Angioimmunoblastic T-Cell Lymphoma-Associated Arthritis

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma with systemic manifestations, including fever, lymphadenopathy, rash, and rarely arthritis. We report the case of a patient who presented with symmetric inflammatory polyarthritis and skin nodules resembling rheumatoid arthritis (RA). The patient responded initially to low-dose prednisolone, but 12 months later, he developed typical features of AITL. The characteristics of AITL-associated arthritis from 16 additional cases from the English literature are also reviewed. AITL-associated arthritis is an uncommon manifestation of angioimmunoblastic lymphoma that can mimic RA, especially when the typical systemic features of lymphoma are absent. This type of arthritis should be included in the differential diagnosis of patients presenting with an inflammatory polyarthritis.A ngioimmunoblastic T-cell lymphoma (AITL) is a nonHodgkin lymphoma (NHL) that was first described independently by Frizzera and Lukes in 1975. Initially, it was termed immunoblastic lymphadenopathy or angioimmunoblastic lymphadenopathy with dysproteinemia. In 1988, it was included in the Kiel Updated Classification for Lymphomas and it is currently classified as a peripheral T-cell non-Hodgkin lymphoma-nodal type in the World Health Organization classification of lymphomas. Currently, it accounts for 2% of all cases of NHLs. 1 Patients with AITL uncommonly develop an inflammatory arthritis with clinical features suggestive of rheumatoid arthritis (RA). 2 In the majority of cases, arthritis appears together or soon after the onset of lymphoma. Arthritis preceding the diagnosis of AITL for more than 6 months is extremely uncommon, having been reported only once in the literature. 3 We present the case of a male patient with symmetric inflammatory polyarthritis and skin nodules that preceded the onset of AITL by 12 months. We also review 16 additional well-described cases of AITL-associated arthritis from the English literature. 2-14 CASE REPORTA 50-year-old man presented to our department with a 1-month history of lymphadenopathy, fever, and rash. Twelve months before his admission, he had developed symmetric polyarthritis that involved the metacarpophalangeal (MCP), proximal interphalangeal (PIP), wrist, elbow, and ankle joints. Small, hard subcutaneous nodules less than 0.5 cm in size were also noted in the extensory surfaces of his elbows bilaterally. A full laboratory workup at that time, including antinuclear antibodies (ANA), rheumatoid factor (RF), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), was unremarkable. His medical history was significant only for Hashimoto thyroiditis.Six months before his admission, the patient reported a generalized erythematous nonpruritic skin rash. A skin biopsy revealed nonspecific perivascular inflammation of the dermis extending to the dermal-epidermal junction. The patient's rash and arthritis responded dramatically to lowdose prednisolone (10 mg/d orally). A month before his admission, while his prednisolon...

Advances in the understanding and management of angioimmunoblastic T‐cell lymphoma

“…Nodular lesions, plaques, purpura and urticarial lesions can also be seen (Martel et al , 2000). Other clinical signs and symptoms (arthralgias or arthritis, pleural effusions, ascitis and/or oedema, lung involvement, neurological manifestations, gastrointestinal involvement) are less common, with a wide variation in their reported frequency (Table I) (Bradley et al , 1981; Kaneki et al , 1999; Tsochatzis et al , 2005; Kang et al , 2007; Lachenal, 2007; Mourad et al , 2008). Overall, most patients have concomitant extranodal disease, and the disease is stage III or IV in more than 80% of cases.…”

Paraneoplastic Inflammatory Arthritis

“…Data regarding synovial fluid findings in paraneoplastic arthritis are limited. Previous studies reported a synovial fluid cell count ranging between 3,500 and 55,000/mm3 in patients diagnosed with lymphoma [8-9]. …”

Skin lesions and neutrophilic leukemoid reaction in a patient with angioimmunoblastic T‐cell lymphoma: a case report and review of the literature

“…In the literature, uncommon skin lesions of AITL are mostly described in case reports or review articles (Table 2). It is not rare that the patient has a generalized pleomorphic rash composed of several types of rashes, such as macula, papules, maculopapules, [30] 77/M Macules; petechiae; purpura 1 year before Alive (4 month) Seehafer [10] 74/M Petechiae Concurrent Died (3 month) Seehafer [10] 61/M Erythroderma and purpura Concurrent Alive (48 month) Seehafer [10] 57/M Petechiae 10 month before Alive (48 month) Schmuth [31] 73/F Macules; petechiae; purpura 4 week before Alive (4 month) Martel [15] Necrotic purpura, maculopapules and urticaria Died (26 day) Martel [15] Pruritic papulovesicular (prurigo-like) lesion Alive (96 month) Hashefi [32] Maculopapules, petechiae 3 month before Died (23 month) Suarez-Vilela [33] 67/F Sarcoidosis 1 month before Huang [34] 62//M Erythroderma; plaques; nodules 3 year after Died (3 year) Jones [35] 67/M Erythroderma, toxic epidermal necrolysis Concurrent Died (5 month) Tsochatzis [36] 50/M Polyarthritis, subcutaneous nodules Concurrent Died (2 month) Jayaraman [11] 61/M Macules, papules, plaques, and nodules Concurrent Alive (5 year) Ortonne [37] 63/F Nodules, gingival ulceration Ortonne [37] 54/M Maculopapules, hemorragic/necrotic nodules Nassar [5] M nodules, erythroderma, urticaria, petechiae, purpura and so on. Necrotic purpura, polyarthritis, gingival ulceration, erythematous plaques (sometimes annular), toxic epidermal necrolysis, and hemorragic/necrotic nodules are also reported.…”