Angiotrophic large cell lymphoma mimicking multiple sclerosis associated transverse myelitis

Angiotrophic large cell lymphoma (ALCL) is a rare disease characterized by a proliferation of malignant lymphoid cells, usually of B-cell origin, with in the lumina of small vessels and frequently involving the central nervous system (CNS). ALCL generally follows a rapidly fatal course and is rarely diagnosed prior to autopsy. Tumour cells are rarely present on examination of peripheral blood, cerebrospinal fluid (CSF) or bone marrow, therefore, a n early diagnosis is heavily dependent on recognition of neurological manif estations which are present in over 80% of cases. A pre-mortem diagnosis of ALCL can be made in cases where brain biopsy has been performed. We report a case of a 45-year-old woman demonstrating clinical and laboratory featur es suggestive of multiple sclerosis associated transverse myelitis (MSA-TM) in whom ALCL was unexpectedly discovered at autopsy. Microscopic examinati on revealed diffuse CNS vascular involvement by neoplastic cells and promin ent myelopathy of the upper thoracic spinal cord associated with ascending and descending tract degeneration of the spinal motor and sensory tracts. N eoplastic cells were immunoreactive for B-cell lymphoid markers. The presen t case is the first to demonstrate neoplastic B-cell clonality in ALCL usin g polymerase chain reaction (PCR) to detect abnormal V-D-J immunoglobulin g ene rearrangements. ALCL should be considered in the differential diagnosis whenever a transverse myelitis is present in association with global encep halopathy and elevated serum lactate dehydrogenase. The neurological manife stations of ALCL with particular reference to its presentation as MSA-TM is discussed. (C) 1999 Harcourt Publishers Ltd.