HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable

Primary Author(s)*

• Snehal Patel, MD, PhD

Graphical Data Links

• N/A

General Disease Overview / Description of Cancer Category

Splenic B-cell lymphoma/leukemia, Unclassifiable (SBLL-U) includes primary splenic lymphomas that cannot be better classified into other mature B-cell neoplasms and are overall rare, accounting for <1% of lymphomas. Two entities in this category, hairy cell leukemia variant (HCL-v) and splenic diffuse red pulp small B-cell lymphoma (SDRPL) may represent distinct entities and are designated as provisional entities in the 2017 WHO. Recent molecular evidence suggests that they are distinct from each other and from splenic marginal zone lymphoma and hairy cell leukemia, which which the have overlapping clinical, morphologic, and immunophenotypic features. Primary splenic lymphomas that cannot be classified as HCL-v or SDRPL should be classified as splenic B-cell lymphoma/leukemia, Unclassifiable.

WHO Classification Pages (Includes Links to Content)

• Splenic Diffuse Red Pulp Small B-cell Lymphoma
• Hairy Cell Leukemia Variant

Other Related Pages (Includes Links to Content)

Differential diagnosis: other small B-cell lymphomas with cytoplasmic projections and significant splenic involvement

• Splenic Marginal Zone Lymphoma
• Hairy Cell Leukemia

Additional Information

• N/A

References

EXAMPLE Book

1. Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171.

Notes

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*The hierarchical tumour classification structure displayed on this page is reproduced from the WHO Classification of Tumours with permission from the copyright holder, ©International Agency for Research on Cancer.