Clinical impact of pregnancy in mastocytosis: a study of the Spanish Network on Mastocytosis (REMA) in 45 cases.

摘要

BACKGROUND: The impact of pregnancy on mast cell (MC)-related symptoms and newborn outcome in women with mastocytosis is not well described. We report a series of 30 women who had 45 pregnancies. METHODS: Patients completed a specific questionnaire concerning MC mediator release symptoms graded according to their frequency to detect clinical changes occurring during pregestation and pregnancy as well as postpartum. Information about the medications received during pregnancy and labor and about newborn medical complications was also recorded. RESULTS: Worsening of MC-related symptoms during pregnancy was observed in 10 cases (22%); additionally, 1 woman developed skin lesions as a manifestation of indolent systemic mastocytosis (ISM) within the third trimester of pregnancy. Conversely, 15 cases (33%) experienced clinical improvement during pregnancy, with a complete resolution of pregestational symptoms in 7 cases. MC mediator release symptoms intrapartum were observed in 5 cases (11%) without any fatal outcome. Newborn medical complications (e.g. prematurity, low birth weight, and respiratory distress) were detected in 7 infants (16%) who were all successfully managed with conservative measures. One infant developed cutaneous mastocytosis several years after birth. CONCLUSIONS: Mastocytosis has a heterogeneous clinical behavior during pregnancy: the profile of MC-related symptoms remained unchanged in half of the cases, while in the other half pregnant women experienced either an improvement or an exacerbation of the symptoms, with the manifestation of ISM during pregnancy in 1 case. To prevent potential life-threatening MC-related symptoms, adequate prophylactic antimediator therapy intrapartum should be systematically administered. The absence of both maternal and infant severe complications suggests that patients with nonaggressive categories of mastocytosis should not be advised against pregnancy.