The Use of Allogeneic Hematopoietic Stem Cell Transplantation in Primary Myelofibrosis.

Primary myelofibrosis (PMF) is a BCR-ABL1 negative myeloproliferative neoplasm characterized by clonal proliferation of myeloid cells. This leads to reactive bone marrow fibrosis, ultimately resulting in progressive marrow failure, hepatosplenomegaly, and extramedullary hematopoiesis. PMF is considered the most aggressive of the BCR-ABL1 negative myeloproliferative neoplasms with the least favorable prognosis. Constitutional symptoms are common, which can impact an individual's quality of life and leukemic transformation remains an important cause of death in PMF patients. The development of the Janus kinase 2 (JAK2) inhibitors have provided a good option for management of PMF-related symptoms. Unfortunately, these agents have not been shown to improve overall survival or significantly alter the course of disease. Allogenic hematopoietic stem cell transplantation (allo-HSCT) remains the only curative treatment option in PMF. However, allo-HSCT is associated with significant treatment-related morbidity and mortality and has historically been reserved for younger, high-risk patients. This review examines patient, disease, and transplant-specific factors which may impact transplant-related outcomes in PMF. Through the vast improvements in donor selection, conditioning regimens, and post-transplant care, allo-HSCT may provide a safe and effective curative option for a broader range of PMF patients in the future.

The Evolving Landscape of Frontline Therapy in Chronic Phase Chronic Myeloid Leukemia (CML).

PURPOSE OF REVIEW: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by uncontrolled proliferation of mature and maturing granulocytes. The disease is characterized by the presence of translocation t(9;22) leading to the abnormal BCR-ABL fusion. Historically, treatment options included hydroxyurea, busulfan, and interferon-α (IFN-α), with allogeneic stem cell transplant being the only potential curative therapy. More recently, the development of tyrosine kinase inhibitors (TKIs) has revolutionized the treatment of CML and turned a once fatal disease into a chronic and manageable disorder. This review aims to discuss the frontline treatment options in chronic-phase CML, provide recommendations for tailoring frontline treatment to the patient, and explore emerging therapies in the field. RECENT FINDINGS: The first-generation TKI, imatinib, was FDA approved in 2001 for use in CML. Following the approval and success of imatinib, second- and third-generation TKIs have been developed providing deeper responses, faster responses, and different toxicity profiles. With numerous options available in the frontline setting, choosing the best initial treatment for each individual patient has become a more complex decision. When choosing a frontline therapy for patients with chronic-phase CML, one should consider disease risk, comorbid conditions, and the goal of therapy.

Things We Do For No Reason: Neutropenic Diet.

For several decades, providers have routinely restricted the diets of neutropenic cancer patients by eliminating foods that might harbor pathogenic microbes to reduce infection rates. These diets, known as neutropenic or low-bacteria diets, are prescribed across the country with little uniformity in the extent or content of prescription. These diets are difficult to follow and force patients to omit fresh fruits and vegetables and limit dairy and meat products from their diet. These dietary omissions compromise nutritional intake in patients who are already at high risk of malnutrition. Randomized trials have shown that these restrictive diets are not superior in preventing infections than more liberalized diets. Evidence shows that adherence to the Safe Food-Handling guidelines issued by the Food and Drug Administration, a mandate for all hospital kitchens, provides adequate protection against food-borne infection, precluding the need for the neutropenic diet. Thus, routine use of the neutropenic diet should be abandoned.