Angelopoulou Maria K, Siakantariz Marina P, Vassilakopoulos Theodoros P, Kontopidou Flora N, Rassidakis George Z, Dimopoulou Maria N, Kittas Christos, Pangalis Gerassimos A
Hematology Section, First Department of Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Laikon General Hospital, 16 Sevastoupoleos Street, 11510 Athens, Greece.
Eur J Haematol. 2002 Jan;68(1):12-21. doi: 10.1034/j.1600-0609.2002.00551.x.
To describe the clinical, immunophenotypic and molecular features, as well as the clinical course of patients with unusual presentation of mantle cell lymphoma (MCL) purely located to the spleen.
We describe seven patients presented with splenomegaly and a leukemic picture without lymphadenopathy, fulfilling the diagnostic criteria of MCL. In addition to clinical and pathologic features, patients were studied with respect to surface immunophenotype, including adhesion molecule profile, immunohistochemical expression of cyclin-D1 and bcl-1 rearrangement by polymerase chain reaction.
Four patients were male and three female. The median palpable spleen size was 15 cm. A preliminary diagnosis of MCL was made, based on blood cell morphology and immunophenotype. All patients underwent splenectomy for therapeutic purposes. Studies done in blood and splenic lymphocytes revealed the following: 7/7 patients were CD19/CD5, CD20 and CD38 positive; CD10 negative and 6/7 CD23 negative. The adhesion molecule expression pattern was consistent in all patients: L-Selectin and CD11c were negative, CD11alpha and CD18 weakly positive and CD54 strongly positive. The median spleen weight was 1775 g. Histology disclosed a cytologic and architectural pattern consistent with MCL. Cyclin-D1 was positive in 6/6 studied patients. Bcl-1 rearrangement was found in 5/7 patients. Splenectomy was applied as the sole treatment and was beneficial in all patients, with median blood values as following: prior to splenectomy, Ht 29.5%, platelets 110 x 10(9)/l, lymphoma cells 5.0 x 10(9)/L, and at 6 months post-splenectomy, Ht 43%, platelets 311 x 10(9)/l and lymphoma cells 3.0 x 10(9)/L. Of the seven patients, two developed progressive disease 11 and 26 months post-splenectomy. The remaining five are in improving clinical and hematological condition without chemotherapy at a median follow up of 20 months.
We conclude that this presentation represents a separate form of MCL which requires splenectomy. It remains to be seen whether it carries a better prognosis than classical MCL.
描述纯位于脾脏的套细胞淋巴瘤（MCL）不寻常表现患者的临床、免疫表型和分子特征以及临床病程。
我们描述了7例出现脾肿大且有白血病样表现但无淋巴结病的患者，他们符合MCL的诊断标准。除临床和病理特征外，还对患者进行了表面免疫表型研究，包括黏附分子谱、细胞周期蛋白D1的免疫组化表达以及通过聚合酶链反应检测bcl-1重排。
4例男性，3例女性。可触及脾脏的中位大小为15厘米。根据血细胞形态和免疫表型初步诊断为MCL。所有患者均因治疗目的接受了脾切除术。对血液和脾脏淋巴细胞进行的研究显示如下：7/7例患者CD19/CD5、CD20和CD38呈阳性；CD10阴性，6/7例CD23阴性。所有患者的黏附分子表达模式一致：L-选择素和CD11c阴性，CD11α和CD18弱阳性，CD54强阳性。脾脏中位重量为1775克。组织学显示细胞学和结构模式与MCL一致。6/6例研究患者的细胞周期蛋白D1呈阳性。5/7例患者发现bcl-1重排。脾切除术作为唯一治疗方法应用于所有患者且均有益，中位血液值如下：脾切除术前，血细胞比容29.5%，血小板110×10⁹/L，淋巴瘤细胞5.0×10⁹/L，脾切除术后6个月，血细胞比容43%，血小板311×10⁹/L，淋巴瘤细胞3.0×10⁹/L。7例患者中，2例在脾切除术后11个月和26个月出现疾病进展。其余5例在中位随访20个月时临床和血液学状况改善且未接受化疗。
我们得出结论，这种表现代表MCL的一种单独形式，需要进行脾切除术。它是否比经典MCL预后更好还有待观察。
