Lymphomatoid granulomatosis after childhood acute lymphoblastic leukemia: Report of effective therapy

Lymphomatoid granulomatosis, a rare condition in children, affects the lung s primarily but may have significant extrapulmonary manifestations, especia lly in the central nervous system. We report a case of lymphomatoid granulo matosis with onset after the completion of chemotherapy for childhood acute lymphoblastic leukemia. Two months after treatment ended, the 7-year-old g irl developed splenomegaly, cervical adenopathy, and bilateral interstitial pulmonary infiltrates. She improved on cefotaxime but experienced a seizur e 1 month later. A computed tomography scan of the head was normal, but her pulmonary infiltrates had become nodular. A computed tomography-guided bio psy of 1 of the nodules revealed cellular interstitial pneumonitis. One mon th later, she had persistent pulmonary infiltrates, marked splenomegaly, an d new seizures. Magnetic resonance imaging of the head revealed cerebral no dules. Itraconazole was begun, and the pulmonary infiltrates resolved. Five months after her initial symptoms, she developed tonic pupil and a decreas ed level of consciousness. Dexamethasone was initiated. Needle biopsies of the brain were carried out, yielding the diagnosis of severe chronic inflam matory changes focally consistent with granuloma. The child redeveloped spl enomegaly and fever, and then suffered an acute decompensation with hypoxem ia, tachypnea, splenomegaly, and cardiac gallop. Open-lung biopsy revealed lymphomatoid granulomatosis. Lymphoma-directed therapy was initiated, and t he patient had complete resolution of pulmonary and cerebral nodules 5 mont hs later. No intrathecal chemotherapy was administered, and radiation thera py was not necessary. Neuropsychological testing obtained after completion of therapy revealed an improvement in attention, coordination, and fine mot or speed over time. She is now in good health and attending school.