Obituary of Prof. Michele Baccarani

Obituary of Prof. Baccarany

ASSESSMENT OF CONGENITAL NEUTROPENIA IN CHILDREN: COMMON CLINICAL SCENERIES AND CLUES FOR MANAGEMENT

A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia”, an ultra-rare disorder revealed by sinusoidal variations of the neutrophil count and periodically-recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. Aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of such patients.

TRANSFUSION PRACTICE, POST-TRANSFUSION COMPLICATIONS AND RISK FACTORS IN SICKLE CELL DISEASE IN SENEGAL, WEST AFRICA.

Context and Objectives: Blood transfusions (BT) remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. We aim to assess infectious markers, red cell alloimmunization and iron overload secondary to BT in SCD patients. Materials and Methods: This is a case-control study included 253 SCD (153 SCD-transfused and 100 SCD non-transfused). We evaluated the transfusion practice (modalities, indications), post-transfusion complications (infections, alloimmunization, iron overload) and risk factors of these complications (socio-demographic, clinical, biological). Results: Median age was 28.5 years (5 - 59). Sex ratio was 0.86. Homozygous SCD was more common (95.3%). Simple BT was performed in 92.8% and transfusion exchange in 18.9%. Transfusion indications were dominated by acute anemia (57.06%) and vaso-occlusive crisis (VOCs) (14%). Red blood cell concentrates (RBC) were administered to 93.46%. Median number of RBC received per patient was 10 (2 - 48). The prevalence of VHC in SCD-transfused was 1.33% and 2% for VHB. Anti-HIV antibodies were not found. Red cell alloimmunization frequency was 16%. The most common alloantibodies were anti-rhesus (34.19%) and anti-Kell (23.67%). Iron overload was detected in 7.84%. The number of RBC transfused was the only risk factor for alloimmunization (p = 0.03) and iron overload (p = 0.023). BT frequency was not related to infectious transmission. Conclusion: Despite advances in blood safety, BT therapy is still a risk for SCD polytransfused patients. Although infectious transmission has rare, the risk of alloimmunization and iron overload is high in these patients.

Obituary of Prof. Eligio Pizzigallo

Obituary fo Prof. Eligio Pizzigallo

The impact of the SARS-CoV-2 pandemic on healthcare provision in Italy to non-COVID patients: a systematic review

Background Italy has been one of the countries most affected by the SARS-CoV-2 pandemic and the regional healthcare system has had to quickly adapt its organization to meet the needs of infected patients. This has led to a drastic change in the routine management of non-communicable diseases with a potential long-term impact on patient health care. We investigated the management of non-COVID-19 patients across all medical specialties in Italy. Methods A PRISMA guideline-based systematic review of the literature was performed using PubMed, Embase, and Scopus, restricting the search to the main outbreak period in Italy (from 20 February to 22 June, 2020). We selected articles in English or Italian that detailed changes in the Italian hospital care for non-COVID-19 patients due to the pandemic. Our keywords included all medical specialties in combination with our geographical focus (Italy) and COVID-19. Results Of the 4643 potentially eligible studies identified by the search, 247 studies were included. A decrease in the management of emergencies in non-COVID patients was found together with an increase in mortality. Similarly, non-deferrable conditions met a tendency toward decreased diagnosis. All specialties have been affected by the reorganization of healthcare provision in the hub-and-spoke system and have benefited from telemedicine. Conclusions Our work highlights the changes taking place in the Italian public healthcare system to tackle the developing health crisis due to the COVID-19 pandemic. The findings of our review may be useful to analyze future directions for the healthcare system in the case of new pandemic scenarios.

VEGF and IL-6 correlation in POEMS: a potential upcoming marker of active disease and early autologous BMT response.

Introduction: VEGF function may be responsible for most POEMS manifestations, and it is considered a reliable marker of disease. COVID-19 era arose increasing interest for other inflammatory cytokines, with particular focus on Interleukin-6; VEGF production is stimulated by IL-6 and IL1β, whose concentrations appear to be elevated in clonal plasma cells. Objectives: This study aims to simultaneously evaluate VEGF and IL-6 values in patients (pts) with POEMS at different stages of the disease to find a correlation between them. Methods: We performed a monocentric study, measuring serum levels of VEGF and IL-6 in 8 POEMS pts at different time points of the disease. Results: We observed elevated serum levels of both VEGF and IL-6 in three pts before transplant, while the day after the infusion of autologous stem cells, we observed a steep decrease of both serum markers. Among the four-pts tested only after transplant, two presented with a consensual level of VEGF and IL-6, while the others did not correlate. One patient observed at POEMS diagnosis, during active disease, presented with strikingly high levels of both serum markers. Conclusions: So far, to the best of our knowledge, IL-6 could be considered as a marker of active disease and reliable up to the very first months after BMT, after which its accuracy appears to be lost due to unknown factors, still to be investigated.

THE EFFECT OF ALPHA THALASSEMIA, HBF and HBC ON HAEMATOLOGICAL PARAMETERS OF SICKLE CELL DISEASE PATIENTS IN IBADAN, NIGERIA.

Background: Sickle cell disease is a protean disease with limited data on the phenotypic and genetic variants in Nigeria. This study was conducted to provide baseline data on these variants by characterizing the existing forms of sickle cell disease and correlating these with basic hematological parameters. Methods: Adult and pediatric patients with SCD were recruited from a tertiary health centre in Nigeria. Patients were age and sex matched with healthy controls. Blood samples were obtained for Full Blood Count, phenotyping by High Performance Liquid Chromatography and genotyping for alpha thalassemia by multiplex gap polymerase chain reaction. Data analysis was done using IBM SPSS statistics version 23. Results: A total of 130 patients with sickle cell disease and 117 controls were studied. Alpha thalassemia in the study population was due to a 3.7kb deletion in the alpha globin gene cluster at a prevalence of 45.4% in the patients and 47% in controls. The prevalence of the various existing forms of SCD genotype was: Homozygous S without alpha gene deletion (HbSS)- 39.2%; HbSC - 10.8%; HbSα+1- 35.4%; HbSα+2 - 6.9% and HbSF- 7.7%. HbA2 was significantly elevated in individuals with two alpha gene deletions (HbSα+2). HbF and HbA2 were negatively correlated with each other (r= -0.587, p < 0.001). Individuals with the HbSC genotype followed by HbSα+2 had the best hematological parameters. Conclusions: Hematological parameters varied with hemoglobin genotype. The C hemoglobin and homozygous alpha thalassemia deletion had better ameliorating effect on SCD hematological parameters than the F hemoglobin in this population.

Clinical and Prognostic Features in a Young Adult Patient with de novo Myelodysplastic Syndrome presenting t(11;16) (q23; q24)

hematopoietic clonal neoplasms. MDS occurs mainly in elderly patients. KMT2A rearrangements (KMT2A-r) are rare in MDS, so little is known about their prognostic value. The present study describes the clinical characteristics of a young adult patient diagnosed with MDS-EB-2, presenting the t(11;16)(q23;q24). The Decitabine treatment was initiated since no matching donor was found. The patient showed improved anemia and thrombocytopenia. However, he still had severe neutropenia and clonal chromosomal alteration. Two months after the fifth cycle of Decitabine, the patient presented a worsening of the clinical parameters with increased blast and evolution to AML. He was treated with intensification chemotherapy, but despite all efforts, the patient evolved to death. Treatment refractoriness and leukemia transformation suggest that t(11;16)(q23;q24) with KMT2A-r was associated with poor prognosis. This study reinforces the importance of characterizing new chromosomal alterations and their impact on prognosis in MDS.

TYPICAL AND ATYPICAL PRESENTATION OF COVID-19 INFECTION IN CHILDREN IN THE TOP OF PANDEMIC IN MINIA GOVERNORATE (TWO CENTER EXPERIENCE)

Back ground: A novel coronavirus which is identified as cause of pandemic situation inFebruary2020 and affecting adult and children with variable presentation and outcome. Objective: We studied the typical and atypical clinical and laboratory presentation of COVID-19 during the peak of the first wave in two main referral hospitals, upper Egypt El Minya governorate. Methods: Among 88 children with suspected cases tested for COVID-19, only 22 who proved to be positive. Studied patients were classified into 3 groups based on age. The first group 2–5years,the second for 5–10years and the third one included those aged more than 10 years. All patients met diagnostic guidelines established by Egyptian Ministry of health. Results: out of the positive 22 (25%) patients, 13(59.1%) of them were male, while 9 (40.9%) were females. All enrolled patients have a history of near contact exposure (100%). Thrombocytopenia was the highest presenting symptom in all enrolled patients18(81.8%), while other hematological findings were anemia in 11 (50%), thrombotic symptoms in 2(9.1%), pancytopenia in 2(9.1%) while bleeding was found in 1 patient (4.5%) .Fever 16 (72.7%) the common constitutional symptoms in COVID-19 were not reported in all enrolled patients (0%) while sore throat was reported in only 2 patients (9.1%).Respiratory presentation was only dominant in positive chest CT finding rather than clinical symptoms 17(72.3%) GIT symptom were the dominant presenting feature as vomiting was found in 15 (68.2%), diarrhea in 10 (45.5%), abdominal pain in 11 (50%), jaundice in 9 (40.9%) and dehydration in 6 (27.3%).Neurological symptoms were convulsions in 4(18.2%) while encephalopathy was 2(9.1%).Nephritis was the only renal presentation in the enrolled patients3 (13.6%).Cardiac presentations were only cyanosis 8 (36.4%) and arrhythmias 6 (27.3%) Conclusion: COVID-19 has many clinical classic presentation in children however other non-typical presentation like hematological. CNS and renal presentation has been reported.

NURSES’ PHYSICAL AND PSYCHOLOGICAL SYMPTOMS DURING THE FIRST COVID-19 LOCKDOWN IN ITALY

Background and objective: Northern Italy was one of the first European territories to deal with the Coronavirus Disease 2019 (COVID-19) outbreak. Drastic emergency restrictions were introduced across the country to contain the spread and limit pressure on healthcare facilities. Nurses were at high risk of developing physical, mental and working issues due to professional exposure. The aim of this cross-sectional study was to investigate these issues among nurses working in Italian hematopoietic stem cell transplant (HSCT) programmes during the COVID-19 pandemic. Methods: Data were collected online immediately after the first "lockdown" period in order to investigate the prevalence of physical issues, sleep disorders and burnout symptoms and explore correlations with COVID-19 territorial incidence in Northern Italian regions versus Central and Southern Italian regions. Results: Three hundred and eight nurses working in 61 Italian HSCT Units responded to the survey. Depression, cough and fever were more frequently reported by nurses working in geographical areas less affected by the pandemic (p=0.0013, p<0.0001 and p=0.0005 respectively) as well as worst sleep quality (p=0.008). Moderate levels of emotional exhaustion (mean±SD - 17.4±13.0), depersonalization (5.3±6.1) and personal accomplishment (33.2±10.7) were reported without significant differences between territories. Conclusions: different COVID-19 incidence among territories did not influenced nurses’ burden of symptoms in HSCT setting. However, burnout and insomnia levels should be considered by health care facilities in order to improve preventive strategies.