title: Dual-Positive (CD4+/CD8+) Acute Adult T-Cell Leukemia/Lymphoma Associated with Complex Karyotype and Refractory Hypercalcemia: Case Report and Literature Review.
abstract: We describe a rare case of adult T-cell leukemia characterized by an expansion of CD4+ CD8+ double-positive lymphocytes associated with human T-lymphotropic virus type 1 (HTLV-1) and a complex karyotype in a 43-year-old Caribbean male who was initially admitted to our hospital with significant lethargy, visual disturbances, dysphagia, right facial palsy and numbness in both feet for 3 days. He was found to have severe hypercalcemia (15.6 mg/dl). Peripheral blood smear showed multilobulated clover-shaped nuclei. Bone marrow and CSF flow cytometries revealed abnormal monoclonal expansion of T cells positive for CD4, CD5, CD8 and CD25 but negative for CD7, CD20, CD56, CD68 and terminal deoxynucleotidyl transferase. The polymerase chain reaction analysis showed a distinct band of the T-cell receptor γ gene, revealing T-cell clonal integration of the proviral DNA of HTLV-1, thus confirming the diagnosis of acute adult T-cell leukemia/lymphoma. Cytogenetic study revealed a male karyotype with monosomy 12, unbalanced translocation 5q and 13q and additional material on 5q, 7q, 14q and 17q. The patient underwent prednisone (EPOCH) chemotherapy followed by autologous transplantation with BEAM regimen. Although patients with a rare mixed CD4+ CD8+ immunophenotype usually present with an aggressive clinical course and have a poor prognosis, our patient was able to survive for 2.5 years.
authors: Division of Hematology and Oncology, Brookdale University Hospital and Medical Center, Brooklyn, N.Y., USA.
authors: Shahzad
authors: S
authors: Raza
authors: Seema
authors: S
authors: Naik
authors: Venkat P
authors: VP
authors: Kancharla
authors: Fekade
authors: F
authors: Tafera
authors: Madhumati R
authors: MR
authors: Kalavar
references: Br J Haematol. 1991 Nov;79(3):428-37
references: 1751370
references: J Exp Med. 1990 Sep 1;172(3):759-65
references: 2388034
references: J Acquir Immune Defic Syndr (1988). 1990;3(11):1096-101
references: 2213510
references: J R Soc Med. 2003 Mar;96(3):126-7
references: 12612113
references: Int J Hematol. 1997 Oct;66(3):257-78
references: 9401272
references: Am J Clin Pathol. 2007 Nov;128(5):875-82
references: 17951212
references: Leuk Res. 1991;15(7):619-25
references: 1861543
references: Leuk Lymphoma. 1994 Aug;14(5-6):395-400
references: 7812198
references: J Natl Cancer Inst. 1985 Feb;74(2):357-69
references: 2983135
references: Br J Cancer. 1991 Oct;64(4):745-8
references: 1911223
references: Proc Natl Acad Sci U S A. 1988 Dec;85(23):9171-5
references: 3194418
references: Leuk Res. 1985;9(11):1353-9
references: 2867255
references: Int J Dermatol. 2006 Oct;45(10):1193-5
references: 17040438
references: Leuk Res. 2000 Apr;24(4):353-8
references: 10713333
references: Nature. 1988 Oct 13;335(6191):642-4
references: 3262830
references: Blood. 2001 Jun 1;97(11):3612-20
references: 11369658
references: Leuk Res. 1992;16(5):435-41
references: 1625468
references: Blood. 2001 Jun 15;97(12):3875-81
references: 11389029
references: J Clin Invest. 2006 Apr;116(4):974-83
references: 16585963
references: Int J Cancer. 1999 Oct 29;83(3):291-8
references: 10495418
references: J Biol Chem. 1993 Jan 15;268(2):1174-9
references: 8380405
references: J Acquir Immune Defic Syndr. 2003 Dec 15;34(5):527-31
references: 14657765
references: Leuk Res. 1987;11(7):665-8
references: 2886703