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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphomatoid Granulomatosis | 1A0 Case 1 History
Case 1 History ---- The patient was a 56 year old man with polycythemia vera diagnosed in 1986 and splenectomy in 1993 on hydroxyurea and anagrelide. His last bone marrow biopsy, in January 2007, showed hypercellular bone marrow with multilineage dysplasia, megakaryocytic hyperplasia and mild fibrosis. He was admitted from 2/23 to 2/26 for evaluation of several weeks of right sided headaches, nausea, and vomiting. CT and MRI CNS studies were normal and temporal artery biopsy showed no active arteritis. He was treated empirically with prednisone. After discharge in February, he developed numbness in the right side of the face and his right arm with progression to the left leg, right leg and right body. He was admitted on 03/22 with spine MRI showing ring enhancing lesions at C2 and T11-T12 and treated empirically with broad spectrum antibiotics, antiviral and antifungal medications. CSF showed atypical lymphoid cells, small lymphocytes and monocytes with PCR demonstrable Epstein-Barr virus with no other organisms. Body CT and biopsy showed lung involvement by a “malignant neoplasm”. He received radiation therapy to the spine for presumed malignancy beginning on 4/5. He deteriorated further and on 4/10 an MRI study of the brain and spinal cord showed multiple new, vaguely enhancing, and T2-intense lesions in the brain. Towards the end of his course, the patient and his family expressed their desire for the patient to enter hospice care where he died on 4/12. ---- General autopsy revealed a 2.5 cm ulcer on the dorsum of the right hand, two ill-defined white nodules in the left lower lobe of the lung, and a pulmonary embolus in the right lower lobe. The weight of the unfixed brain was 1440 gm. Lesions were located in the cerebral hemispheres bilaterally including periventricular white matter, brainstem and cerebellum. Lesions consisted of multifocal infarcts with an atypical angiocentric mixed inflammatory infiltrate that involves vessels in the leptomeninges and parenchyma, and causes focal angionecrosis of small vessels sparing the circle of Willis. The infiltrate includes atypical epithelioid histiocytes and atypical lymphocytes with large, irregular nuclei, very prominent nucleoli, immunoreactivity for B-cell markers, and positivity for Epstein Barr virus (EBV). This histological/immunohistochemical/in situ hybridization pattern and anatomic distribution is consistent with lymphomatoid granulomatosis, WHO grade III. This granulomatous inflammation involves the leptomeninges in many places, in company with more widespread lymphocytic inflammation. In areas without direct involvement by this atypical inflammation, the cortex and underlying white matter show abundant scattered activated microglia, and rare microglial nodules.
