Progressive multifocal leukoencephalopathy in a patient with B-cell lymphoma during rituximab-containing chemotherapy: case report and review of the literature
Purchase on Springer.com
$39.95 / €34.95 / £29.95*
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC polyomavirus. We describe a rare case of PML in a 48-year-old female patient with diffuse large B-cell lymphoma who received rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) therapy. While she was undergoing five cycles of R-CHOP, she noticed gradually progressive neurological symptoms, such as slurred speech and gait disturbance, and she eventually developed high-grade fever. She also developed Pneumocystis jiroveci pneumonia. The neurological symptoms deteriorated thereafter, and she developed spastic quadriparesis and bulbar palsy. Magnetic resonance imaging showed hyperintensity within the right cerebellar hemisphere on T2-weighted images. Polymerase chain reaction-based tests of the cerebrospinal fluid revealed the presence of the JC virus. Despite intravenous and intrathecal cytarabine treatment, the patient died of PML 5 months after it was diagnosed. Retrospective analysis of her laboratory data showed that her CD4+ T-cell count before R-CHOP therapy had decreased to 68 μL−1. Thus, when administering rituximab-containing chemotherapy, even to patients with no prior history of opportunistic infections, attention should be paid to the potential occurrence of PML, particularly in patients with low CD4+ T-cell counts.
- Koralnik IJ. New insights into progressive multifocal leukoencephalopathy. Curr Opin Neurol. 2004;17:365–70. CrossRef
- Padgett BL, Walker DL, ZuRhein GM, Eckroade RJ, Dessel BH. Cultivation of papova-like virus from human brain with progressive multifocal leucoencephalopathy. Lancet. 1971;1:1257–60. CrossRef
- Berger JR, Major EO. Progressive multifocal leukoencephalopathy. Semin Neurol. 1999;19:193–200. CrossRef
- Vidarsson B, Mosher DF, Salamat MS, Isaksson HJ, Onundarson PT. Progressive multifocal leukoencephalopathy after fludarabine therapy for low-grade lymphoproliferative disease. Am J Hematol. 2002;70:51–4. CrossRef
- García-Suárez J, de Miquel D, Krsnik I, Banas H, Arribas I, Burqaleta C. Changes in the natural history of progressive multifocal leukoencephalopathy in HIV-negative lymphoproliferative disorders: Impact of novel therapies. Am J Hematol. 2005;80:271–81. CrossRef
- Gasnault J, Kahraman M, de Herve MG, Durali D, Delfraissy JF, Taoufik Y. Critical role of JC virus-specific CD4 T-cell responses in preventing progressive multifocal leukoencephalopathy. AIDS. 2003;17:1443–9. CrossRef
- Major EO, Amemiya K, Tormatore CS, Houff SA, Berger JR. Pathogenesis and molecular biology of progressive multifocal leukoencephalopathy, the JC virus-induced demyelinating disease of the human brain. Clin Microbiol Rev. 1992;5:49–73.
- Fong IW, E Toma. The natural history of progressive multifocal leukoencephalopathy in patients with AIDS Canadian PML Study Group. Clin Infect Dis. 1995;2:1305–10.
- Smith DK, Neal JJ, Holmberg SD. Unexplained opportunistic infections and CD4+ T lymphocytopenia without HIV infection: an investigation of cases in the United States. The Centers for Disease Control Idiopathic CD4+ T lymphocytopenia Task Force. N Engl J Med. 1993;328:373–9. CrossRef
- Goldberg SL, Pecora AL, Alter RS, et al. Unusual viral infections (progressive multifocal leukoencephalopathy and cytomegalovirus disease) after high-dose chemotherapy with autologous blood stem cell rescue and peritransplantation rituximab. Blood. 2002;99:1486–8. CrossRef
- Gibson PE, Gardner SD, Field AM. Use of a molecular probe for detecting JCV DNA directly in human brain material. J Med Virol. 1986;18:87–95. CrossRef
- Fong IW, Britton CB, Luinstra KE, Toma E, Mahony JB. Diagnostic value of detecting JC virus DNA in cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy. J Clin Microbiol. 1995;33:484–6.
- Whiteman ML, Post MJ, Berger LG, Tate LG, Bell MD, Limonte LP. Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: Neuroimaging with clinical and pathologic correlation. Radiology. 1993;187:233–40.
- Post MJ, Yiannoutsos C, Simpson D, et al. Progressive multifocal leukoencephalopathy in AIDS: are there any MR findings useful to patient management and predictive of patient survival? AIDS Clinical Trials Group, 243 Team. AJNR Am J Neuroradiol. 1999;20:1896–906.
- Hall CD, Dafni U, Simpson D, et al. Failure of cytarabine in progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. AIDS Clinical Trials Group 243 Team. N Engl J Med. 1998;338:1345–51. CrossRef
- Dworkin MS. A review of progressive multifocal leukoencephalopathy in persons with and without AIDS. Curr Clin Top Infect Dis. 2002;22:181–95.
- Hou J, Major EO. The efficacy of nucleoside analogs against JC virus multiplication in a persistently infected human fetal brain cell line. J Neurovirol. 1998;4:451–6. CrossRef
- Bauer WR, Turel AP Jr, Johnson KP. Progressive multifocal leukoencephalopathy and cytarabine: remission with treatment. JAMA. 1973;226:174–6. CrossRef
- Marriott PJ, O’Brien MD, Mackenzie IC, Janota I. Progressive multifocal leucoencephalopathy: remission with cytarabine. J Neurol Neurosurg Psychiatry. 1975;38:205–9. CrossRef
- O’Riordan T, Daly PA, Hutchinson M, Shattock AG, Gardner SD. Progressive multifocal leukoencephalopathy—remission with cytarabine. J Infect. 1990;20:51–4. CrossRef
- Aksamit AJ. Treatment of non-AIDS progressive multifocal leukoencephalopathy with cytosine arabinoside. J Neurovirol. 2001;7:386–90. CrossRef
- Koralnik IJ, Schellingerhout D, Frosch MP. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 14–2004. A 66-year-old man with progressive neurologic deficits. N Engl J Med. 2004;350:1882–93. CrossRef
- Progressive multifocal leukoencephalopathy in a patient with B-cell lymphoma during rituximab-containing chemotherapy: case report and review of the literature
International Journal of Hematology
Volume 88, Issue 4 , pp 443-447
- Cover Date
- Print ISSN
- Online ISSN
- Springer Japan
- Additional Links
- Progressive multifocal leukoencephalopathy
- CD4+ T-cell counts
- JC virus
- Industry Sectors