49 research outputs found
Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review.
No full textBACKGROUND
About 1%-5% of acquired haemophilia A cases affect mothers in the postpartum setting.
AIMS
This study delineates the characteristics of this disease, specific to the postpartum setting, notably relapse in subsequent pregnancies.
METHODS
Report of two cases and literature study (1946-2019), yielding 73 articles describing 174 cases (total 176 cases).
RESULTS
Patients were aged 29.9 years (17-41) and 69% primigravidae. Diagnosis was made at a median of 60 days after delivery (range 0-308). Bleeding types were obstetrical (43.4%), cutaneous (41.3%), and muscular (36.7%). In >90% of the cases, FVIII at diagnosis was <1% (range 0%-8%). FVIII inhibitor was documented in 75.4% cases (median titre of 20 BU/ml, range 1-621). Haemostatic treatment was necessary in 57.1% using fresh frozen plasma (16%), factor concentrate (27.6%) and/or bypassing agents (37.4%). Immunosuppressive treatment was administered in 90.8%, mostly steroids (85.3%), alone or combined with immunosuppressants (27%). Rituximab was used mostly as a second line treatment. Only 24 patients (13.6%) had documented subsequent pregnancies and 6 (22.2%) suffered haemophilia recurrence during pregnancy.
CONCLUSION
This study allows better definition of: (1) clinical and laboratory characteristics of postpartum acquired haemophilia, (2) response to therapy, and (3) the risk of relapse for subsequent pregnancies
Alternative 5-Azacitidine 5-Day 100 mg/m2 Dosage Shows Non-Inferiority to Classical Schedule for Myelodysplastic Neoplasm (MDS) and Chronic Myelomonocytic Leukaemia (CMML) Treatment
No full text5-Azacitidine (AZA) is a major treatment option for myelodysplastic neoplasms (MDS) and myelodysplastic/myeloproliferative neoplasms (MDS/MPN). Here we evaluate the efficacy and toxicity of an alternative AZA regimen (100 mg/m2/day for 5 days/28 days) in 68 patients (51 MDS, 17 MDS/MPN) treated between 2008 and 2018.
Median patient age was 66 years, with most patients (98%) having intermediate or high-risk disease. Overall response rate (ORR) was 62% with 22% complete responses (CR). Median OS and median PFS were 22.5 and 18.2 months, respectively. Inferior response rates were calculated in therapy-related MDS (t-MDS) and MDS with excess blast II, with t-MDS having also statistically worse OS and PFS. MDS/MPN patients showed 73.6% ORR with 31.5% CR. Transfusion independence (TI) for red blood cells (RBC) was achieved in 45.9% of transfusion-dependent patients and in 30% for platelets. CR patients showed longer mOS and mPFS (70.6 and 64.7 months, respectively). Longer mOS was also correlated with allogeneic transplantation (48.8 vs. 16.9 months, p = 0.01) and RBC TI (25.4 vs. 13.3 months, p = 0.01). Grade 3/4 cytopenias occurred in 41.1% (neutropenia in 33.8%), and treatment-related mortality was 7.4%.
This study demonstrates that this alternative AZA regimen has comparable efficacy and safety to the standard regimen, compared with historical data.
The authors have confirmed clinical trial registration is not needed for this submission.
© 2025 The Author(s). eJHaem published by British Society for Haematology and John Wiley & Sons Ltd
Shall we rethink the timing of epidural anesthesia in anticoagulated obstetrical patients? [editorial].
No full textModulation of astrocytic metabolic phenotype by proinflammatory cytokines
No full textAstrocytes play an important role in nervous system homeostasis. In particular, they contribute to the regulation of local energy metabolism and to oxidative stress defence. In previous experiments, we showed that long-term treatment with interleukin 1alpha (IL-1alpha) or tumor necrosis factor-alpha (TNFalpha) alone increases glucose utilization in primary culture of mouse astrocytes. In our study, we report that a combination of IL-1beta and TNFalpha exerts a synergistic effect on glucose utilization and markedly modifies the metabolic phenotype of astrocytes. Thus, IL-1beta+TNFalpha treated astrocytes show a marked decrease in glycogen levels, a slight but not significant decrease in lactate release as well as a massive increase in both the pentose phosphate pathway and TCA cycle activities. Glutamate-stimulated glucose utilization and lactate release, a typical feature of astrocyte energy metabolism, are altered after pretreatment with IL-1beta+TNFalpha. As far as mechanisms for oxidative stress defence are concerned, we observed that treatment with IL-1beta+TNFalpha decreases cellular glutathione content and increases glutathione release into the extracellular space while stimulating superoxide anion and nitric oxide production as well as H(2)O(2) release. Interestingly, stimulation of glucose utilization by IL-1beta+TNFalpha is not affected by the antioxidant N-acetyl-L-cysteine, suggesting that cellular stress does not account for this effect. Finally, the effects of cytokines on glucose utilization appear to involve multiple signaling cascades including the phosphoinositide 3-kinase and mitogen-activated protein kinases. Taken together these results establish that a proinflammatory environment such as observed in several neuropathological conditions including Alzheimer's disease, markedly modifies the metabolic phenotype of astrocytes.LND
Immune Tolerance Breakdown in Pregnancy: Transplantation Parallels in Chronic Intervillositis and Villitis of Unknown Etiology (Letter-to-the-Editor)
No full text
Une dyspnée pas comme les autres, ou quand on parle du loup.. [Unusual cause for a dyspnea.].
No full textWe report the case of a patient presenting with late onset systemic lupus erythematosus presenting as a haemolytic anemia and pleuritis. We describe the clinical features, diagnosis, prognosis and treatment of the disease with special focus on haematological and pulmonary involvement
