Thalassemia Reports

5 pages, 458 KiB

Open AccessCase Report

The First Case of Haemophagocytic Lymphohistiocytosis Triggered by the Booster Dose of Anti-SARS-CoV-2 Vaccine in a Patient with β-Thalassemia

by Giuseppina Calvaruso, Marta Chiavetta, Disma Renda, Simona Raso, Francesco Dieli, Vincenzo Luca Lentini, Massimo Gentile, Antonio Carroccio and Aurelio Maggio

Thalass. Rep. 2022, 12(2), 46-50; https://doi.org/10.3390/thalassrep12020009 - 16 Jun 2022

Cited by 1 | Viewed by 1476

Abstract

Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare and potentially life-threatening systemic hyperinflammatory disease, which can have several aetiologies. Clinical case: a 48-year-old woman affected by a transfusion-dependent β-thalassemia was hospitalized in our haematology unit presenting with intermittent fever, haepatosplenomegaly and pancytopenia, which developed a few days after the booster dose of anti-SARS-CoV-2 mRNA vaccine. The investigations performed during hospitalization led to a diagnosis of HLH and steroid therapy where IV dexamethasone was initiated and provided benefits. Conclusions: the severity of HLH mandates early treatment, but the management of patients with post-vaccine HLH is still challenging and requires further study. No cases of HLH in patients with thalassemia were previously described. Full article

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7 pages, 511 KiB

Open AccessArticle

The Effect of COVID-19 on Hemoglobinopathy Patients’ Daily Lives While Quarantined: Four Greek Hospitals’ Experiences

by Sophia Delicou, Aikaterini Xydaki, Konstantinos Manganas, Emmanouil Koullias, Loukia Evliati, Chryssoula Kalkana, Michael D. Diamantidis, Achilles Manafas, Marianna Katsatou, Leonidas Roumpatis and Theodoros Aforozis

Thalass. Rep. 2022, 12(2), 39-45; https://doi.org/10.3390/thalassrep12020008 - 6 Jun 2022

Cited by 3 | Viewed by 1923

Abstract

During a pandemic, people are fearful of becoming infected with the virus, which causes anxiety, loss of purpose, and depression. This study aimed to evaluate the social and psychological impact, as well as the impact on homecare, of patients with hemoglobinopathies during the pandemic. Material and Methods: In total, 130 patients from four Thalassemia and Sickle Cell Disease Units of the National Health System of Greece Hospitals were examined via an anonymous questionnaire developed and distributed through stratified sampling. Results: Transfusion-dependent thalassemia, transfused sickle cell disease, and other hemoglobinopathies were represented by 130 patients. During the pandemic, the main concern of patients was the affordability of blood for transfusion. During the lockdown, patients’ moods varied, and their daily lives were disrupted by a lack of access to basic goods and communication with friends and family. Their eating habits, access to exercise, and, to a lesser extent, their financial situation have all been affected in their daily lives. It is crucial to highlight that while access to health services did not suffer in terms of medication and regular visits for their actual disease, it did suffer in terms of the systematic monitoring of complications. Full article

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5 pages, 231 KiB

Open AccessArticle

Peripheral Blood Erythrocyte Parameters in Β-Thalassemia Minor with Coexistent Iron Deficiency: Comparisons between Iron-Deficient and -Sufficient Carriers

by Deniz Aslan and Şeyda Değermenci

Thalass. Rep. 2022, 12(2), 34-38; https://doi.org/10.3390/thalassrep12020007 - 6 Jun 2022

Cited by 1 | Viewed by 1731

Abstract

Changes in erythrocyte parameters are well known in both β-thalassemia minor (BTM) and iron deficiency (ID) when either is present alone; however, to our knowledge, there has been no study showing the changes when the two conditions coexist. We herein assessed erythrocyte parameters in BTM with coexistent ID. The BTM cases were divided into two groups based on ferritin levels as ID+ and ID−; the ID+ group was then further divided based on hemoglobin (Hb) levels as iron-deficient carriers with (IDA+) and without (IDA−) anemia. When compared to the ID− group, all parameters were significantly different in the IDA+ group except mean corpuscular volume (MCV) and red blood cells (RBC). All parameters except RBC were significantly different between the IDA+ and IDA− groups. Hb, hematocrit (Hct), MCV, and mean corpuscular hemoglobin (MCH) levels in the IDA− group were found to be lower than in the ID− group. Changes in erythrocyte parameters in iron-deficient carriers are critical in screening for BT, particularly for correct formulation of mathematical algorithms utilized by artificial intelligence programs. Full article

4 pages, 2007 KiB

Open AccessCase Report

Post-COVID-19 Lymphocytopenia and Opportunistic Pathogens Infection in a Thalassemia Major Patient

by Annamaria Petrungaro, Eugenia Quartarone, Paolo Sciarrone and Luciana Rigoli

Thalass. Rep. 2022, 12(2), 30-33; https://doi.org/10.3390/thalassrep12020006 - 29 Apr 2022

Viewed by 1784

Abstract

Transfusion-dependent thalassemia patients undergo transfusion immunomodulating effects, which result in a general immune response depression and, consequently, an increase in the frequency of infectious episodes and neoplastic events due to a reduction in phagocytic function. Altered natural killer functions and IL-2-mediated lymphocytic response, defects in antigen presentation due to monocyte–macrophage cells, and decreases in bone marrow precursors and HLA II+ cells all play key roles in immunodepression in thalassemia major. SARS-CoV-2 infection presents marked lymphopenia, occurring in 96.1% of severe cases. COVID-19-related lymphopenia is due to various mechanisms, which lead to an increase in lymphocytic apoptosis. Post-COVID-19 lymphocytic quantitative and functional disorders may compromise immune response and promote the onset of infections via opportunistic pathogens. Herein, we report a case of a thalassemia major patient who developed severe post-COVID-19 lymphocytopenia, which may have facilitated the onset of a severe Klebsiella Pneumoniae infection. Full article

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Thalass. Rep., Volume 12, Issue 2 (June 2022) – 4 articles

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