UNCOMMON CLINICAL PRESENTATION OF A LYMPHOCYTIC LYMPHOMA OF INTERMEDIATE DIFFERENTIATION IN A PATIENT WITH SYSTEMIC-SCLEROSIS

The association of systemic sclerosis (SSc) and non-Hodgkin lymphoma i s a rare event and its pathogenetic mechanism remains to be clarified. We describe a previously unreported association of SSc with a lymphoc ytic lymphoma of intermediate differentiation (IDL), involving the gas trointestinal tract, gallbladder and one salivary gland. Whereas the m orphological, immunological and cytogenetic features were typical of I DL, the extensive extranodal involvement and the association with an a utoimmune disorder were suggestive of two other lymphomas of mantle li neage: mucosa-associated lymphoid tissue (MALT) lymphoma and monocytoi d B-cell lymphoma (MBCL).