Browsing by Author "Duarte, FP"
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- Bone Marrow Langerhans Cell Histiocytosis in Association with Kasabach-Merritt Syndrome: The Difficulty of a Differential DiagnosisPublication . Serra, JT; Silva, R; Aldomiro, F; Duarte, FPLangerhans cell histiocytosis is a rare haematological disorder with variable clinical findings and a high mortality rate. On the other hand, Kasabach-Merritt syndrome is of rare onset at adult age, requiring the simultaneous presentation of vascular lesion, thrombocytopenia, and consumptive coagulopathy. We present the first reported case of both diseases in a single patient and highlight the difficulties of diagnostic. A 69-year-old woman with immune thrombocytopenic purpura underwent surgery for the removal of giant skin haemangiomas. During post-operative care, intravascular disseminated coagulopathy developed. After weeks of corticosteroids and immunosuppressive therapy with no clinical improvement, pulmonary tuberculosis was diagnosed and appropriate treatment initiated. Despite all the efforts, the patient's clinical condition kept worsening and she eventually died. An autopsy revealed bone marrow Langerhans cell histiocytosis. In this case, the patient's autoimmune background together with tuberculosis and intravascular disseminated coagulopathy masked the presentation and made the diagnosis of a rapidly progressive fatal disease very difficult.
- Pregnancy with anti-PP1Pk antibody managed with prednisolone and low-molecular-weight heparin – A case report and literature reviewPublication . Nascimento, S; Rodrigues, M; Henriques, M; Duarte, FP; Barra, A; Matos, TThe anti-PP1Pk is a rare antibody associated with recurrent miscarriages, mainly in the first half of pregnancy. There seems to be a direct correlation between the antibody titer and risk of miscarriage. As this is a rare entity, few case reports have been published. The most frequently proposed therapeutic approaches are doublefiltration plasmapheresis and plasma exchange therapy. The rationale behind them is to remove the cytotoxic antibodies from maternal circulation. Here, we present the case of a 30-year-old woman with a history of two spontaneous miscarriages and a pre-conception anti-PP1Pk antibody titer of 1:4. As soon as she became pregnant, she was placed on prednisolone and low-molecular-weight heparin (LMWH). Biweekly antibody titers were performed throughout the entire gestation and remained below 1:16. As the titers were considered to be low, plasmapheresis was not performed. The pregnancy was uneventful and she delivered a healthy newborn child at 37 weeks of gestation, with no signs of anaemia.