Primary synovial non-Hodgkin's lymphoma in association with ankylosing spondylitis

S. Y. Khan1,2 and D. G. Hutchinson2

1Rheumatology Department, Queen Alexandra Hospital, Southwick Hill Road, Portsmouth and 2Rheumatology Unit, Royal Cornwall Hospital, Truro, UK

Correspondence to: D. G. Hutchinson, Rheumatology Unit, Royal Cornwall Hospital, Truro, Cornwall TR1 3LJ, UK. E-mail: david.hutchinson{at}rcht.swest.nhs.uk

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 x 109/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 3-cm 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 kidney-shaped 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.



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FIG. 1. MRI showing a T1-weighted image with intravenous gadolinium. This demonstrates grossly thickened synovium, a small joint effusion and bony erosion of the distal femur and patella. A 3 cm lymph node is seen in the popliteal fossa.

 
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 x 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].

The authors have declared no conflicts of interest.

References

  1. Dorfman HD, Siegel HL, Perry MC, Oxenhandler R. Non-Hodgkin's lymphoma of the synovium simulating rheumatoid arthritis. Arthritis Rheum 1987;30:155–61.[ISI][Medline]
  2. Jantunen E, Myllykangas-Luosujärvi R, Kaipiainen-Seppänen O, Nousiainen T. Autologous stem cell transplantation in a lymphoma patient with a long history of ankylosing spondylitis. Rheumatology 2000;39:563–4.[Free Full Text]
  3. Gau JP, Chiu CF, Liu SM, Hsu HC, Ho CH, Chau WK. Angiotropic lymphoma manifesting as a solitary adrenal tumour in a case of ankylosing spondylitis. Zhonghua Yi Xue Za Zhi 2000;63:62–5.[Medline]
  4. Jamieson KA, Beggs I, Robb JE. Synovial presentation of non-Hodgkin's lymphoma. Br J Radiol 1998;71:980–2.[Abstract/Free Full Text]
Accepted 21 July 2003





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