Diego V. Clé

Repeat course of rabbit antithymocyte globulin as salvage following initial therapy with rabbit antithymocyte globulin in acquired aplastic anemia

The treatment for the majority of patients with acquired aplastic anemia (AA) who are not suitable for hematopoietic stem cell transplant (HSCT) is immunosuppression with standard horse antithymocyte globulin (h-ATG) and cyclosporine (CSA) where hematologic responses are observed in 60–70%.[1][1]

Treatment of inherited bone marrow failure syndromes beyond transplantation

Despite significant progress in transplantation by the addition of alternative hematopoietic stem cell sources, many patients with inherited bone marrow failure syndromes are still not eligible for a transplant. In addition, the availability of sequencing panels has significantly improved diagnosis by identifying cryptic inherited cases. Androgens are the main nontransplant therapy for bone marrow failure in dyskeratosis congenita and Fanconi anemia, reaching responses in up to 80% of cases. Danazol and oxymetholone are more commonly used, but virilization and liver toxicity are major adverse events. Diamond-Blackfan anemia is commonly treated with corticosteroids, but most patients eventually become refractory to this treatment and toxicity is limiting. Growth factors still have a role in inherited cases, especially granulocyte colony-stimulating factor in congenital neutropenias. Novel therapies are warranted and thrombopoietin receptor agonists, leucine, quercetin, and novel gene therapy approaches may benefit inherited cases in the future.

Blood film in the era of streaming cells

he complete blood count (CBC) with leukocyte differential ount (LDC) is a powerful tool to diagnose and monitor disase progression and therapy; it is one of the most ordered aboratory tests. Since Dr. Wallace H. Coulter introduced an utomated cell counter in 1953,1 the laborious and timeonsuming eye-count method using a microscope and a emocytometer has been replaced by automated analyzers ith faster turnaround times, reduced burden on technoloists and laboratory costs, and improved count accuracy and eproducibility.2,3 Modern machines incorporating flow cytometry, with or ithout cytochemical staining, maximize cell recognition and rovide reliable LDC results in the majority of cases.4 Although utomated hematology analyzers vary in method, a signifiant overlap exists. A typical instrument discriminates a cell epending on its size, complexity or staining. If a cell does ot fit a predetermined setting expected for neutrophils, lymhocytes, monocytes, eosinophils or basophils (e.g., immature eukocytes, plasma cells, fragmented red cells, platelet aggreates), it is not classified and triggers a flag. A warning flag is resent in 10–30% of samples and should prompt the technolgist to prepare and review a blood smear using a microscope 5–7 r a digital imaging device. Blood smear examinations are routine in clinical laboratoies to review flags but also crucial to analyze red and white