Haematologica
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We report the case of a 23-year-old woman who presented with an adult form of metachromatic leukodystrophy (MLD) evolving over one year with a progressive neurological deterioration. A non-myeloablative matched related haematopoietic stem cell transplantation (HSCT) with concomitant mesenchymal stromal cells (MSCs) infusion was performed. ⋯ At a follow up of 40 months, the patient had a stabilisation of all neurological manifestations of her disease. This case report suggests the feasibility and the potential efficacy of reduced intensity conditioning (RIC) allogeneic HSCT combined with MSC infusion for patients with the adult form of MLD.
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We studied the utility of pancreatic magnetic resonance imaging (MRI) in 72 thalassemia major patients (21 diabetic, 51 normoglycemic). Diabetic patients were significantly older (p<0.0001) and had smaller pancreas volume (p<0.0001). ⋯ In normoglycemic patients, cardiac T2* and log-pancreatic T2* values correlated with homeostatic model assessments HOMA-B (beta cell reserve), HOMA-IR (insulin resistance) and fasting insulin/C-peptide levels. This suggested that improved chelation may improve beta cell reserve and prevent pancreatic atrophy.
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Bleomycin-related pneumonitis (BIP) has recently emerged as one of the main causes of death in Hodgkin's disease treated with standard chemotherapy ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine). We used 18-fluorodeoxyglucose (FDG) positron emission tomography (PET) scanning in a patient with Hodgkin's disease who developed bleomycin lung toxicity following the 4(th) cycle of chemotherapy. The PET scan done two month after the acute presentation with BIP showed uptake of FDG in the lungs. ⋯ Corticosteroids were tapered off subsequently, without recurrence of the respiratory symptoms. Conventional CT scanning was not able to distinguish between residual changes and active inflammation. Thus PET represents a useful diagnostic tool and, independently of CT, indicates the resolution of disease activity, even in the presence of residual pulmonary scarring.
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Our objective was to determine whether the goal of high-dose therapy should be a partial hematologic response or a complete response. We analyzed 282 consecutive stem cell transplant patients. ⋯ The degree of response was affected by the intensity of chemotherapy conditioning, septal thickness, and cardiac biomarkers. Hematologic response translates to longer survival.
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Few data are available on the long-term risk of recurrence of venous thromboembolism (VTE) and on the impact of established thrombosis risk factors in young women. We aimed to study the recurrence rate and the predictive value of laboratory and clinical thrombosis risk factors in young women. ⋯ The risk of recurrent VTE in young women is higher than previously expected and remains constant over at least 20 years. Neither clinical features nor laboratory parameters help predict this risk. Thus, also in young women VTE should be regarded as a chronic disease.