Erythrocytapheresis is a valid and safe therapeutic option in dysmetabolic iron overload syndrome.

Dysmetabolic iron overload syndrome is a rare event causing hepatic impairment with serious long-term side effects. Here, we describe a case of metabolic syndrome-related hepatic iron overload that showed a rapid, effective, and safe response to erythrocytapheresis. J. Clin. Apheresis 31:443-447, 2016. © 2015 Wiley Periodicals, Inc.

Percutaneous aortic valve replacement in a 65-year old patient with thalassemia intermedia: A case report.

Thalassaemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Organ damage caused by iron overload represents the major cause of illness and the heart complications secondary to extensive myocardial iron overload are the leading cause of mortality. These patients are generally not considered as candidates for heart transplantation. We report a case of a patient with thalassemia intermedia (TI) with severe cardiac dysfunction and severe aortic stenosis who underwent percutaneous aortic valve replacement (PARV). PARV was a safe and a valid therapeutic approach dramatically to improve the clinical evolution of cardiomyopathies in our patient.

Hypochromic microcytic anemia as a clinical presentation of celiac disease.

To determine the prevalence of celiac disease in patients with hypochromic microcytic anemia and to evaluate if and how celiac disease influenced the outcome of anemia, 207 women with hypochromic microcytic anemia were enrolled. Multivariate statistical analysis showed significant differences for the values of hemoglobin comparing the celiac group with the other groups. Comparison of the clinical complications showed an evident statistical significance. Our study showed that among patients with iron deficiency anemia there was a high prevalence of celiac disease. Delayed diagnosis can lead to long-term chronic anemia and various clinical complications. We therefore believe that an early diagnosis prevents and minimizes the complications related to celiac disease, and that serologic screening should become routine in the diagnostic protocol for hypochromic microcytic anemia.

Acute Chest Syndrome: the role of erythro-exchange in patients with sickle cell disease in Sicily.

Acute Chest Syndrome (ACS) describes a syndrome characterized by the presence of a new pulmonary infiltrate on a chest X-ray, fever, and respiratory symptoms and is the leading cause of death and hospitalization in sickle cell disease (SCD). We studied 21 patients affected by SCD (13 HbSbeta+, 4 HbS beta(o), 4 HbSS, mean age 38.2 years). Six out of the 21 patients developed one episode of ACS (two patients had positive blood cultures, for Mycoplasma pneumoniae and Haemophilus influenzae respectively). The aim of our study was to evaluate the therapeutic efficacy of red cell-exchange during ACS. This procedure decreases HbS levels. The patients who underwent erythro-exchange showed a dramatic clinical and radiographic improvement with stabilized HbS levels between 20% and 30%. During follow up (14-32 months), none of the 6 patients developed viral complications related to transfusion therapy, alloimmunization or recurrence of ACS. In conclusion, in regard to the pre- and post-red cell-exchange clinical and laboratory data, we can say that red cell-exchange provides a dramatic resolution of the episode of ACS, minimizes the development of iron overload, and rapidly decreases HbS and hematocrit levels. In light of our results, we hypothesize that ACS episodes are secondary to pulmonary damage and to a gradual worsening related to age, and that there is some evidence that individuals affected by SCD in the third to fourth decade of life are more susceptible to ACS and/or other severe disease-related complications, needing repeated and strict clinical follow up.