NK‐cell related neoplasms in Osaka, Japan

Abstract

The frequency of NK-cell related neoplasms was estimated among lymphoproliferative diseases diagnosed and treated in Osaka, Japan, from 1999 to 2003. The total number of registered cases was 1,400, among which 1,092 patients were diagnosed as having malignant lymphomas. There were 987 cases of non-Hodgkin's lymphoma (NHL) and 105 (9.6%) of Hodgkin's lymphoma. Immunophenotypic analysis revealed that 743 patients had B-cell lymphomas and 209 T/NK-cell lymphomas. Among the T/NK-cell lymphomas, 40 showed positive immunoreactivity for CD56, and thus they were judged to be NK/T-cell lymphomas. They included one blastic NK-cell lymphoma and 39 NK/Tcell lymphomas. NK/T-cell lymphomas were further divided into three categories based on the main site of lesions: nasal type (23 cases), non-nasal extranodal type (11 cases), and nodal type (5 cases). The positive rate of infection with the Epstein-Barr virus determined by in situ hybridization was 83%, 36%, and 25% in the nasal, non-nasal, and nodal type, respectively. A mosquito allergy was found in one patient with EBVpositive non-nasal NK/T-cell lymphoma. The present study showed that the frequency of NK-cell related neoplasms among all NHLs was 4% in an ATL-non-endemic area of Japan.

Original paper Evaluation of the role of Epstein-Barr virus in cases of nodal or extranodal T- and NK-cell lymphoma using eber in situ hybridization

“…Similarly PTCL, NOS was the most prevalent subtype in our patient population, followed by ALCL. However, the second most prevalent type reported was extranodal NK/T-cell lymphoma, nasal type in Eastern countries, accounting for 4% to 8% of all cases of NHL [5,8,30,31]. The extranodal NK/T-cell lymphoma, nasal type was scarce in our dataset, and adult T-cell leukaemia/lymphoma was not found, which is more similar to Western case series.…”

“…Studies gathering information from different geographic areas, including America, Europe, Asia, and South Africa, have documented that mature T-and NK-cell neoplasms comprise about 12% to 15% of all NHLs [2,3]. The percentage of T-and NK-cell neoplasms among all NHLs is 7% to 10% in Western countries [2,28], 25% in Korea [29], 21% in Osaka [30], a region of Japan not endemic for adult T-cell leukaemia/lymphoma, and 18% in South Taiwan [31]. According to these studies, PTCL, NOS is the most frequent subtype, followed by ALCL, AITCL, and extranodal NK/T-cell lymphoma, nasal type, which comprise 3.7%, 2.4%, 1.2%, and 1.4%, respectively, of all NHLs.…”

“…Miyazato et al [30] identified EBER-positive staining by in situ hybridization in 83%, 36%, and 25% of nasal, non-nasal, and nodal NK/T-cell lymphomas, respectively. The largest series by Oshimi et al [34] found EBER-positive cells in 100% of nasal and 88% of extranasal cases.…”

Chronic active Epstein-Barr virus infection of natural killer cells presenting as severe skin reaction to mosquito bites

“…Despite varying classifications, a form of chronic active EBV infection (CAEBV) involving NK cells presents with severe inflammatory and necrotic skin reactions considered pathognomonic of EBV 1 NK cell lymphoproliferative disease. [1][2][3] Most patients presenting with this condition are of Asian descent, and there is no sex predominance. Skin biopsy from a bullous lesion revealed subepidermal bullae with a dense dermal infiltrate of eosinophils, lymphocytes, and histiocytes and a negative gram stain analysis (hematoxylin-eosin stain; Figs 5 and 6).…”

Annular Erythematous Patches as the Presenting Sign of Extranodal Natural Killer/T-Cell Lymphoma

“…It usually presents as a localized disease in the upper aerodigestive tract, from the nasal cavity to the hypopharynx [2,3], but it may rapidly extend to the neighboring tissues and disseminate to various organs such as the small intestine, epiglottis, testes, adrenal gland, kidneys, and breasts [4,5]. As nasal/upper aerodigestive tract involvement may only cause nonspecific symptoms in the early period, diagnosis may be initially established based upon skin lesions [6].…”