Theįive-year survival rate for children who have undergone a stem cell transplant is 66 percent. Stem cell transplant is the only way to cure familial HLH. Chemotherapy and/or immunotherapy temporarily control the disease, but symptoms inevitably return. Antibiotics or antiviral medications to prevent or treat infectionsįamilial HLH is fatal without treatment, with median survival of about two to six months.Immunoglobulin, a substance made from human blood plasma that contains antibodies.Immunosuppressive medications, such as cyclosporine.Chemotherapy administered directly into the spinal fluid (also called intrathecal therapy).Steroid medication, such as dexamethasone.Rapid diagnosis and early therapy are key. Spinal tap (also called lumbar puncture).Thrombocytopenia: low count of platelets that prevent bleedingĬhildren with HLH may also have central nervous system symptoms, including headache, irritability, sleepiness, or seizures.Anemia: low count of red blood cells that carry oxygen.Neutropenia: low count of white blood cells that fight infection.Cytopenias-low count of one or more of the types of blood cells:.All senior medical staff participate in clinical research activities, so our patients haveĪccess to the very best and up-to-date treatments available. Pulmonologists, endocrinologists, gastroenterologists, neurologists, dentists, orthopedic surgeons, neurosurgeons, radiologists, and radiation oncologists. Because many organ and body systems may be involved with the disease, a patient’s treatment team may also include dermatologists, Hemophagocytic Lymphohistiocytosis Treatment at Dana-Farber/Boston Children'sĬhildren and teens with HLH are treated at the Dana-Farber/Boston Children's Histiocytosis Program, one of the top centers worldwide focused on these rare conditions.Īt the core of the treatment team are hematologists and oncologists who specialize in treating children with histiocytosis. Familial HLH usually affects children under 1 year of age, while the secondary form typically occurs after age 6. HLH may be inherited (familial) or caused by another condition (secondary), such as infection.
Certain aspects of immune function, such as natural killer cell and cytotoxic T-cell activity, are abnormal in HLH.HLH most commonly affects infants and young children.Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition in which certain white blood cells (histiocytes and lymphocytes) build up in and damage organs, including the bone marrow, liver, and spleen, and destroy other blood cells.
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