Al-Radi L S, Moiseeva T N, Smirnova S Yu, Shmakov R G
National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia.
Acad. V.I. Kulakov Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia, Moscow, Russia.
Ter Arkh. 2017;89(7):99-104. doi: 10.17116/terarkh201789799-104.
The paper presents experience in following up and treating hairy cell leukemia (HCL) during pregnancy. The combination of HCL and pregnancy was observed in 5 patients. The patients' median age was 35 years (range, 28-42 years). The diagnosis of HCL was based on a conventional examination protocol: clinical blood analysis with the morphological assessment of lymphocytes, a myelogram and trepanobiopsy, immunophenotypic analysis of lymphocytes or bone marrow (in all the patients), cytochemical determination of tartrate-resistant acid phosphatase in 3 patients, and identification of BRAFV600E mutation in 3 patients. Three pregnant women were treated for HCL in the postpartum period. In one patient with HCL, pregnancy was seen in remission after treatment with cladribine. In one patient with HCL detected at 11 weeks' gestation, interferon-α therapy during the second trimester of pregnancy was performed for increased cytopenia, which was followed by cladribine therapy after delivery. Pregnancy and delivery were uncomplicated in all the patients; 3 patients had vaginal delivery and 2 patients underwent cesarean section. All infants were healthy, with no developmental abnormalities during a follow-up period of 6-140 months (median 30 months). All the patients with HCL are currently in remission: 4 patients in first remission at a follow-up of 10 to 48 months (median 15 months) and one patient in second remission at a follow-up of 88 months. Possible observational tactics is possible when HCL is detected during pregnancy. Treatment of HCL during pregnancy is necessary in cases of deep or progressive cytopenia and/or splenomegaly. The use of interferon-α or splenectomy is preferable.
本文介绍了妊娠期间毛细胞白血病（HCL）的随访和治疗经验。观察到5例HCL合并妊娠的患者。患者的中位年龄为35岁（范围28 - 42岁）。HCL的诊断基于传统检查方案：临床血液分析及淋巴细胞形态学评估、骨髓造影和骨髓活检、淋巴细胞或骨髓的免疫表型分析（所有患者均进行）、3例患者进行抗酒石酸酸性磷酸酶的细胞化学测定以及3例患者检测BRAFV600E突变。3名孕妇在产后接受了HCL治疗。1例HCL患者在接受克拉屈滨治疗后妊娠处于缓解期。1例在妊娠11周时检测出HCL的患者，因血细胞减少加重在妊娠中期进行了干扰素-α治疗，产后接着进行了克拉屈滨治疗。所有患者的妊娠和分娩均无并发症；3例患者经阴道分娩，2例患者接受剖宫产。所有婴儿均健康，在6 - 140个月（中位30个月）的随访期内无发育异常。所有HCL患者目前均处于缓解期：4例患者在随访10至48个月（中位15个月）时处于首次缓解期，1例患者在随访88个月时处于第二次缓解期。妊娠期间检测到HCL时可能有可行的观察策略。妊娠期间HCL出现严重或进行性血细胞减少和/或脾肿大时需要进行治疗。使用干扰素-α或脾切除术较为可取。
