A case of recurrent severe pre-eclampsia associated with essential cryofibrinogenaemia

Abstract

Essential cryofibrinogenaemia is a rare disorder characterized by cryofibrinogens without cryoglobulins. Connective tissue disorders and thrombophilia are known to increase risk of pre-eclampsia, but pre-eclampsia has not previously been reported in association with cryofibrinogenaemia. We report the case of a 32-year-old woman with recurrent severe pre-eclampsia diagnosed with essential cryofibrinogenaemia.

Keywords: Severe pre-eclampsia; cryofibrinogenaemia; eosinophilic/T-cell vasculitis.

Figure 1.

Livedo reticularis of the hands and lower limbs. Note: hyperaemia of the digits. The photographs were taken in an air-conditioned clinic room at 22°C.

Figure 2.

Eosinophilic/T-cell vasculitis of the placenta. (A) Low power showing crescentic area of deeper pink staining in the superior aspect of a chorionic vessel, corresponding to a site of vasculitic damage with deposition of fibrinoid material. (B) High power of the area of fibrinoid degeneration, demonstrating a significant inflammatory infiltrate of eosinophils (dark red cytoplasm) and lymphocytes and (at the bottom of the image) loss of integrity of the endothelial layer.