Successful treatment of natural killer (NK) cell leukemia following a long-standing chronic active Epstein–Barr virus (CAEBV) infection with allogeneic bone marrow transplantation

Abstract

Summary:The optimal treatment for natural killer (NK) cell leukemia after chronic active Epstein-Barr virus (CAEBV) infection has not been determined. A 15-yearold boy presented with NK cell leukemia following CAEBV infection for 5 years. The peripheral blood and BM had an increased number of CD3 À CD56 + large granular lymphocytes and a monoclonal integration of the EBV genome was detected. Chemotherapy was not sufficiently effective to control the disease. Allogeneic BMT from an HLA-identical sister was performed using a conditioning regimen consisting of total body irradiation, cyclophosphamide and thiotepa. The patient is diseasefree with a perfect performance status 24 months after BMT. This is the first report to show that allogeneic BMT is potentially able to cure NK cell leukemia after CAEBV infection.

Natural killer-cell malignancies: diagnosis and treatment

“…19,21 The use of allogeneic hematopoietic stem cell transplantation (HSCT) had been described to result in remission in a few patients, although whether long-term survival could be achieved was unknown. 51,52 Non-nasal NK-cell lymphomas fare marginally better, with long-term remission reported in less than 10% of cases.…”

“…79 However, overall treatment results are poor, although occasional success has been reported after allogeneic HSCT. 80 Blastic NK-cell lymphoma/CD4 þ CD56 þ hematodermic neoplasm Blastic NK-cell lymphoma was previously regarded by the WHO classification as a distinct pathologic entity. 81 In the recent WHO-EORTC classification, 82 however, they are referred to CD4 þ /CD56 þ hematodermic neoplasm.…”

Large Granular Lymphocyte Leukemia

“…Induction chemotherapy with an intensive acute lymphoblastic leukemia (ALL)-like regimen followed by allogeneic stem cell transplantation has been used with curative intent in a limited number of cases (Fig. 5) [75,76]. Chronic NK-cell leukemia/lymphocytosis usually has an indolent course.…”

Leg ulcer and thigh telangiectasia associated with natural killer cell CD56– large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome

“…2,16 Cyclophosphamide, doxorubicin, vincristine, and prednisoneelike chemotherapy has been ineffective, and induction chemotherapy with an intensive lymphoblastic leukemia-like regimen, followed by allogeneic stem cell transplantation, has been used with a curative intent. 1,40 In conclusion, the leg ulcer and thigh telangiectatic lesions observed in this patient with RA, splenomegaly, and neutropenia led further investigation, resulting in the diagnosis of a pseudo-Felty syndrome, supported by the association of NK-cell CD56 e LGL. In our patient, the LGL leukemia became aggressive, with skin infiltration, systemic manifestations, multiorgan failure, and death before the institution of chemotherapy.…”