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Advances in Hematology (1)
American Journal of Blood Research (1)
Ambler, Kimberley L. S. (1)
Busque, Lambert (1)
Dias Lima, Viviane (1)
Foltz, Lynda (1)
Foltz, Lynda M. (1)
Gupta, Vikas (1)
Harris, Marianne (1)
Leger, Chantal S. (1)
Leitch, Heather A. (1)
Montaner, Julio S. G. (1)
Sirhan, Shireen (1)
Turner, A Robert (1)
Vickars, Linda M. (1)
Year of Publication
Emerging therapeutic options for myelofibrosis: a Canadian perspective
Turner, A Robert
American Journal of Blood Research
Myelofibrosis (MF) is a clonal stem cell disorder characterized by cytopenias, splenomegaly, marrow fibrosis, and systemic symptoms due to elevated inflammatory cytokines. MF is associated with decreased survival. The quality of life of patients with MF is similar to other advanced malignancies. Allogeneic hematopoietic cell transplantation is a curative treatment, but is applicable to a minority of patients with MF. None of the conventional therapies are known to alter the natural history of the disease. Significant progress has been made in the last few years in the understanding of disease biology of MF. Discovery of the JAK2V617F mutation paved the way for drug discovery in MF, and the first JAK1/2 inhibitor, ruxolitinib, has been approved by FDA and Health Canada. Several other JAK1/2 inhibitors are at various stages of clinical development. As a consequence, the therapeutic landscape of MF is changing from a disease where no effective therapies existed to one with several novel treatment options on the horizon. In this report, we assess the changing therapeutic options for MF, and critically analyze the position of novel treatments in the current armamentarium.
Myelofibrosis; JAK1/2; ruxolitinib; splenomegaly; treatment options
Clinical Features, Treatment, and Outcome of HIV-Associated Immune Thrombocytopenia in the HAART Era
Ambler, Kimberley L. S.
Vickars, Linda M.
Leger, Chantal S.
Montaner, Julio S. G.
Dias Lima, Viviane
Leitch, Heather A.
Advances in Hematology
The characteristics of HIV-associated ITP were documented prior to the HAART era, and the optimal treatment beyond HAART is unknown. We performed a review of patients with HIV-associated ITP and at least one platelet count <20 × 109/L since January 1996. Of 5290 patients in the BC Centre for Excellence in HIV/AIDS database, 31 (0.6%) had an ITP diagnosis and platelet count <20 × 109/L. Initial ITP treatment included IVIG, n = 12; steroids, n = 10; anti-RhD, n = 8; HAART, n = 3. Sixteen patients achieved response and nine patients achieved complete response according to the International Working Group criteria. Median time to response was 14 days. Platelet response was not significantly associated with treatment received, but complete response was lower in patients with a history of injection drug use. Complications of ITP treatment occurred in two patients and there were four unrelated deaths. At a median followup of 48 months, 22 patients (71%) required secondary ITP treatment. This is to our knowledge the largest series of severe HIV-associated ITP reported in the HAART era. Although most patients achieved a safe platelet count with primary ITP treatment, nearly all required retreatment for ITP recurrence. New approaches to the treatment of severe ITP in this population are needed.
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