Corticosteroids interfere with the cell cycle of inflammatory cells and modify the activity of other immune components.
Calcineurin inhibitors halt the activity of calcineurin, a phosphatase involved in T-cell activation. The targets can be tumor necrosis factor (TNF), interleukins (ILs), or B- or T-cell activity. Biologic agents are medications derived from living organisms that target particular components of the immune system. There are multiple targets in the immune system, as well as varied mechanisms in inhibiting inappropriate immune activity. The general effect is dampening of the immune response. Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. Survival is improved by vaccination against bacterial infections, prophylactic antibiotics, and aggressive treatment of infections. The management of painful episodes consists of IV fluids and analgesics, and in severe episodes, exchange transfusions may be required. Sickle cells can usually be seen on the peripheral blood smear, but Hb electrophoresis is needed for diagnosis. Individuals with SCD are susceptible to infection, infarction of various organs, and bone marrow aplasia lung involvement in acute chest syndrome can be rapidly fatal. Triggers such as stress and hypoxia can induce or worsen the sickling of RBCs. Sickle cell trait, which is the heterozygous condition, is the only 1 of the group that is generally benign and rarely associated with serious SCD-like complications.
Sickle cell disease (SCD) is a group of genetic disorders in which an abnormal Hb molecule (HbS) transforms RBCs into sickle-shaped cells, resulting in chronic anemia, vasoocclusive episodes, pain, and organ damage. Radiation therapy is used in adults but not in children, and stem cell transplantation is used for patients with aggressive disease. Management is with chemotherapy or targeted drugs. Diagnosis is made by lymph node biopsy, bone marrow biopsy, or both. T-cell NHLs include adult T-cell lymphoma and mycosis fungoides. B-cell NHLs include diffuse large B-cell lymphoma, follicular lymphoma, Burkitt lymphoma, mantle cell lymphoma, and marginal zone lymphoma. Clinical features include lymphadenopathy and hepatosplenomegaly, but some individuals present with extranodal involvement and abnormal lab findings. Like Hodgkin lymphoma, which has distinct pathologic features and treatments, NHL often presents with constitutional signs of fever, night sweats, and weight loss. Most pediatric cases are aggressive and high-grade (but curable) in adults, low-grade subtypes are more common. Non-Hodgkin lymphomas (NHLs) are a diverse group of hematologic malignancies that are clonal proliferative disorders of mature or progenitor B cells, T cells, or natural killer (NK) cells.
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