The incidence of extranodal non Hodgkin Lymphoma (NHL) is increasing due to the use of new immunohistochemical diagnosis techniques. They account for about 25-30% of the all NHL. Gastrointestinal tract, skin, central nervous system are the main localizations of extranodal lymphoprolipherative disease. Recent reports suggest that HCV, a trigger for clonal B cell proliferation, may also account for extranodal high grade NHL. We describe a 70 year old woman with HCV infection who developed ocular conjunctiva lymphoma MALT type,and subsequently 6 extranodal sites relapses of large Bcells NHL,and policitemia vera as secondary neoplasm. She had a monoclonal gammopaty of uncertain significance since 1997 and concomitant HCV infection (genotype 2a/2c).In December 1999 she developed right eye conjunctival lesion (stage IE). The histological pattern suggested MALT lymphoma. Immunophenotype showed CD19+, CD20+, CD22+ and CD5–,CD10–,CD23–,CD25–. The patient was treated with local radiotherapy and steroids with complete remission. In March 2001 she developed cutaneous-subcutaneous lesion at left lower limb. Histology showed large B cells NHL.The patient was treated with CVP regimen (8 cycles) plus 4 cycles of Rituximab, with complete remission. In September 2002 she developed conjunctival relapse in the same eye (histology confirmed large B-cell NHL). She was treated successfully with CEOP regimen (4 cycles). In August 2003 she developed cutaneous-subcutaneous relapse in left arm, treated with CVP regimen (4 cycles) with complete remission. In May 2005 she developed a rapidly increasing cutaneous-subcutaneous mass in left sub scapular and paravertebral region, involving nervous roots. Histology indicated relapse of large B cell NHL. The patient was treated with RMiniCEOP regimen (4 cycles) with complete clinical and radiological remission. In January 2007 she developed a big mass in left thigh and right arm. Biopsy proved again a large B cell NHL relapse. She started DHAP regimen with a good response after three cycles. In addition in 2006 she developed a JAK2 positive Polycitemia Vera. Our observations confirm that HCV can be a trigger for clonal B cells proliferation also related to MALT NHL, they show that MALT NHL can progress to more aggressive disease such as large B cell subtype, and that polycitemia vera is a rare but possible secondary neoplasm due to expansion and dysregulation of myeloid progenitors as a consequence of drug-induced acquired somatic mutations.
Multiple sites relapses of extranodal Non Hodgkin Malt lymphoma in a patient with HCV Infection : clinical course, hystological transformation and developing of secondary Polycitemia Vera / G. Moreo, R. Castelli, G. Ogliari, A. Maino, G. Bettoni, S. Paina, A. Guariglia, G. Cambiaghi. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 92:s3(2007 Oct), pp. 216-216. ((Intervento presentato al 41. convegno Congress of the Italian Society of Haematology tenutosi a Bologna nel 2007.
|Titolo:||Multiple sites relapses of extranodal Non Hodgkin Malt lymphoma in a patient with HCV Infection : clinical course, hystological transformation and developing of secondary Polycitemia Vera|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||ott-2007|
|Enti collegati al convegno:||Italian Society of Haematology|
|Appare nelle tipologie:||01 - Articolo su periodico|