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Non-B, non-T neoplasms with lymphoblast morphology: further clarification and classification
Kennosuke Karube1, Koichi Ohshima, Takeshi Tsuchiya
1Department of Pathology, School of Medicine, Fukuoka University, Nanakuma 7-45-1, Jonan-ku, Fukuoka 814-0180, Japan. xs255@cis.fukuoka-u.ac.jp
Insights
This study classified 158 lymphoblastic lymphoma cases, finding non-B, non-T types have poorer prognoses. Aggressive treatments improved outcomes for these subtypes, suggesting a need for refined classification of blastic NK lymphoma.
Area of Science:
- Hematology
- Oncology
- Immunopathology
Background:
- Lymphoblastic lymphoma (LBL) classification is crucial for prognosis and treatment.
- Immunophenotyping and immunohistochemistry are key diagnostic tools for lymphoid neoplasms.
- The World Health Organization (WHO) classification evolves, requiring re-evaluation of disease entities.
Purpose of the Study:
- To characterize morphologic, immunohistochemical, and clinical features of 158 LBL cases.
- To classify LBL into B-cell, T-cell, and non-B, non-T types.
- To investigate subtypes within the non-B, non-T category and their clinical implications, particularly blastic NK lymphoma.
Main Methods:
- Morphologic and immunohistochemical analysis of 158 LBL cases.
- Immunophenotyping to classify cases into B-cell, T-cell, and non-B, non-T lineages.
- Subclassification of non-B, non-T lymphomas using specific markers (CD4, CD7, CD33, CD56, CD123).
Main Results:
- Cases were classified: 53 B-cell, 84 T-cell, and 21 non-B, non-T.
- Non-B, non-T subtypes included CD7+ stem cell lymphoma, blastic NK cell lymphoma (B-NKL), myeloid/NK precursor cell leukemia, and CD4+CD56+ hematodermic malignancy.
- CD4+CD56+ types were associated with skin lesions; CD7+SCL and M/NKL with bone marrow/mediastinal involvement.
- Non-B, non-T lymphomas had poorer prognoses than T-cell types.
- Aggressive chemotherapy and stem cell transplantation improved prognosis for non-B, non-T subtypes.
Conclusions:
- Non-B, non-T lymphoblastic lymphomas, including subtypes of blastic NK lymphoma, present distinct clinical features and prognoses.
- The current WHO classification of blastic NK lymphoma may require refinement, potentially separating B-NKL from the CD4+CD56+ type.
- Aggressive treatment strategies, including stem cell transplantation, offer improved survival for patients with aggressive non-B, non-T LBL.
Abstract:
We studied the morphologic, immunohistochemical, and clinical characteristics of 158 cases of lymphoblastic lymphoma. Based on immunophenotyping and cell lineage, cases were classified into B-cell type (CD20,CD19 or CD79a+, n = 53), T-cell type (surface CD3+, n = 84), and non-B, non-T type (B cell marker- and surface CD3-, n = 21). The latter group was further divided based on immunohistochemistry into: 1) CD7+ stem cell lymphoma (CD7+SCL) [CD4-, CD7+, CD33+/-, CD56-], 2) blastic natural killer cell lymphoma (B-NKL) [CD4-, CD7+/-, CD33-, CD56+, CD123-], 3) myeloid/NK precursor cell leukemia (M/NKL) [CD4-, CD7+, CD33+, CD56+], and 4) CD4+CD56+ hematodermic malignancy (CD4+CD56+) type [CD4+, CD7+/-, CD33-, CD56+, CD123+]. The CD7+SCL and M/NKL types frequently exhibited bone marrow invasion and mediastinal masses. All CD4+CD56+ types were associated with skin lesions. B-NKL type is included into Blastic NK lymphoma in new World Health Organization classification with CD4+CD56+ type. But the cases of B-NKL were more reminiscent of CD7+SCL or M/NKL type than the CD4+CD56+ type, both clinically and histologically. We propose that blastic NK lymphoma, a disease entity in the new WHO classification, should be divided into two types based on phenotypes and clinical features. The non-B, non-T lymphomas exhibited poorer prognoses, similar to that of B-cell lymphomas, than T-cell type tumors (P = 0.009). Among the 21 tumors, the prognosis of the four subtypes did not differ significantly; however, cases receiving aggressive chemotherapy and stem cell transplantation had a more favorable prognosis than those receiving only traditional chemotherapy and radiation therapy (P = 0.0089).
