ABSTRACT
Pregnancy is a potential trigger of acute thrombotic thrombocytopenic purpura (TTP). The management of pregnancy-associated immune-mediated TTP (iTTP) can be challenging, especially when it is refractory to standard treatment. Caplacizumab, a nanobody to von Willebrand factor (VWF) blocking its A1 domain, is a valuable new therapeutic option. Its use is, however, not approved during pregnancy and breastfeeding. We describe the successful off-label administration of caplacizumab during pregnancy and delivery in a patient with refractory iTTP. The favourable outcome without significant thrombotic or haemorrhagic complications indicates that caplacizumab may be an effective and safe treatment option in refractory iTTP during pregnancy.
Subject(s)
Pregnancy Complications, Hematologic , Purpura, Thrombotic Thrombocytopenic , Single-Domain Antibodies , Humans , Pregnancy , Female , Purpura, Thrombotic Thrombocytopenic/drug therapy , Purpura, Thrombotic Thrombocytopenic/immunology , Single-Domain Antibodies/therapeutic use , Adult , Pregnancy Complications, Hematologic/drug therapy , von Willebrand Factor/antagonists & inhibitorsABSTRACT
Solid papillary carcinoma (SPC) is a rare neoplasm of the breast showing a distinct morphology, neuroendocrine differentiation and should be divided into invasive and in situ subtype according to the current 2012 WHO classification of breast tumors. Here, we describe a case of a pure SPC, invasive-type, in a 31-years old female with an associated mammary Paget's disease (MPD) of the nipple showing a rare, CK7-negative immune phenotype, which has not been reported so far. This unusual differential diagnosis should be added to the rare condition of CK7-negative Paget's disease of the breast and complement a new feature to the characterization of SPC.
