[PDF][PDF] Indolent mantle-cell lymphoma: immunoglobulin variable region heavy chain sequence analysis reveals evidence of disease 10 years prior to symptomatic …

IVRH Chain - Journal of Clinical Oncology, 2011 - researchgate.net
IVRH Chain
Journal of Clinical Oncology, 2011researchgate.net
Discussion MCL is a rare but aggressive form of non-Hodgkin's lymphoma. It represents
approximately 6% to 8% of all non-Hodgkin's lymphoma cases and generally has a worse
prognosis than the other types of disease, with a median survival of 3 to 5 years. 1 The
disease is commonly advanced at presentation, with extranodal and bone marrow
involvement. 2 In addition, bowel involvement at diagnosis is extremely common if actively
looked for, with approximately 80% of patients demonstrating evidence of bowel infiltration …
Discussion
MCL is a rare but aggressive form of non-Hodgkin’s lymphoma. It represents approximately 6% to 8% of all non-Hodgkin’s lymphoma cases and generally has a worse prognosis than the other types of disease, with a median survival of 3 to 5 years. 1 The disease is commonly advanced at presentation, with extranodal and bone marrow involvement. 2 In addition, bowel involvement at diagnosis is extremely common if actively looked for, with approximately 80% of patients demonstrating evidence of bowel infiltration. 3 In retrospect, this patient had MCL evident 10 years before it presented clinically as proven definitively by demonstrating both the t (11; 14) and an identical immunoglobulin sequence in the splenic tissue. Although he did not receive treatment in the form of systemic chemotherapy at that time, he underwent splenectomy, which by itself would have partially treated the disease. The use of splenectomy to treat patients with MCL presenting with cytopenias or splenic symptoms has been described in a series of 26 patients. 4 It is important to note that 15% of these patients did not require chemotherapy afterward, including three patients who were previously untreated and had stable disease for 8 years after splenectomy. In a smaller series of seven patients with MCL presenting with a leukemic picture and splenomegaly, 5 all were treated by splenectomy alone. With a median follow-up of 20 months, only two patients experienced disease progression. In addition, Ruchlemer et al6 describe a retrospective
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