Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya3230710.17116/terarkh201789799-104Editorial articleПередовая статьяShort CommunicationHairy cell leukemia and pregnancyВолосатоклеточный лейкоз и беременностьAl-radiL SАль-радиЛ СMoiseevaT NМоисееваТ НSmirnovaS YuСмирноваС ЮShmakovR GШмаковР ГФГБУ «Гематологический научный центр» Минздрава РоссииФГБУ «Научный центр акушерства, гинекологии и перинатологии им. акад. В.И. Кулакова» Минздрава России15072017897VOL 89, NO7 ()ТОМ 89, №7 (2017)9910410042020Copyright ©; 2017, Consilium MedicumCopyright ©; 2017, ООО "Консилиум Медикум"2017Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/32307

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.

Аннотация Представлен опыт наблюдения и лечения волосатоклеточного лейкоза (ВКЛ) при беременности. Сочетание ВКЛ и беременности наблюдали у 5 пациенток. Медиана возраста больных составила 35 лет (28—42 года). Диагноз ВКЛ устанавливался с помощью общепринятого протокола исследований: клинический анализ крови с оценкой морфологии лимфоцитов, миелограмма и трепанобиопсия, иммунофенотипический анализ лимфоцитов крови или костного мозга (у всех больных), цитохимическое определение тартратустойчивой кислой фосфатазы у 3 больных, определение мутации BRAFV600E у 3 больных. У 3 беременных лечение ВКЛ проведено в послеродовом периоде. У 1 пациентки беременность наблюдали в период ремиссии ВКЛ после лечения кладрибином. У 1 пациентки с ВКЛ, выявленным на сроке беременности 11 нед, в связи с нарастанием цитопении проведена терапия интерфероном-α со II триместра беременности, с последующей терапией кладрибином после родов. Течение беременности и родов у всех пациенток проходило без осложнений, у 3 пациенток через естественные родовые пути и у 2 — путем кесарева сечения. Все дети здоровы, без отклонения в развитии за период наблюдения 6—140 мес (медиана 30 мес). Все пациентки в настоящее время в ремиссии ВКЛ: 4 в первой ремиссии со сроком наблюдения от 10 до 48 мес (медиана 15 мес), одна — во второй ремиссии со сроком наблюдения 88 мес. При выявлении ВКЛ на фоне беременности возможна наблюдательная тактика. Лечение ВКЛ при беременности необходимо в случае глубокой или нарастающей цитопении и/или спленомегалии. Предпочтительно применение интерферона-α или выполнение спленэктомии.

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