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Immune thrombocytopenia with antiphospholipid syndrome and monoclonal gammopathy of undetermined significance poses therapeutic dilemmas – whether we should modify the immune thrombocytopenia treatment in antiphospholipid syndrome, what is the influence of monoclonal gammopathy of undetermined significance on the course of immune thrombocytopenia and whether we should and how to prevent the progression of monoclonal gammopathy of undetermined significance to multiple myeloma.
EN
Background: Fibrinogen is a protein playing pleiotropic role in human body. It is engaged in maintaining hemostasis. Congenital fibrinogen disorders comprise quantitative and qualitative fibrinogen anomalies. The symptoms range from bleeding, thrombosis to asymptomatic at all what is the most common case. Hypodysfibrinogenemia with lower level of fibrinogen of reduced activity, is the least common of all congenital fibrinogen disorders. Case report: A 31-year-old woman was reported at the 21 weeks of gestation, suffered from genital tract bleeding and there was a history of stillbirth. Clinical examination with no pathology, however laboratory tests revealed coagulation abnormalities due to prolonged thrombin test, decreased protein S and lower fibrinogen level (70 mg/dl). Autoimmune diseases were excluded and the diagnosis was widened with rotational thromboelastometry and genetic test for hypodysfibrinogenemia. The patient was treated with fibrinogen substitution and prophylactic dose of heparin throughout pregnancy and 2 weeks following labour. At 39 week of gestation Caesarean section was done, with no complications. Results: Genetic test revealed heterozygous mutation in fibrinogen gamma gene confirming hypodysfibrinogenemia. Due to bleeding manifestation in this patient of congenital fibrinogen disorders, fibrinogen substitution was implemented with heparin as a paranticoagulant prophylaxis, what turned out to be successful and enabled the patient to maintain the pregnancy. Conclusions: As hypodysfibrinogenemia symptoms are diverse the management is difficult and each patient’s therapy should be planned separately. Pregnancy may be the first time when congenital fibrinogen disorders reveal and it is especially challenging to prevent from obstetrical complications.
EN
Introduction: Nowadays, rituximab is available both as intravenous (IV) and subcutaneous (SC) formulations. The aim of this study is to compare quality of life (QOL) of patients treated in Hematooncology Clinic in Lodz with B-cell nonHodgkin lymphoma (B-NHL) treated with rituximab IV and SC. Material and Methods: In 50 adult patients with B-NHL diagnosis we assessed QOL by three different questionnaries (EORTC QLQ-C30, FACT/ GOG-NTx and EQ-5D). We compared it between patients treated with rituximab IV and SC. Results: In comparison of general condition in EQ-5D-3L and EORTC QLQ-C30 questionnaire patients treated with rituximab SC felt statistically better than with IV administration (p<0.01). In SC group general health was evaluated for 72 while in IV for only 58,1. According to FACT/GOG-NTx questionnaire SC group had significantly better physical well-being. In SC group we observed statistically more complete responses (CR) 21 (88%) versus 17 (65%) in IV group. Moreover, trend was observed in emotional well-being in favor of SC treatment. Discussion: In our study we observed for the first time in the literature statistical difference in frequency of vomiting, nausea, and lack of appetite mentioned from our patients in questionnarie. It is very important that this findings were not published in any other study comparing SC and IV route od administration. Also looking at economic side of SC administration, this type of dosing. As a result it should be preferred form for B-cell lymphoma patients.
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