Price S K
Department of Anatomical Pathology, University of Cape Town, South Africa.
Histopathology. 1990 Jul;17(1):7-17. doi: 10.1111/j.1365-2559.1990.tb00658.x.
The pathology of 13 cases of immunoproliferative small intestinal disease (IPSID) associated with alpha-heavy-chain disease--one an apparent non-secretor and another with localized infiltration--is described. Four cases exhibited immunohistological light-chain monotypia. In one of these, evolution of a light-chain negative cell population was observed over a 7-year period. In the intestine, centrocyte-like cells produced lympho-epithelial lesions in 11 cases and enlarged lymphoid follicles in three. In lymph nodes, perifollicular infiltration was observed in 11 cases and abnormal follicles in six. Of three patients with high-grade lymphoma at presentation, one died untreated at 2 months, and two are alive at 34 and 91 months. Of 10 patients with low-grade disease at presentation, two died--one at 76 months, the other after transforming to high-grade lymphoma at 73 months. Eight patients with low-grade disease are alive, an average of 67 months after presentation. Four of five conservatively treated low-grade cases (including three in remission) showed evidence of monoclonality at presentation (light-chain monotypia in two and gene rearrangement in two), while two of the five exhibited DNA aneuploidy. It is concluded that IPSID with alpha-heavy-chain disease is neoplastic in all its stages and is a variant of mucosa-associated lymphoma. The role of centrocyte-like cells and the response to conservative therapy are discussed.
本文描述了13例与α重链病相关的免疫增殖性小肠疾病（IPSID）的病理情况——其中1例为明显的非分泌型，另1例为局限性浸润。4例呈现免疫组化轻链单克隆性。其中1例在7年期间观察到轻链阴性细胞群的演变。在肠道中，11例中心细胞样细胞形成淋巴上皮病变，3例出现淋巴滤泡增大。在淋巴结中，11例观察到滤泡周围浸润，6例出现异常滤泡。3例初诊时为高级别淋巴瘤的患者中，1例未经治疗在2个月时死亡，2例分别在34个月和91个月时仍存活。10例初诊时为低级别疾病的患者中，2例死亡——1例在76个月时死亡，另1例在73个月时转化为高级别淋巴瘤后死亡。8例低级别疾病患者存活，初诊后平均存活67个月。5例接受保守治疗的低级别病例中（包括3例缓解期病例），4例初诊时显示单克隆性证据（2例轻链单克隆性，2例基因重排），而5例中有2例表现为DNA非整倍体。结论是，伴有α重链病的IPSID在所有阶段均为肿瘤性疾病，是黏膜相关淋巴瘤的一种变体。文中讨论了中心细胞样细胞的作用以及对保守治疗的反应。
