Sakai R, Maruta A, Tomita N, Taguchi J, Fujisawa S, Ogawa K, Motomura S, Kodama F, Mohri H, Ishigatsubo Y
Department of Hematology and Chemotherapy, Kanagawa Cancer Center, Yokahama-shi, Japan.
Leuk Lymphoma. 1999 Nov;35(5-6):607-11. doi: 10.1080/10428199909169626.
A 34-year-old woman of HTLV-I carrier with T-PLL, whose quality of life improved and survival was prolonged after splenectomy, is described. The patient had marked splenomegaly, generalized lymphadenopathy and marked proliferation of abnormal lymphocytes in the peripheral blood with an irregular nucleus, deeply basophilic cytoplasm and a single prominent nucleolus, which were positive for CD2, CD3, CD5, CD7, CD4 and CD8. Although the patient had serum antibody against HTLV-I, HTLV-I proviral DNA integration was not detected. She was diagnosed as an HTLV-I carrier with T-PLL and received combination chemotherapy and 15.1 Gy splenic irradiation. However, the generalized lymphadenopathy and splenomegaly did not improve. The patient underwent splenectomy to palliate abdominal distension and hypersplenism. After the operation, her symptoms improved dramatically and within a week her hemoglobin concentration and platelet count normalized. She was discharged from hospital two weeks after the splenectomy, however 11 months later, she relapsed and despite treatment with chemotherapy and alpha-interferon, she died two months after the second admission. Autopsy findings revealed that PLL cells had invaded the bone marrow, lymph nodes, liver, lungs, kidneys, uterus, ovaries and adrenal glands.
本文描述了一名34岁患有成人T细胞白血病/淋巴瘤（T-PLL）的人类嗜T淋巴细胞病毒I型（HTLV-I）携带者，其在脾切除术后生活质量改善，生存期延长。该患者有明显脾肿大、全身淋巴结肿大，外周血中异常淋巴细胞显著增殖，细胞核不规则，胞质深嗜碱性，有单个明显核仁，这些细胞CD2、CD3、CD5、CD7、CD4和CD8呈阳性。尽管患者血清中有抗HTLV-I抗体，但未检测到HTLV-I前病毒DNA整合。她被诊断为患有T-PLL的HTLV-I携带者，并接受了联合化疗和15.1 Gy的脾脏照射。然而，全身淋巴结肿大和脾肿大并未改善。患者接受脾切除术以缓解腹胀和脾功能亢进。术后，她的症状显著改善，一周内血红蛋白浓度和血小板计数恢复正常。脾切除术后两周她出院，但11个月后复发，尽管接受了化疗和α干扰素治疗，第二次入院两个月后死亡。尸检结果显示，PLL细胞已侵犯骨髓、淋巴结、肝脏、肺、肾脏、子宫、卵巢和肾上腺。
