ABSTRACT
Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). The identification of KIT D816V mutation and the emergence of novel targeted therapies have significantly improved the diagnosis and treatment of systemic mastocytosis. However, certain aspects of clinical care, particularly the diagnosis, assessment, and management of mediator-related symptoms continue to present challenges. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with systemic mastocytosis.
Subject(s)
Anaphylaxis/therapy , Mastocytosis, Systemic/therapy , Medical Oncology/standards , Patient Care Team/standards , Anaphylaxis/diagnosis , Anaphylaxis/immunology , Antineoplastic Agents/pharmacology , Antineoplastic Agents/therapeutic use , Biomarkers, Tumor/blood , Biomarkers, Tumor/genetics , Biopsy , Bone Marrow/drug effects , Bone Marrow/pathology , Hematopoietic Stem Cell Transplantation/methods , Hematopoietic Stem Cell Transplantation/standards , Histamine Antagonists/pharmacology , Histamine Antagonists/therapeutic use , Humans , Immunophenotyping/methods , Immunophenotyping/standards , Mast Cells/drug effects , Mast Cells/immunology , Mast Cells/metabolism , Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/genetics , Mastocytosis, Systemic/immunology , Medical Oncology/methods , Molecular Targeted Therapy/methods , Molecular Targeted Therapy/standards , Mutation , Oncogene Proteins, Fusion/genetics , Proto-Oncogene Proteins c-kit/genetics , Receptor, Platelet-Derived Growth Factor alpha/genetics , Societies, Medical/standards , Transplantation, Homologous/methods , Transplantation, Homologous/standards , Treatment Outcome , mRNA Cleavage and Polyadenylation Factors/geneticsABSTRACT
Chronic myeloid leukemia (CML) is defined by the presence of Philadelphia chromosome (Ph), resulting from a reciprocal translocation between chromosomes 9 and 22 [t(9;22] that gives rise to a BCR-ABL1 fusion gene. CML occurs in 3 different phases (chronic, accelerated, and blast phase) and is usually diagnosed in the chronic phase. Tyrosine kinase inhibitor (TKI) therapy is a highly effective first-line treatment option for all patients with newly diagnosed chronic phase CML (CP-CML). The selection TKI therapy should be based on the risk score, toxicity profile of TKI, patient's age, ability to tolerate therapy, and the presence of comorbid conditions. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with CP-CML.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Medical Oncology/standards , Protein Kinase Inhibitors/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/pharmacology , Antineoplastic Combined Chemotherapy Protocols/standards , Biomarkers, Tumor/antagonists & inhibitors , Biomarkers, Tumor/genetics , Biomarkers, Tumor/isolation & purification , Bone Marrow/pathology , Clinical Trials as Topic , Disease Progression , Dose-Response Relationship, Drug , Drug Resistance, Neoplasm/genetics , Fusion Proteins, bcr-abl/antagonists & inhibitors , Fusion Proteins, bcr-abl/genetics , Fusion Proteins, bcr-abl/isolation & purification , Humans , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/genetics , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/mortality , Medical Oncology/methods , Patient Selection , Philadelphia Chromosome , Prognosis , Progression-Free Survival , Protein Kinase Inhibitors/pharmacology , Protein Kinase Inhibitors/standards , Real-Time Polymerase Chain Reaction/standards , Risk Assessment/methods , Risk Assessment/standards , Societies, Medical/standards , United StatesABSTRACT
Myeloproliferative neoplasms (MPNs) are a group of heterogeneous disorders of the hematopoietic system that include myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET). PV and ET are characterized by significant thrombohemorrhagic complications and a high risk of transformation to MF and acute myeloid leukemia. The diagnosis and management of PV and ET has evolved since the identification of mutations implicated in their pathogenesis. These NCCN Guideline Insights discuss the recommendations outlined in the NCCN Guidelines for the risk stratification, treatment, and special considerations for the management of PV and ET.
Subject(s)
Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/therapy , Combined Modality Therapy/adverse effects , Combined Modality Therapy/methods , Humans , Risk Assessment , Treatment OutcomeABSTRACT
The use of systemic therapy as part of curative treatment and palliation is an evolving paradigm for squamous cell cancer of the head and neck (SCCHN), which historically has been treated with local modalities. At present, the treatment armamentarium includes traditional cytotoxic therapy, targeted biological agents, and emerging immunotherapeutics. This article discusses the use of all of these systemic approaches for the curative and palliative treatment of SCCHN.
