Uncommon clinical presentation of a lymphocytic lymphoma of intermediate differentiation in a patient with systemic sclerosis

Abstract

The association of systemic sclerosis (SSc) and non-Hodgkin lymphoma is a rare event and its pathogenetic mechanism remains to be clarified. We describe a previously unreported association of SSc with a lymphocytic lymphoma of intermediate differentiation (IDL), involving the gastrointestinal tract, gallbladder and one salivary gland. Whereas the morphological, immunological and cytogenetic features were typical of IDL, the extensive extranodal involvement and the association with an autoimmune disorder were suggestive of two other lymphomas of mantle lineage: mucosa-associated lymphoid tissue (MALT) lymphoma and monocytoid B-cell lymphoma (MBCL).

Systemic Sclerosis Revealing T-Cell Lymphoma

“…In 1 case, regression of skin sclerosis was observed during the treatment of lymphoma, but it was transient [10]. In spite of successful treatment of lymphoma, skin sclerosis did not improve in 2 cases [12, 13]. In contrast, in the present case, the extension and reduction of skin sclerosis paralleled the development and disappearance of lymphoma.…”

“…At least 16 cases of SSc with malignant lymphoma have been published in the literature [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15](table 1). Concerning the type of lymphoma, all of them were Hodgkin’s disease or B-cell lymphoma.…”

Multilobated B cell lymphoma (Pinkus variant) with isolated muscular localization in a patient with systemic sclerosis: a case report

“…We have recently observed another case of SSc that developed an aggressive NHL [1]. Obviously, we cannot formulate any possible pathogenetic connection between lymphoproliferative disorders and SSc.…”

“…We have recently observed two cases of SSc; they developed NHL of different histologies but had an equally aggressive clinical behavior. The first case has already been reported [1] and consisted of a lymphocytic lymphoma of intermediate differentiation (IDL), curiously involving MALT zones (the gastrointestinal tract, gallbladder, and a salivary gland).…”

Lymphome B cutané au cours d’une sclérodermie systémique

“…Certains cas font discuter des syndromes cutané s sclé rodermiformes parané oplasiques plus que de ré elles associations sclé rodermie systé mique-lymphome [7,27]. Comme dans notre observation, la lymphopathie survient le plus souvent aprè s le diagnostic de sclé rodermie systé mique avec un dé lai moyen de six, huit ans (extrê mes : un à quatorze ans) [1,3,5,13,17,19,21,22,24,25,[28][29].…”