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VEXAS syndrome is a recently described condition characterized by systemic inflammation, predisposition to hematologic malignancy and a high rate of venous thrombosis. Here we report the case of an elderly male with erythema nodosumlike lesions, ankle arthralgia, and general symptoms. B-mode and Doppler ultrasound of the subcutis diagnosed superficial thrombophlebitis of the lower limbs, which turned out to be the manifestation of a paucisymptomatic VEXAS syndrome. VEXAS should be considered in any patient who presents with unexplained superficial thrombophlebitis, macrocytic anemia and unexplained systemic inflammation.
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INTRODUCTION: Acute myeloid leukaemia (AML) is a haematological disease associated with a dismal prognosis, despite major progress made in recent years in terms of antileukemic agents and supportive care. METHODS: We investigated the results of the intensive treatment of 133 fit AML patients (de novo and secondary) from a referral cancer centre in Romania, treated between January 2015 and December 2021. RESULTS: We included 79 male and 54 female patients with a median age of 53 years (range 18-70). Molecular biology analysis was available for 82.7% of patients, whereas karyotype analysis was only available for 33% of patients. The median overall survival (OS) was 8.7 months, and the disease-free survival rate was 26.3% at a median follow-up of 33.7 months. The complete remission (CR) rate after induction was 48.9% for all patients and 61.9% for patients who were assessable (excluding patients who died before being assessed for response). Twelve patients underwent allogeneic bone marrow transplantation (BMT), with the median OS not reached. Early mortality (EM), defined as death during the first 30 days after admission, was 17.3%, with the main cause of death being septic shock (78.3%). Elderly patients (≥60 years of age) had a lower OS, more primary refractory disease, and higher rates of early mortality. CONCLUSION: Complete remission rates and OS in our cohort were lower than in other reports. Early mortality was unexpectedly high, mainly due to infections, which were the main causes of death in our cohort.
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Macrophage activation syndrome (MAS) represents an acute and severe inflammatory syndrome, idiopathic (primary) or secondary to infections, rheumatic diseases, malignancies, or drugs. MAS is underdiagnosed, being confused with sepsis, adverse effects of anti-arthritic drugs or exacerbated symptoms of evolving rheumatologic or infectious diseases. Because of the late diagnosis, most patients do not benefit from effective therapy, leading to death. Elucidation of valid early diagnostic criteria of MAS would be a particularly important step in reducing the mortality due to this pathology. Thus, the purpose of this review based on 40 studies centered on the diagnostic criteria of MAS. We detailed the main diagnostic criteria and the few diagnostic scores or sets of criteria that have been recently published. The criteria most frequently encountered in the literature include: Fever, hepatosplenomegaly, hyperferritinemia, hepatopathy, coagulopathy, thrombocytopenia, hypertriglyceridemia, decrease in erythrocyte sedimentation rate and bone marrow hemophagocytosis. The most elaborate diagnostic score will result following an ongoing international project and consensus, the Delphi International Survey.
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BACKGROUND AND AIMS: Chelating agents therapy is recommended for polytransfused patients that have evidence of iron overload (an elevated serum ferritin or received over 20 units of red blood cell transfusions). Deferasirox showed efficacy and safety in maintaining or reducing body iron. Iron chelation therapy was associated with hematopoiesis improvement in transfusion-dependent patients.Our objectives were to analyze differences in ferritin level in adult polytransfused patients treated with Deferasirox, to estimate the erythroid improvement and variation of the number of red blood cell transfusion after introducing Deferasirox, to evaluate the side effects of the treatment. METHODS: Retrospective study including all the adult polytransfused patients treated with Deferasirox in Hematology Departments of three county hospitals in the North-West of Romania. RESULTS: We included 40 polytransfused patients treated with Deferasirox in standard doses. There was a significant reduction in serum ferritine from baseline for all the patients (Friedman test, χ2(2)=26.82, p<0.001). Safety profile of Deferasirox was good (three digestive side effects). RBCT were administered before (mean 2.43±1.09 units/month) and after starting Deferasirox (mean 1.40±0.97 units/month), the difference is statistically significant (Student Test, t(39)=6.98, p<0.001). CONCLUSIONS: Deferasirox proves to be an effective iron chelator, the serum level of ferritine decreased for all the patients during the treatment and 22.5 % of the patients developed an erythroid improvement. Safety and compliance were good.