A 26-year-old woman with fever, migratory polyarthritis, and pericarditis subsequent to a T-cell lymphoma

Abstract

A 26-year-old woman was hospitalized for evaluation of persistent fever, migratory arthritis, and general malaise. Eighteen months before admission, Ki-1-positive T-cell lymphoma with inguinal lymphadenopathy, hepatosplenomegaly, and bone marrow localization (stage IV B) had been diagnosed. She received six cycles of CHVmP/BV consisting of cyclophosphamide, adriamycin, teniposide, prednisone, bleomycin, and vincristine. Six months later she developed skin lesions on her legs and chest, which later spread to her abdomen and extremities. A biopsy of one of the lesions showed perivascular infiltrates of mainly CD30 (Ki-1)-positive cells. A diagnosis of lymphomatoid papulosis (LP) was made. During the following weeks the lesions disappeared spontaneously. Shortly afterwards, she underwent an autologous bone marrow transplantation following BEAC (carmustine, etopiside, cytarabine, and cyclophosphamide) polychemotherapy.One year prior to admission a relapse of the skin lesions had occurred with fever, malaise, and lymphadenopathy. All of these symptoms disappeared without therapy. Follow-up chest X-rays, computed tomographic (CT) scans of the abdomen and thorax, and bone marrow biopsies showed no lymphoma. Five months before admission she had complained of pain and swelling of her left wrist, thumb, and ankle. Tendinitis was diagnosed. Fever then developed with increasing chest pain, dyspnea, and general malaise. A chest X-ray showed cardiomegaly with pleural fluid on the left side. An echocardiogram revealed pericardial effusion. In total, 650 ml of pericardial fluid were aspirated; all cultures including those for tuberculosis and fungi were negative. Cytological examination disclosed no lymphoma cells. Laboratory results revealed weakly positive antinuclear antibodies (ANA), without anti-double-stranded DNA (anti-dsDNA) antibodies. No antibodies against extractable nuclear antigen (ENA) or rheumatoid factor were detected. Serum complement levels were normal.

Cardiac non‐Hodgkin's lymphoma: clinical characteristics and trends in survival

“…About two‐thirds of patients with primary cardiac NHL were male, and DLBCL was the most common histologic subtype in both subgroups. Contrary to B‐cell lymphomas, which were evenly distributed between the primary and secondary subsets, the majority of T‐cell lymphomas were cases with secondary involvement .…”

“…Among the reported cases, 51 patients had primary cardiac lymphoma , which was defined as de novo lymphomatous involvement confined to the myocardium and/or pericardium without the evidence of involvement of the lymphatic organs (lymph nodes, spleen). The remaining 43 patients had secondary cardiac involvement at the initial diagnosis of NHL . Patients with primary cardiac lymphoma were diagnosed at an older age (mean age 59.4 ± 2.1 vs. 50.2 ± 2.7 yrs, P = 0.007).…”

The pale and limping child

“…Apart from this patient there have been only two reported cases of migratory polyarthritis subsequent to T cell lymphoma4 and myelofibrosis 5…”