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

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.