Publications by authors named "Pierre Cougoul"
Introduction: Sickle cell disease (SCD) is one of the most common genetic diseases in the world, annually affecting approximately 310 000 births and causing >100 000 deaths. Vaso-occlusive crisis (VOC) is the most frequent complication of SCD, leading to bone pain, thoracic pain (acute chest syndrome) and/or abdominal spasms. It is the main cause of mortality in patients with SCD, reducing life expectancy.
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- Immunological checkpoint inhibitors, used in treating various cancers, can cause toxic side effects, particularly dermatological disorders.
- Common skin issues like maculopapular erythema and pruritus often occur, but rarer conditions such as fasciitis and scleroderma can also arise.
- Recognizing these unusual manifestations is crucial for internists, as they may mimic other diseases or paraneoplastic syndromes, requiring specific diagnostic and treatment approaches related to ICI toxicity.
Article Synopsis
- - Immune checkpoint inhibitors (ICIs) like nivolumab can lead to rare immune-related adverse events, including acquired amegakaryocytic thrombocytopenia (AAT), which is characterized by low platelet counts and absence of megakaryocytes in the bone marrow.
- - A case study of a patient with metastatic melanoma showed that after 12 cycles of nivolumab, he developed AAT, but responded positively to eltrombopag, an oral drug known to increase platelet production.
- - Despite similar cases reported in literature, there’s uncertainty in treatment guidelines for immune-related AAT; ongoing research is needed to establish safety measures for therapies such as thrombopoietin receptor agonists in cancer patients.
Background And Purpose: White matter lesions (WMLs) are frequent in sickle cell disease (SCD), with a prevalence described to be as high as 53% by age 30. Cerebrovascular regulation and cardiovascular autonomic regulation, more specifically the sympatho-vagal balance, can be altered in SCD. In this study the association between WMLs, cerebrovascular regulation and sympatho-vagal balance was assessed in SCD patients.
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- Inborn errors of immunity (IEI) with dysregulated JAK/STAT signaling can lead to immune dysfunction and infections, and while hematopoietic stem cell transplantation (HSCT) is a potential cure, initial outcomes were not promising.
- This study evaluated the effectiveness of off-label JAK inhibitors (JAKi) as a treatment option for patients with hyperactive JAK/STAT signaling disorders at various European medical centers.
- Results showed that 87% of patients with STAT1 gain of function and 90% with STAT3 gain of function saw symptom improvement, with mild adverse events reported; a significant portion of patients continued JAKi treatment successfully, and some proceeded to HSCT, achieving a 91%
Article Synopsis
- The study investigates the role of autophagy induced by ruxolitinib in JAK2-driven myeloproliferative neoplasms (MPNs), highlighting its association with treatment resistance.
- Ruxolitinib activates protein phosphatase 2A (PP2A), leading to autophagy in JAK2 cells, and inhibiting either autophagy or PP2A enhances the drug's effectiveness by reducing cell proliferation and increasing their death.
- Using a strong autophagy inhibitor, Lys05, alongside ruxolitinib improved treatment outcomes in mice by reducing leukemia burden and extending survival, suggesting that targeting autophagy could make JAK2 MPN therapies more effective.
The association between endometriosis and autoimmune diseases is well known, however no acquired platelet function defect has been described so far. We describe the case of two patients with endometriosis associated with an antiplatelet glycoprotein VI (anti-GPVI) antibody. The two women with deep pelvic endometriosis associated with secondary infertility presented a mild bleeding tendency, a deficient platelet aggregation response to collagen, convulxin or CRP and a severe GPVI deficiency.
View Article and Find Full Text PDFMANAGEMENT OF ACUTE COMPLICATIONS OF SICKLE CELL DISEASE. Acute complications are the most frequent causes of hospitalization and morbidity in patients with sickle cell disease. Vaso-occlusive crisis are responsible of more than 90% of hospitalization, but numerous acute complications can affect multiples organ or function, that may be life-threatening.
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- - Sickle cell disease (SCD) is a common genetic disorder caused by a mutation in the beta-globin chain, leading to distorted red blood cells and various severe health complications, including pain crises and infections.
- - Corticosteroids, although widely used for their anti-inflammatory properties and low cost, have well-documented adverse effects that may be particularly harmful to SCD patients, including inducing pain crises and increased hospitalizations.
- - Recent research suggests that high doses of corticosteroids do not provide benefits for acute SCD events, prompting the need for alternative preventive treatments like hydroxyurea or blood transfusions when corticosteroids are necessary.
Vaso-occlusive episodes (VOEs) are a major concern in patients with sickle cell disease (SCD). Exposure to systemic corticosteroids has been suspected to increase the occurrence of VOEs in case reports or series. No comparative study has been conducted to investigate this risk, which is still debated.
View Article and Find Full Text PDFPurpose: Patients with primary immunodeficiency (PID) are at risk of serious complications. However, data on the incidence and causes of emergency hospital admissions are scarce. The primary objective of the present study was to describe emergency hospital admissions among patients with PID, with a view to identifying "at-risk" patient profiles.
View Article and Find Full Text PDFCommentary on « Ipilimumab induced vasculitis » by Padda A. et al., J Immunother Cancer.
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