Acute deterioration following an apparently uncomplicated vaso-occlusive episode in sickle cell disease poses significant diagnostic challenges. When respiratory failure, neurological impairment, and progressive cytopenias develop abruptly, distinguishing between infectious, inflammatory, and thrombotic mechanisms becomes critical. We describe a 20-year-old woman with sickle cell/thalassaemia admitted for severe limb pain consistent with vaso-occlusion. Within 24 hours, she developed acute respiratory failure, encephalopathy, and thrombocytopenia requiring intensive care support. Initial management targeted presumed sepsis. However, evolving laboratory abnormalities including disproportionate lactate dehydrogenase elevation, extreme hyperferritinaemia, and marked peripheral erythroblastosis without schistocytosis , prompted reconsideration of the differential diagnosis. Brain magnetic resonance imaging revealed a characteristic pattern supported by futher assessment such as bronchoalveolar lavage and bone marrow biopsy establishing the final diagnosis. Treatment with red cell exchange and therapeutic plasma exchange was followed by progressive haematological and neurological recovery. This case underscores the importance of stepwise diagnostic reasoning in sickle cell disease and highlights how dynamic laboratory interpretation and early referral to specialised centres may be decisive in rare but life-threatening complications.

Background and Aims. Real-world evidence on fostamatinib (FST) in immune thrombocytopenia (ITP) has expanded substantially in recent years, with multicentre national experiences from Spain and Italy confirming its effectiveness and safety in routine clinical practice. Additional cohort analyses remain clinically relevant to better define treatment patterns and outcomes in patients with complex biological and clinical features, including autoimmune diathesis and cardiovascular/thrombotic risk. We therefore aimed to describe the characteristics of a real-world cohort of chronic ITP treated with FST, and to assess response rate, time to response, treatment exposure, and cardiovascular safety, while also exploring whether earlier response was associated with longer treatment exposure.Methods. We retrospectively evaluated 29 patients with chronic ITP treated with FST in a real-world setting, with data cut-off on April 12, 2026. Outcomes of interest included overall response rate, time to response, treatment exposure, and cardiovascular safety. We also explored whether earlier response was associated with longer treatment exposure. Baseline patient and treatment characteristics are reported in Table 1.Results. Baseline characteristics are summarized in Table 1. Briefly, the cohort showed a median TH2 score of 1, with only 4/29 patients (13.8%) displaying a hemorrhagic-risk profile, while the remainder had a prothrombotic profile; moreover, 18/29 patients (62.1%) had at least one cardiovascular risk factor. Overall response rate was 75.9% (22/29), including 15 complete responses and 7 partial responses, while 7 patients were non-responders. Among responders, median time to response was 15.5 days (IQR 10–42.5; range 3–409), with a median platelet count at response of 160×10^3/mmc. In heavily pretreated patients, overall response rate was 62.5% (5/8). At cut-off, 16 patients were still on treatment, with a median platelet count of 170×10^3/mmc (range 65–328); response was maintained in all but 2 patients who shifted from complete to partial response. No significant association was observed between shorter time to response and longer treatment exposure (Spearman rho=0.37, p=0.091). No new cardiovascular events were observed.Conclusions. In this real-world chronic ITP cohort, fostamatinib showed high response rates and rapid activity, including in heavily pretreated patients. Outcomes were consistent with published real-world evidence. While early response did not predict longer treatment exposure, fostamatinib delivered meaningful and sustained benefit across this real-world cohort. Particularly reassuring was the absence of new cardiovascular events in a population enriched for cardiovascular risk factors.

Background: This study presents a young man with hereditary spherocytosis (HS) who underwent intensive chemotherapy for newly diagnosed diffuse large B-cell lymphoma (DLBCL) and achieved complete remission. This case challenges the idea of HS as a barrier to standard DLBCL treatment. Discussion: By meticulously monitoring blood counts and providing timely transfusions, the team successfully mitigated potential complications associated with chemotherapy-induced stress on red blood cells. Conclusions: This experience underscores the importance of a multidisciplinary approach and tailored treatment plans for patients with co-existing conditions, suggesting that HS should not automatically disqualify them from potentially curative therapies for aggressive lymphomas.

Thalassemia is a heterogenous group of hemoglobinopathies; intermediate thalassemia's phenotype can be very variegated due to different genetic matching. Before NGS-era, diagnosis often mismatched with phenotypes, hiding some genetic findings that nowadays could completely explain clinical presentation. In this report, we emphasize the importance of reevaluating genetic testing to achieve a correct diagnosis in case of phenotype mismatch thalassemia. Starting from a suspect of delta/beta thalassemia heterozygosity, reevaluating revealed heterozygosity for alpha-gene triplication combined to delta and beta heterozygosity, a new finding that completely suited patient's clinical manifestation. This case provided the opportunity to underline that an extended study on total globin genes is essential for correct diagnosis of thalassemia, especially when clinical onset phenotypes are more divisive and questionable at a first clinical work-up.