Monomorphic Agranular Natural Killer Cell Lymphoma/Leukemia with No Epstein-Barr Virus Association

Matano, Sadaya; Nakamura, Shinobu; Nakamura, Shigeo; Annen, Yusei; Hattori, Noritaka; Kobayashi, Kihei; Kyoda, Katsunori; Sugimoto, Tatsuho

doi:10.1159/000040955

Cited by 30 publications

(30 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…NK-cell neoplasms are characterized by the presence of EBV in tumor cells; however, tumor cases/cell lines without EBV have been reported. 25,[37][38][39][40] In our series, EBV status has no impact on GEP either in tumor specimen or in cell lines. Our molecular classifier not only identified gd T-cell cases of ENKTL, but also a subset of gd-PTCL (NHS) in other extranodal areas that showed a strikingly similar GEP to NK cells, NK-cell lines and to gd T-cell lines derived from ENKTL.…”

Section: Discussionmentioning

confidence: 83%

Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic γδ T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro

et al. 2010

View full text Add to dashboard Cite

Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic cd T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro Natural killer (NK) cell lymphomas/leukemias are rare neoplasms with an aggressive clinical behavior. The majority of the cases belong to extranodal NK/T-cell lymphoma, nasal type (ENKTL) in the current WHO classification scheme. Geneexpression profiling (GEP) of 21 ENKTL and NK-cell lymphoma/ leukemia patients, 17 NK-and T-cell lines and 5 indolent NK-cell large-granular-lymphocytic proliferation was performed and compared with 125 peripheral T-cell lymphoma (PTCL) patients previously studied. The molecular classifier derived for ENKTL patients was comprised of 84 transcripts with the majority of them contributed by the neoplastic NK cells. The classifier also identified a set of cd-PTCLs both in the ENKTL cases as well as in cases initially classified as PTCL-not otherwise specified. These cd-PTCLs expressed transcripts associated with the T-cell receptor (TCR)/CD3 complex, suggesting T cell rather than NK-cell lineage. They were very similar to NK-cell tumors by GEP, but were distinct from cytotoxic (ab)-PTCL and hepatosplenic T-cell lymphoma, indicating derivation from an ontogenically and functionally distinct subset of cd T cells. They showed distinct expression of Vc9, Vd2 transcripts and were positive for TCRc, but negative for TCRb by immunohistochemistry. Targeted inhibition of two oncogenic pathways (AURKA and NOTCH-1) by small-molecular inhibitors induced significant growth arrest in NK-cell lines, thus providing a rationale for clinical trials of these inhibitors in NK-cell malignancies.

show abstract

Section: Discussionmentioning

confidence: 83%

Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic γδ T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro

et al. 2010

View full text Add to dashboard Cite

show abstract

“…Similar to acute leukemias, blastic NK-cell leukemia/ lymphoma has a predilection for multi-organ involvement, lymphadenopathy, splenomegaly, and an aggressive course with rapid bone marrow involvement [2,[5][6][7][8][9][10][11][12][13][14][15]. Thrombocytopenia, anemia, and leukopenia may be observed [15].…”

Section: Discussionmentioning

confidence: 99%

“…Monotonous infiltrates of medium-sized cells with fine chromatin are observed, resembling lymphoblastic or myeloblastic leukemias [2,14,16]. Azurophilic granules are infrequently seen in blastic NK-cell leukemia/lymphoma relative to the NKcell malignancies derived from mature cytotoxic cells [2,9,12,14].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Complete remission in advanced blastic NK‐cell lymphoma/leukemia in elderly patients using the hyper‐CVAD regimen

et al. 2003

View full text Add to dashboard Cite

Although its cell of origin is still controversial, the blastic NK-cell leukemia/lymphoma clearly represents a distinct type of hematopoietic neoplasm that is particularly clinically aggressive when it occurs in elderly patients as a disseminated, multi-organ disease. Consistently effective treatments have not been developed for this malignancy. The present report describes two elderly patients with widespread blastic NK-cell leukemia/ lymphoma involving the skin, bone marrow, peripheral blood, lymph nodes, and viscera.

show abstract

“…18 Recently, 25 cases of blastic NK cell leukemia/lymphoma were reported and the prognosis of NK cell lymphoma was very poor. 3,[6][7][8][9][10][11][12][13][14][15][16][17] It was possible that the patients were generally too old to tolerate intensive treatment including allogeneic stem cell transplantation. Among the 25 patients, only 5 (aged 24 to 34) were alive and in CR.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of relapsed blastic natural killer cell lymphoma with unrelated cord blood transplantation

Yoshimasu

Manabe

Tanaka

et al. 2002

Bone Marrow Transplant

View full text Add to dashboard Cite

Summary:The prognosis for blastic natural killer (NK) cell lymphoma is generally dismal. We report a patient who was successfully treated with unrelated cord blood transplantation (UCBT). A 15-year-old boy was diagnosed as having blastic NK cell lymphoma in the cervical lymph nodes. Autologous peripheral blood stem cell transplantation was performed on achieving a complete remission. However, the disease recurred in the bone marrow 6 months later. Chemotherapy induced a second remission and the patient received UCBT with a conditioning regimen consisting of total body irradiation, thiotepa and cyclophosphamide. Chronic GVHD of the lung occurred, but it was well controlled with steroids. At the time of writing, he remains in remission 18 months after UCBT with an excellent performance status. UCBT may be an option for patients with blastic NK cell lymphoma. Bone Marrow Transplantation (2002) 30, 41-44. doi: 10.1038/sj.bmt.1703597 Keywords: blastic natural killer cell lymphoma; unrelated cord blood transplantation; bone marrow relapse Natural killer (NK) cell leukemia was first characterized as a CD3 Ϫ /CD56 + subtype of large granular lymphocyte leukemia.1 Subsequently, other investigators proposed two NK precursor cell malignancies: myeloid/NK precursor cell acute leukemia and blastic NK cell leukemia/lymphoma. 2-4The cells of blastic NK cell leukemia/lymphoma are considered to be more mature than those of myeloid/NK precursor cell acute leukemia.5 Blastic NK cell leukemia/ lymphoma may be identical to lymphoblastic lymphoma or acute lymphoblastic leukemia with a CD56 + , surface CD3(sCD3) Ϫ , non B and non-myeloid phenotype. 5 The prognosis for blastic NK cell leukemia/lymphoma is very poor with standard chemotherapy. There have been few reports describing the outcome of patients who received allogeneic stem cell transplantation.3,6-17 We report a favorable clinical course of a patient with blastic NK cell lymphoma who received unrelated cord blood transplantation (UCBT) during the second complete remission (CR). Case reportA 15-year-old boy received medical attention for large cervical lymph nodes and weight loss in December 1998. Physical examination was unrevealing apart from the palpable cervical, axillary and inguinal lymph nodes. Leukocyte, red blood cell and platelet counts were within normal limits. Results of blood chemistry and a coagulation test were also within the normal range. A biopsy of a cervical lymph node was performed and a diagnosis of blastic NK cell leukemia/lymphoma was made based on histological and immunohistochemical examination (Figure 1). A flow cytometric analysis of the surface antigens demonstrated Figure 1 Blastic NK cell lymphoma of the cervical node. Picture shows a diffuse monotonous infiltrate of medium-sized cells with fine chromatin and scanty cytoplasm, resembling lymphoblasts (H&E, ϫ400).

show abstract

Monomorphic Agranular Natural Killer Cell Lymphoma/Leukemia with No Epstein-Barr Virus Association

Cited by 30 publications

References 6 publications

Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic γδ T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro

Natural killer cell lymphoma shares strikingly similar molecular features with a group of non-hepatosplenic γδ T-cell lymphoma and is highly sensitive to a novel aurora kinase A inhibitor in vitro

Complete remission in advanced blastic NK‐cell lymphoma/leukemia in elderly patients using the hyper‐CVAD regimen

Successful treatment of relapsed blastic natural killer cell lymphoma with unrelated cord blood transplantation

Contact Info

Product

Resources

About