Peripheral T-cell lymphoma in a patient with osteopetrosis

Osteopetrosis is a rare genetic disorder in which the function of osteoclas ts is defective, resulting in impaired bone resorption. This disease is usu ally accompanied by myelosuppression due to decreased marrow space and by o steomyelitis, especially in the submandibular bone. We report the case of a 72-year-old woman with an autosomal dominant form of osteopetrosis who suf fered from peripheral T-cell lymphoma. Accurate clinical and pathological d iagnoses and staging were difficult due to nonspecific reactive hyperplasia of the lymph nodes, even though we used several scintigraphic techniques a nd [F-18]fluorodeoxyglucose positron emission tomography. We also paid spec ial attention to myelosuppression and exacerbation of osteomyelitis after c ombination chemotherapy. Severe infectious complications were avoided by ea rly administration of G-CSF for leukocytopenia and by continuous oral admin istration of antibiotics. The patient achieved complete remission after fou r courses of chemotherapy and this status has been maintained for 6 months.