fibrin and Histiocytosis

Maternal floor infarction/massive perivillous fibrin deposition (MFI/MFD), chronic histiocytic intervillositis (CHIV) and villitis of unknown etiology (VUE) are lesions of the placenta which are characterized morphologically. The cause is thought to be pathological immunotolerance/rejection reaction at the fetomaternal interface. The risk of recurrence is elevated and the lesions can lead to severe pediatric diseases.. This article provides an overview of the pathological and anatomical characteristics of each of these lesions, including diagnostic criteria, suspected etiology, clinical relevance and suggested therapy options.. A selective search of the literature was carried out and experiences from own diagnostic clientele are presented.. While MFI/MFD and CHIV occur more rarely, VUE is relatively common occurring in up to 15 % of placentas at term. Both MFI/MFD and CHIV can occur in the first and second trimester, while VUE typically manifests in the third trimester. All lesions can lead to intrauterine growth retardation or abortion and have a tendency towards disease recurrence. Furthermore, VUE and MFI/MFD can be associated with an adverse neurodevelopmental outcome in the children. For all these entities potential therapy strategies have been reported, which are mainly based on anticoagulation and immunosuppression in subsequent pregnancies.

Topics: Abortion, Spontaneous; Chorionic Villi; Chronic Disease; Female; Fetal Growth Retardation; Fibrin; Histiocytosis; Humans; Infant, Newborn; Infarction; Placenta; Placenta Diseases; Placental Circulation; Pregnancy; Pregnancy Trimester, Third; Recurrence; Risk Factors

Chronic histiocytic intervillositis of unknown etiology (CIUE) is a rare placental inflammatory disease, associated with severe obstetric complications. Its pathophysiologic mechanism remains to be elucidated.. To establish anatomical-clinical correlations to improve our understanding of CIUE pathophysiology.. Retrospective study of all cases of CIUE occurring during a 9-year period in a university tertiary hospital center.. CIUE was diagnosed in 69 pregnancies in 50 different women, after early spontaneous abortions (30.4%), late spontaneous abortions (13.0%), in utero deaths (26.1%), and live births (30.4%). Of 39 fetuses surviving to at least 22 weeks, 24 had severe intrauterine growth restriction (61.5%) and 18 died in utero (46.2%). Twelve in utero deaths occurred before 32 weeks of gestation (66.7%). Substantially elevated alkaline phosphatase levels (>600 IU/L) were observed in 55.6% of cases. Microscopic examination of placentas showed that both spontaneous early abortions and intrauterine growth restriction were significantly associated with more intense fibrin deposits.. A diagnosis of CIUE must be considered in cases of severe obstetric complications. We hypothesize that the elevated alkaline phosphatases (ALP) observed during the pregnancy demonstrate the presence of syncytiotrophoblastic lesions due to histiocytosis in the intervillous space, before fibrin deposits cover them.

Topics: Abortion, Spontaneous; Adolescent; Adult; Alkaline Phosphatase; Chorionic Villi; Female; Fetal Death; Fetal Growth Retardation; Fibrin; Histiocytes; Histiocytosis; Humans; Inflammation; Placenta Diseases; Pregnancy; Premature Birth; Retrospective Studies