Progressive Multifocal Leukoencephalopathy in a Patient with Hepatosplenic T Cell Lymphoma

( fi g. 1 ). Cerebrospinal fl uid protein, glucose and cell values were all within the normal ranges. PCR analysis of the cerebrospinal fl uid was positive for JC virus DNA. The diagnosis of PML was established. Because of the advanced state of the hematological malignancy, no empirical treatment was applied. The patient died 6 weeks after the onset of his neurological symptoms. The family denied permission for autopsy. PML was described in 1958 as a rare, fatal complication occurring in the setting of chronic lymphocytic leukemia [2] . With the AIDS epidemic, PML has become one of the deadliest opportunistic infections, strongly correlated with depressed CD4+ lymphocyte counts [1] . However, it is also a rare complication in patients with other immunosuppressive disorders, such as chronic lymphocytic leukemia [3], follicular nonHodgkin’s lymphoma, in recipients of renal, heart and lung allografts [4, 5] , and allogenic bone marrow transplants due to the pharmacological immunosuppression state [6] . T cell lymphoma is a lymphoproliferative disorder that causes a profound immunosuppression [7] . Among peripheral T cell lymphomas, hepatosplenic T cell lymphoma is a rare and distinct clinicopathologic entity. It usually manifests by splenomegaly and hepatomegaly without lymphadenopathy [8] . The treatment of lymphoproliferative disorders is being increasingly intensifi ed by the introduction of purine nucleoside analogues (fl udarabine, pentostatine), thus producing an additional immunosuppresDear Sir, Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system caused by the JC polyomavirus. The diagnosis is made on the basis of clinical and neuroradiological criteria with a positive JCV DNA polymerase chain reaction (PCR) in the cerebrospinal fl uid or by means of histopathological characteristics [1] . We report the fi rst case, to our knowledge, of PML associated with hepatosplenic T cell lymphoma. In April 2003, A 56-year-old HIV-negative male was diagnosed with hepatosplenic T cell lymphoma. The patient underwent standard chemotherapy with cyclophosphamide, doxorubicine, vincristine and prednisone (CHOP). He failed multiple conventional therapies aimed at controlling his disease, including pentostatine, etoposide, methylprednisolone, cytarabine and cisplatin (ESHAP) and methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin (MACOP-B). In August 2004, the patient was admitted to the hospital because of a rapidly progressive speech disorder. He was afebrile. Neurological examination showed a normal mental status and occasional word-fi nding diffi culties with semantic and paraphasic errors. Cranial magnetic resonance imaging revealed nonenhancing lesion in the left insula and temporal lobe subcortical white matter, without mass effect on T 1 -weighted images. The lesion was hyperintense on T 2 weighted and on fl uid-attenuated inversion recovery magnetic resonance images Received: September 16, 2005 Accepted: November 22, 2005 Published online: February 9, 2006