La Revue de médecine interne
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Ten years after their licence in France, the use of the two thrombopoietin receptor agonists (TPO-RA), eltrombopag and romiplostim, has deeply modified the landscape of immune thrombocytopenia (ITP) treatment. In this review, we summarise data on efficacy and safety of these treatments during ITP, as well as their use in clinical practice. ⋯ However physician have to keep in mind that thromboembolism rates appear to be higher with TPO-RA treatment in ITP patients at high risk of thrombosis, and that data from "real-life" studies with very long term follow up are not available. Finally, the cost of these treatments should also be evaluated in future therapeutic strategies comparisons.
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The concept of IgG4-related disease (IgG4-RD) has recently been individualized in the early 2000s, but most of the organ involvements are known since more than 100 years. IgG4-RD is a non-malignant fibroinflammatory disorder, characterized by peculiar immunological and pathological abnormalities, which can affect virtually all organs or tissues. ⋯ The objective of this review is to discuss the evolution from organ specific and general diagnostic criteria toward the 2019 ACR/EULAR classification criteria, as well as respective benefits and limits of these criteria. The use of the 2019 ACR/EULAR classification criteria will help to better define homogeneous group of IgG4-RD patients in future clinical, epidemiological and basic science research studies on the disease.
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Review Case Reports
[Pituitary apoplexy following coronary bypass surgery: A case report and literature review].
Pituitary apoplexy is a >rare entity that presents with a sudden onset of headache associated with visual and endocrinological disturbances due to pituitary hemorrhage or infarction. It usually occurs in patients with an unknown pituitary adenoma. Cardiac surgery, and especially coronary artery bypass grafting, can be a precipitating factor in these patients. ⋯ Pituitary apoplexy is a rare and life-threatening complication that may occur after cardiac surgery (coronary artery bypass, cardiac valve surgery), often precipitated by the use of cardiopulmonary bypass. It can occur after other surgical procedures (orthopedic, digestive, thoracic). The diagnosis must be considered during the early postoperative period in the presence of unusual and severe headache associated with visual disturbances.
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Tryptase is the most abundant endopeptidase released by mast cells degranulation, involved in many pro and anti-inflammatory processes. Normal serum tryptase range is 0-11.4 μg/L. Tryptase is a useful diagnostic tool for anaphylaxis, systemic mastocytosis (SM) and mast cell activation syndrome (MCAS), where specific threshold values must be used. ⋯ A clonal mast cell disorder is a central question in anaphylaxis and appropriate explorations should be conducted in these patients. Triggers for anaphylactic reactions vary significantly in the general population and in patients with MS or MCAS. Finally, physicians must be aware of the many pathological and physiological situations that affect tryptase levels.
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Aceruloplasminemia is a rare iron-overload disease that should be better known by physicians. It is an autosomal recessive disorder due to mutations in ceruloplasmin gene causing systemic iron overload, including cerebral and liver parenchyma. The impairment of ferroxidase ceruloplasmin activity leads to intracellular iron retention leading aceruloplasminemia symptoms. ⋯ Iron chelators are widely used. Despite their efficacy on systemic and cerebral iron overload, iron chelators tolerance is poor. Early initiation of iron chelation therapy might prevent or slowdown neurodegeneration, highlighting the need for an early diagnosis but their clinical efficacy remains uncertain.