Nodular lesions of the liver in multiple myeloma

An 83-year-old woman was admitted because of asthenia, weight loss and abdominal pain. She had been diagnosed with MGUS IgGk twenty years earlier and the monoclonal protein levels had remained persistently stable. At admission, physical examination showed pallor, marked hepatomegaly (5 cm below th

transferrin and structural anomalies of chromosome 3 in the blastic phase of chronic myelocytic leukemia

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Myelodysplastic syndrome: classification and prognostic systems

Myelodysplastic syndromes (MDS) are acquired clonal disorders of hematopoiesis, that are characterized most frequently by normocellular or hypercellular bone marrow specimens, and maturation that is morphologically and functionally dysplastic. MDS constitute a complex hematological problem: differences in disease presentation, progression and outcome have made it necessary to use classification systems to improve diagnosis, prognostication and treatment selection. On the basis of new scientific and clinical information, classification and prognostic systems have recently been updated and minimal diagnostic criteria forMDS have been proposed by expert panels. In addition, in the last few years our ability to define the prognosis of the individual patient with MDS has improved. In this paper World Health Organization (WHO) classification refinements and recent prognostic scoring systems for the definition of individual risk are highlighted and current criteria are discussed. The recommendations should facilitate diagnostic and prognostic evaluations in MDS and selection of patients for new effective targeted therapies

Ring sideroblasts and sideroblastic anemias

The sideroblastic anemias include both hereditary and acquired conditions. The two most common forms of congenital sideroblastic anemia, i.e. the X-linked form due to an ALAS2 mutation and the autosomal recessive form due to SLC25A38 mutations, have similar hematologic pictures but completely different clinical courses. Overall, XLSA is a benign disorder that generally responds to pyridoxine with substantial amelioration of anemia; prevention and treatment of iron overload is also important and can be generally achieved through phlebotomy. By contrast, the congenital autosomal recessive congenital sideroblastic anemia due to SLC25A38 mutations is a severe disease, not responsive to pyridoxine and with a clinical course very similar to that of thalassemia major: allogeneic stem cell transplantation should, therefore, be considered in young patients with this disease