Italian patients with hemoglobinopathies exhibit a 5-fold increase in age-standardized lethality due to SARS-CoV-2 infection.

Since the beginning of the COVID-19 pandemic, concerns have been expressed worldwide for patients with hemoglobinopathies and their vulnerability to SARS-CoV-2 infection. Data from Lebanon confirmed a role of underlying comorbidities on COVID-19 severity, but no deaths among a cohort of thalassemia patients.1 Patients with sickle cell disease (SCD) displayed a broad range of severity after SARS-CoV-2 infection, spanning from a favorable outcome unless pre-existing comorbidities (UK cohort)2 to high case mortality in US.3 History of pain, heart, lung, and renal comorbidities was identified as risk factors of worse COVID-19 outcomes by the US SECURE-SCD Registry.4 While Italy experienced a death rate in the general population among the highest in the world, preliminary data from the first wave of the pandemic showed a lower than expected number of infected thalassemia patients (updated up to April 10, 2020), likely due to earlier and more vigilant self-isolation compared to the general population.

P1580: MANAGEMENT OF PATIENTS WITH MASTOCYTOSIS DURING THE COVID-19 PANDEMIC: A SINGLE CENTER EXPERIENCE

The risk of developing severe symptoms caused by SARS-CoV2 infection in patients (pts) with cutaneous mastocytosis (CM), or indolent systemic mastocytosis (ISM) is like that of healthy people. Unfortunately, the risk may increase in pts with aggressive systemic mastocytosis (ASM), or mastocytosis associated to a hematologic neoplasm (SM-AHN). SARS-CoV2 vaccination reduces the risk of a potentially fatal infection. Considering the increased risk of allergic reactions, it is crucial for these pts to be safely vaccinated. The incidence of vaccine-related anaphylaxis has been reported to be 5 times higher for mRNA vaccines than others. The Italian Ministry of Health (MH) suggests mRNA vaccines for frail individuals. In pts with mastocytosis, it recommended prophylaxis with antihistamines from the day prior to the vaccination to 5 days after, and an observation of 60 min after the vaccination. The administration of the vaccine in a hospital setting was recommended in those with a history of anaphylaxis episodes. In our hospital a vaccination campaign for frail pts in 2 phases was organized

Cell cycle regulation and induction of apoptosis by IL-6 variants on the multiple myeloma cell line XG-1.

The aim of the study was to evaluate the antileukemic effectiveness and toxicity of high-dose hydroxyurea (HHY) and to assess its acute toxicity. Between August 1997 and October 1998, 12 consecutive adult patients (>18 years) with high-risk acute myeloid leukemia (AML) (four patients in first early relapse, seven patients with secondary AML, and one patient with de novo AML concomitant to a lymphoproliferative disorder) were enrolled to receive a single course of HY (100 mg/kg per day) until bone marrow aplasia or for a maximum of 30 days. Of the 12 patients, 5 (41.6%) achieved complete remission (CR), 1 achieved partial remission (PR), 4 were resistant to treatment, and 2 died during induction from infection. No patient with relapsed AML achieved CR, while it was achieved by five of eight patients with secondary AML at diagnosis; five of six MDR1+ patients achieved CR. As concerns follow-up of the CR patients, one did not receive any further treatment and died in CR from pulmonary aspergillosis, and one with a concomitant chronic lymphocytic leukemia (CLL) received two courses of FLAG (fludarabine, cytarabine, granulocyte colony-stimulating factor) regimen with disappearance of the clonal Ig rearrangement, but relapsed after 11 months and died from pneumonia. The remaining three patients were consolidated with two courses of high-dose cytosine arabinoside (AraC), followed by peripheral blood stem cell transplantation (PBSCT) in one patient. One of them relapsed after 3 months, while the other two are still in continuous complete remission (CCR) after 16 and 28 months, respectively. This study has demonstrated the safety and efficacy of HHY in inducing CR in AML patients with unfavorable prognosis. Despite the small number of patients, these encouraging results warrant further studies