Agranular CD4+CD56+ blastic natural killer leukemia/lymphoma

Abstract

Blastic natural killer cell leukemia/lymphoma (blastic NKL/L) is characterized by blastic morphology and a distinctive immunophenotype combining blastic features and cytologically resembling acute myeloid or lymphoid leukemia. The clinical, pathologic, and cytogenetic features of blastic NKL/L have not yet been systematically identified. We report herein a case of blastic NKL/L with skin lesion, adenopathy, and systemic lymphoadenopathy. The identified tumor cells were positive for CD4 and CD56, and negative for T-cell, B-cell, and myeloid markers. T-cell receptor beta, gamma, delta, and immunoglobulin heavy chain genes in the bone marrow cells showed germ-line configurations. Southern blot analysis with a terminal probe did not reveal any Epstein-Barr virus infection. Although patients diagnosed as blastic NKL/L have generally shown chemotherapy resistance and poor prognosis, our patient was treated with a combined chemotherapy, which is also used for acute lymphoblastic leukemia, and has maintained complete remission (CR) for more than 13 months. In addition to clinical investigations, we thoroughly analyzed his karyotype by using a combination of G-banding and a new technique, spectral karyotyping. The karyotype was described as 45, XY, der(1)t(1;20)(p32;q11.2), der(6) (1pter-->1p32:: 6p21.1-->6q13:: 7q11.2-->7qter), der(7) t(7;20)(q11.2;q11.2), t(13;14)(q14;q32), der(13)t(6;13) (p21.1; q14), -20.

Clinical and biologic features of CD4+CD56+ malignancies

“…Cases of CD4 ϩ CD56 ϩ malignancies reported in the literature interestingly were also characterized by cutaneous nodules with lymphadenopathy, spleen enlargement, frequent cytopenia, or bone marrow infiltration. [1][2][3]6,[17][18][19] CD68 expression by CD4 ϩ CD56 ϩ tumor cells has also been previously reported. 2,3,6 The series published by Petrella et al describes 7 cases, identifying del(5q) as a recurrent cytogenetic event.…”

Nephrotic syndrome with crescent formation and massive IgA deposition following allogeneic bone marrow transplantation for natural killer cell leukemia/lymphoma

“…T-cell receptor ␤, ␥, ␦, and immunoglobulin heavy chain genes in the bone marrow cells showed germ-line configurations. A diagnosis of bNKL/L was reached, and he received allo-BMT in the first remission from a healthy 36-year-old HLA-identical sister in June 2000 (details before BMT has been described elesewhere 10 ). The patient received Lphenylalanine mustard (L-PAM) and total body irradiation for conditioning and cyclosporin A (CsA) for GVHD prophylaxis.…”

Long‐term remission of blastic natural killer‐cell lymphoma after autologous peripheral blood stem‐cell transplantation

“…As reported previously [9], regimens for ALL could be effective for this disease. Even if complete remission is achieved by chemotherapy alone, the period of CR is usually short and most patients relapse within several months [3][4][5].…”