Lymphomatoid granulomatosis, a rare condition in children, affects the lungs primarily but may have significant extrapulmonary manifestations, especially in the central nervous system. We report a case of
lymphomatoid granulomatosis with onset after the completion of
chemotherapy for childhood
acute lymphoblastic leukemia. Two months
after treatment ended, the 7-year-old girl developed
splenomegaly, cervical
adenopathy, and bilateral interstitial pulmonary infiltrates. She improved on
cefotaxime but experienced a seizure 1 month later. A computed tomography scan of the head was normal, but her pulmonary infiltrates had become nodular. A computed tomography-guided biopsy of 1 of the nodules revealed cellular
interstitial pneumonitis. One month later, she had persistent pulmonary infiltrates, marked
splenomegaly, and new
seizures. Magnetic resonance imaging of the head revealed cerebral nodules.
Itraconazole was begun, and the pulmonary infiltrates resolved. Five months after her initial symptoms, she developed
tonic pupil and a decreased level of consciousness.
Dexamethasone was initiated. Needle biopsies of the brain were carried out, yielding the diagnosis of severe chronic inflammatory changes focally consistent with
granuloma. The child redeveloped
splenomegaly and
fever, and then suffered an acute decompensation with
hypoxemia,
tachypnea,
splenomegaly, and cardiac gallop. Open-lung biopsy revealed
lymphomatoid granulomatosis.
Lymphoma-directed
therapy was initiated, and the patient had complete resolution of pulmonary and cerebral nodules 5 months later. No intrathecal
chemotherapy was administered, and
radiation therapy was not necessary. Neuropsychological testing obtained after completion of
therapy revealed an improvement in attention, coordination, and fine motor speed over time. She is now in good health and attending school.