Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor): prognostication and categorization according to anatomic sites of involvement.
BACKGROUND
Blastic natural killer (NK) cell lymphoma/leukemia (BNKL) is an immature CD56-positive neoplasm, which was recognized recently and characterized by systemic proliferation of tumor cells including skin, lymph node, and bone marrow.


METHODS
The current study analyzed 47 patients with BNKL (27 had leukemias and 20 had lymphomas). Patient data were collected for the survey of the NK-Cell Tumor Study Group.


RESULTS
There were 33 males and 14 females, with a median age of 53 years (range, 3 months to 89 years). There were few clinicopathologic differences between the leukemia and lymphoma types. Cutaneous involvement was noted at diagnosis in 28 patients, who presented a tendency for older age of onset (median: 56 vs. 46 years, P = 0.11) than patients with noncutaneous BNKL. Cutaneous BNKL showed less frequent mediastinal involvement (4% vs. 53%, P = 0.0002) and less severe thrombocytopenia (P =0 .03). Phenotypic characteristics were also different, with cutaneous BNKL favoring CD4 and HLA-DR expression, and noncutaneous BNKL favoring CD16 and CD34 expression. Both groups responded well to chemotherapy for lymphoid malignancies, but disease recurrence was frequent. The prognosis of patients with noncutaneous BNKL was significantly poorer than that of patients with cutaneous BNKL (median survival: 15 vs. 25 months, P = 0.02). Multivariate analysis confirmed that cutaneous involvement was a significant and independent prognostic factor for BNKL, as were age of onset and leukocyte count.


CONCLUSIONS
These findings suggested that BNKL is a heterogeneous disease and contains at least two subtypes. Although further investigations are needed to settle a marker for distinction, the presence of cutaneous involvement is a useful prognostic factor.
Division of Molecular Medicine, Aichi Cancer Center, Nagoya, Japan.
Ritsuro
R
Suzuki
Shigeo
S
Nakamura
Junji
J
Suzumiya
Koichi
K
Ichimura
Masako
M
Ichikawa
Kiyoyuki
K
Ogata
Yoshimasa
Y
Kura
Keiko
K
Aikawa
Hirofumi
H
Teshima
Masahiro
M
Sako
Hiroshi
H
Kojima
Mitsufumi
M
Nishio
Tadashi
T
Yoshino
Hiroki
H
Sugimori
Keisei
K
Kawa
Kazuo
K
Oshimi
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019002:CD56 Antigen / Q000032:analysis*; D004253:DNA Nucleotidylexotransferase / Q000032:analysis; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D053650:Interleukin-3 Receptor alpha Subunit; D007621:Karyotyping; D007694:Killer Cells, Natural / Q000473:pathology*; D007945:Leukemia, Lymphoid / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016185:Receptors, Interleukin-3 / Q000032:analysis; D012878:Skin Neoplasms / Q000276:immunology / Q000401:mortality / Q000628:therapy
D019002:CD56 Antigen; C115022:IL3RA protein, human; D053650:Interleukin-3 Receptor alpha Subunit; D016185:Receptors, Interleukin-3; D004253:DNA Nucleotidylexotransferase