系统性硬化症患者中罕见的中度分化淋巴细胞淋巴瘤临床表现

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

作者信息

Baldini L, Guffanti A, Ferrari A, Castagnone D, Mascagni B, Fracchiolla N, Colombi M, Maiolo A T

机构信息

Servizio Autonomo di Ematologia Centro G. Marcora, Ospedale Maggiore IRCCS, Universita' degli Studi, Milano, Italy.

出版信息

Br J Haematol. 1994 Mar;86(3):657-8. doi: 10.1111/j.1365-2141.1994.tb04803.x.

DOI:10.1111/j.1365-2141.1994.tb04803.x

PMID:8043451

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).

摘要

系统性硬化症（SSc）与非霍奇金淋巴瘤的关联是一种罕见事件，其发病机制仍有待阐明。我们描述了一例此前未报道的SSc与中间分化淋巴细胞淋巴瘤（IDL）的关联，该淋巴瘤累及胃肠道、胆囊和一个唾液腺。虽然形态学、免疫学和细胞遗传学特征是IDL的典型特征，但广泛的结外受累以及与自身免疫性疾病的关联提示了另外两种套细胞谱系淋巴瘤：黏膜相关淋巴组织（MALT）淋巴瘤和单核细胞样B细胞淋巴瘤（MBCL）。