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"Hepatic, pulmonary, or hematopoietic.

•"Stage is determined by the addition of points for the three variables. From Levin PT, Osband ME. Evaluating the role of therapy in histio-cytosis-X. Hematol Oncol Clin North Am 1987;1:35, with permission.

"Hepatic, pulmonary, or hematopoietic.

•"Stage is determined by the addition of points for the three variables. From Levin PT, Osband ME. Evaluating the role of therapy in histio-cytosis-X. Hematol Oncol Clin North Am 1987;1:35, with permission.

Long-term complications include:

• Pulmonary: Progressive fibrosis, pulmonary cyst formation, and chronic pneumo-thoraces. There is no effective therapy for these complications and progression to cor pulmonale and respiratory failure occurs. Opportunistic infections are common.

• Hepatic: Cirrhosis and portal hypertension are associated with healing in LCH. Another serious sequela of LCH, sclerosing cholangitis, has been reported and may lead to secondary biliary cirrhosis and liver failure. The etiology is not understood. The only successful treatment for either has been liver transplantation. Sclerosing cholangitis joins severe central nervous dysfunction as one of the most devastating sequelae of LCH.

• Neuropsychiatrie: CNS manifestations can occur without any relationship to radiotherapy or other treatments. This may manifest with learning disability, ataxia, pyramidal signs, and behavioral changes. MRI studies are helpful in localizing structural changes in the brain.

• Endocrinal: Diabetes insipidus and growth retardation are the most frequent complications. They result from histiocytic infiltration of the pituitary and hypothalamus. They are sometimes associated with panhypopituitarism.

• Orthopedic: Deformities of the spine can result in long-term disabilities.

• Malignant: Second primary malignancies associated with radiation therapy include astrocytoma, medulloblastoma, meningioma, hepatoma, osteosarcoma of the skull, and thyroid carcinoma.

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