Primary systemic amyloidosis may present with features suggesting a vasculitis, including giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). In this report, we describe the clinical characteristics, temporal artery biopsy findings, and the response of vascular and musculoskeletal symptoms to corticosteroid therapy in 4 patients with primary systemic amyloidosis who presented with manifestations of GCA or PMR.
Previous reports of patients with persistent polyclonal B lymphocytosis have found associations with female sex, cigarette smoking, HLA-DR7 phenotype, and moderate elevation of peripheral blood polyclonal B lymphocytes. The presence of binucleated cells and atypical lymphocytes in the peripheral blood of these patients was highly suggestive of a viral infection, such as with the Epstein-Barr virus. We report a 47-year-old asymptomatic woman who was incidentally found to have persistent polyclonal B lymphocytosis and serum IgG against virus capsid antigen (VCA) and Epstein-Barr virus (EBV)-associated nuclear antigen (EBNA) of EBV. The presence of EBV was investigated in the peripheral blood lymphocytes from this patient by in situ hybridization and polymerase chain reaction methods. EBV DNA was demonstrated in the lymphocyte fraction by polymerase chain reaction, and it was further located in lymphoid cells by in situ hybridization. These results indicate that persistent polyclonal B lymphocytosis is strongly associated with EBV.
Diagnosis of central nervous system (CNS) non-Hodgkin's lymphomas may be difficult despite the use of sophisticated scans and routine cytologic methods. The use of an immunoalkaline phosphatase technique to examine cerebrospinal fluid (CSF) containing many mononuclear cells is described. Monoclonal proliferations of B-lymphocytes were demonstrated in six patients with neurologic abnormalities, whose clinical findings and subsequent clinical courses were those of lymphoma. The diagnosis of CNS lymphoma could not be made, despite multiple diagnostic procedures, until the immunocytochemical studies were performed. In three other patients, a lymphoproliferative disorder was suspected; however, examination of CSF showed many T-lymphocytes but no monoclonal B-lymphocytes, consistent with a reactive lymphocytosis. The subsequent clinical courses of these patients have shown no evidence of CNS lymphoma. Immunocytochemical studies of CSF lymphocytes are useful in differentiating benign from malignant proliferations.
Three patients with extranodal peripheral T-cell lymphoma and a distinctive clinical presentation are described. They had acute onset of fever, weight loss, progressive liver failure, bleeding diathesis, pancytopenia, and myelodysplastic changes in the bone marrow. Each patient had one or more paraneoplastic complications: severe rhabdomyolysis with myoglobinuria and secondary renal failure, cutaneous vasculitis, gluten-sensitive enteropathy, polyserositis, and increased macrophages with hemophagocytic activity. They did not have peripheral lymphadenopathy. The complex clinical presentations simulated collagen vascular disorders, systemic infections, or severe liver disease rather than a malignant lymphoma. Routine histologic studies revealed a small population of lymphoma cells in the bone marrow, spleen, and liver. Immunophenotyping studies demonstrated their T-cell phenotype, and cytogenetic analysis showed the clonality in Patients 1 and 2; clonal T-cell receptor gene rearrangement was found in Patients 2 and 3. These studies should be considered in the evaluation of patients with constitutional symptoms, liver failure, coagulopathy, and pancytopenia even in the absence of peripheral lymphadenopathy.
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