Unusual presentation of extranodal peripheral T-cell lymphomas with multiple paraneoplastic features

Abstract

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.

Rhabdomyolysis caused by peripheral T-cell lymphoma in skeletal muscle

“…First, given that biopsy results will typically be uninformative at an early stage, it is important to wait several weeks or months after the clinical event of rhabdomyolysis to perform a biopsy [8], because the results of a biopsy will typically be uninformative at an early stage. Second, Diez et al reported that muscle biopsy was nondiagnostic [4]. Given that lymphoma is a systemic illness, there was no need to search for a primary focus when the lymphoma spread throughout the body.…”

“…Only three previous reports of malignant lymphoma in skeletal muscle with rhabdomyolysis have been documented [4][5][6] (Table). In concordance with the previous reports, T-cell lymphoma occurred in the lower limbs in the present case and involved the gastrocnemius, psoas, gluteal, and soleus muscles.…”

"Rheumatoid nodules" and lymphoma!

“…Previous reports, however, were of T cell4 rather than B cell lymphoma occurring with panniculitis and the good response to corticosteroids is unusual for a paraneoplastic syndrome. Seronegative polyarthritis has been reported as a rheumatological manifestation of B and T cell lymphomas2 5 6 and arthritis indistinguishable from seronegative rheumatoid arthritis has been described in association with cutaneous T cell lymphoma 2.…”

Lymphome non Hodgkinien intramusculaire primitif chez le sujet jeune: à propos d’un cas et revue de la littérature

“…Le diagnostic de certitude repose sur l'examen histologique d'une biopsie chirurgicale de la tumeur. Dans la grande majorité des cas, la masse est un lymphome de type B [11]. (Plus de 95%des cas), dont le pronostic semble-t-il meilleur que celui des lymphomes de type T [5].…”