Case Report
J Indian Rheumatol Assoc 2004 : 12 : 153 - 155
ANGIOIMMUNOBLASTIC LYMPHADENOPATHY PRESENTING AS INFLAMMATORY POLYARTHRITIS Charu G Sachdeva 1, Atul Kakar 2, P S Gupta 3, S Bhalla4 1-Senior Resident, Medicine, 2, 3 - Consultants Medicine & Immunorheumatology Clinic, 4-consultant Histopathology
Abstract : Angioimmunoblastic lymphadenopathy with dysproteinemia is a peripheral T cell lymphoma which can present with variety of rheumatological manifestations. Herein an elderly male with symmetric inflammatory polyarthritis, mimicking rheumatoid arthritis is presented. In addition he had constitutional symptoms, genralized lymphadenopathy and hepato-splenomegaly. Cervical lymphnode biopsy clinched the diagnosis. The case highlights the need to consider this entity as a possibility eespecially in setting of acute onset inflammatory polyarthritis in an elderly. Key Words: Lymphoma, angioimmunoblastic lymphadenopathy, polyarthritis Case history A 65 year old male, presented elsewhere with complaints of joint pains for 2 months involving proximal inter-phalangeal, knees and shoulder joints, bilaterally. He gave history of morning stiffness lasting for 30 minutes and worsening of pain at rest. He was afebrile and joint examination revealed swelling of proximal inter-phalangeal joints and painful restriction of movements of knee joints. Systemic examination was normal. Investigations revealed raised ESR, positive CRP and negative rheumatoid factor. Blood counts, liver and renal parameters were normal. A working diagnosis of acute inflammatory polyarthritis was made and he was started on methylprednisolone acetate 80 mg weekly for 4 weeks. Joint pains subsided and he could carry out his daily activities. He presented to us, two weeks after the last steroid injection, with fever, dry cough and breathlessness. Fever was continuous and associated with night sweats. He lost 9 kg of weight in 2 months and had intractable itching not relieved by antihistaminic. ExAddress for correspondence Dr. Atul Kakar 31, South Patel Nagar, New Delhi – 110 008. E.mail :
[email protected]
amination revealed fever (101°F), pulse rate 110/min, BP 100/70 mmHg, significant pallor and multiple pallpable left cervical lymph nodes. These nodes measured 1-2 cm, were non tender and not fixed to the underlying tissues. Chest examination revealed bilateral crepitations. Abdominal examination revealed hepatosplenomegaly. Musculoskeletal examination showed swelling of the knee joints and mildly painful movements at these joints, other joints being normal. Investigations showed hemoglobin 7.1 gm/dl, direct Coomb’s test positive, TLC -5400/cu mm, DLC – N68%, L25%, E6% M1%, ESR -120 mm 1st hr, platelets 5.12 lakhs, blood and urine cultures were sterile. There was renal impairment with BUN/Cr.-57.4/ 3.1 mg%, and hypergammaglobulinemia with S.Protein./ albumin 7.0/2.7 gm%, IgA – 657 mg/dl (N 35-350), IgG 2535 (N 650-1600), IgM 296 mg/dl and SGOT/ SGPT/SAP 40/30/70 IU/L respectively. Immunological markers (ANA, Rheumatoid Factor, C-ANCA, PANCA) were negative. Antibodies to HIV 1 & 2 – negative, Chest X-ray showed bilateral pneumonitis and serum electrophoresis was suggestive of polyclonal gammaglobulinemia. CT abdomen showed enlarged lymph nodes in para-portal, para-aortic, left and right iliac fossa region with hepatosplenomegaly. Cervical
Charu G Sachdeva et al
lymph node biopsy done was suggestive of angioimmunoblastic lympadenopathy (figure-1) with special stains positive for CD20, CD30 cells. Bone marrow aspiration and biopsy revealed mild eosinophils.
Figure 1 The section shows many blood vessels and immature lymphoid cells
Patient was treated with antibiotics and his renal profile improved, subsequently he was given 8 cycles of CHOP therapy along with rituximab for a period of 8 months and had a remarkable recovery. Discussion This case had 2 rare features. Firstly, the diagnosis of angioimmunoblastic lympadenopathy which is a rare tumor comprising only 1-2% of non Hodgkin’s lymphomas1. This is usually a T-cell lymphoma but our patient had both T and B cell markers for which reason he could be given rituximab which is an anti CD20 antibody. The other rare feature is the initial manifestation of inflammatory polyarthritis which antedated the diagnosis of lymphoma. Angioimmunoblastic lympadenopathy is a lymphoproliferative disorder of well established clinical and histological features. According to the Revised European American Lymphoma (REAL) classification this is one of the peripheral post-thymic T cell lymphoma. It is a rare lymphoma and in a series of 3194 cases of lymphoma in Japan, 35% were diagnosed as angioimmunoblastic lympadenopathy2. It affects patients
in later life mainly above the age of 40 years. The typical features are pyrexia, weight loss (58%), rash (4050%), pruritis, night sweats and arthralgias (20%), generalized lymphadenopathy (87%), hepato-splenomegaly and hemolytic anemia, hypergammaglobulinemia, skin involvement (morbilliform or sclero-myxedema like) and renal failure are other common features1. Thus the manifestations are not specific and may resemble SLE, Sjogren’s syndrome, mixed cryoglobulinemia and adult onset still’s disease3. The gold standard for diagnosis is the characteristic lymph node histology which shows an effacement of normal architecture associated with prominent vascular arborization and polymorphic cell lymph nodes which includes immunoblasts, plasma cells, lymphocytes, eosinophils and histiocytes. Arthralgias may be common presenting complaints in lympadenopathy but arthritis is a rare feature. In angioimmunoblastic lympadenopathy, very few cases of arthritis have been reported in literature3-5. Articular involvement in immunoblastic lymphadenopathy can vary from mild non-deforming to erosive and deforming polyarthritis. Usually arthritis is seronegative. Raskin et al reported a patient with severe arthritis with contractures 3 of the joints . Our patient presented with arthritis which antedated the diagnosis of angioimmunoblastic lympadenopathy by 10 weeks, similar to earlier reports 4-6 . Davis et al had reported a 73 year old male in whom arthritis preceded the diagnosis of angioimmunoblastic lympadenopathy by 18 months. He had both large and small joints involvement and was seronegative for RF. Radiology of joints did not show any erosion. He did not respond to aspirin or naproxen and was later treated with azathioprine. Eighteen months later he developed 4 angioimmunoblastic lympadenopathy . In another such patient, a 60 year old female, the arthritis preceded the development of angioimmunoblastic lympadenopathy 154
Angioimmunoblastic lymphadenopathy presenting as inflammatory polyarthritis
by 4 months5. She too was seronegative for RF. Radiograph showed periarticular osteoporosis and soft tissue swelling, but no erosions. To control the arthritis ASA, indomethacin and gold were used. Although initially thought to be benign, it is now known that angioimmunoblastic lympadenopathy carries 50% mortality at 12 months reaching 90% if no remission is achieved with chemotherapy. References 1. 2.
Scheinfeld NS. Angioimmunoblastic lymphadenopathy with Dysproteinemia. eMedicine 2003; 1-8. Lymphoma study group of Japanese pathologists (2000),
3. 4. 5. 6.
World Health Organization Classification of malignant lymphoma in Japan: Incidence of recently recognized entities. Pathology International 2000; 50: 696-702. Raskin HJ, Tesar JT, Lawless OJ. Polyarthralgias in immunoblastic lymphadenopathy. Arthritis Rheum 1982; 25:1481-1485. Davis PG, Fordham JN. Arthritis and angioimmunoblastic Lymphadenopathy. Ann Rheum 1983; 42: 516-518. McHugh NJ, Campbell GJ, Landreth JJ, Laurent MR. Polyarthritis and angioimmunoblastic lymphadenopathy. Ann Rheum Dis 1987; 46: 555-558. Pay SI, Dinc A, Simsek I, Can C, Erdem H. Sulfasalazine induced angioummunoblastic lymphadenopathy developing in a patient with juvenile chronic arthritis. Rheumatol Int 2000; 20: 25-7.
CORRIGENDUM The designation of Dr Nausser Ahmad Khan B and Dr Ajit Kumar was incorrect in the paper entitled " Silent Gut inflammation in seronegative spondyloarthropathy " J Indian Rheumatol Assoc 2004;12: 91-97. Dr Nausser Ahmad Khan is a Post graduate student in the Department of Medicine and Dr Ajit Kumar is Additional Professor and Head of the department of Gastroenterology at the Nizam Institute of Medical Sciences, Hyderabad. The mistake is regretted.
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