearances were those of a diffuse large B-cell non-Hodgkin’s lymphoma. A biopsy of the left inguinal lymph node was undertaken. The appearance was again consistent with that of a large B-cell lymphoma. Positivity for CD10 suggested that this might initially have been of follicle centre cell origin. A bone marrow biopsy showed no evidence of involvement with non-Hodgkin’s lymphoma (NHL). A staging CT scan of his thorax, abdomen and pelvis revealed no abnormality other than an external iliac lymph node mass measuring 2.7 2.5 cm. Six courses of chemotherapy (CHOP) and five courses of anti-CD20 therapy (rituximab) resulted in a dramatic response, with his knee returning back to normal. In NHL there can be skeletal involvement in up to 25% of cases. Synovial involvement is less common and is usually due to direct extension from bone [1]. In this case the synovium was felt to be the primary site of the tumour due to the extent of the synovial hypertrophy compared with the amount of bony involvement. There have been few cases of primary synovial extranodal NHL reported in the literature and only two cases of lymphoma reported in association with ankylosing spondylitis, neither affecting the knee [2, 3]. The MRI appearances of synovial involvement from NHL have only been reported once in the literature [4].