HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable: Difference between revisions

Created page with "==Primary Author(s)*== * Snehal Patel, MD, PhD __TOC__ ==Graphical Data Links== * N/A ==General Disease Overview / Description of Cancer Category== Splenic B-cell lymphom..."
 
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==General Disease Overview / Description of Cancer Category==
==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|mature B-cell neoplasms]] and are overall rare, accounting for <1% of lymphomas.  Two entities in this category, [[Hairy Cell Leukemia Variant|hairy cell leukemia variant]] (HCL-v) and [[Splenic Diffuse Red Pulp Small B-cell Lymphoma|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|splenic marginal zone lymphoma]] and [[Hairy Cell Leukemia|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.
Splenic B-cell lymphoma/leukemia, Unclassifiable (SBLL-U) includes primary splenic lymphomas that cannot be better classified into other [[HAEM4:Mature B-Cell Neoplasms|mature B-cell neoplasms]] and are overall rare, accounting for <1% of lymphomas.  Two entities in this category, [[HAEM5:Splenic B-cell lymphoma/leukaemia with prominent nucleoli|hairy cell leukemia variant]] (HCL-v) and [[HAEM5:Splenic diffuse red pulp small B-cell lymphoma|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 [[HAEM5:Splenic marginal zone lymphoma|splenic marginal zone lymphoma]] and [[HAEM5:Hairy cell leukaemia|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)==
==WHO Classification Pages (Includes Links to Content)==


* [[Splenic Diffuse Red Pulp Small B-cell Lymphoma]]
* [[HAEM5:Splenic diffuse red pulp small B-cell lymphoma]]
* [[Hairy Cell Leukemia Variant]]
* [[HAEM5:Splenic B-cell lymphoma/leukaemia with prominent nucleoli]]


==Other Related Pages (Includes Links to Content)==
==Other Related Pages (Includes Links to Content)==
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Differential diagnosis:  other small B-cell lymphomas with cytoplasmic projections and significant splenic involvement
Differential diagnosis:  other small B-cell lymphomas with cytoplasmic projections and significant splenic involvement


* [[Splenic Marginal Zone Lymphoma]]
* [[HAEM5:Splenic marginal zone lymphoma]]
* [[Hairy Cell Leukemia]]
* [[HAEM5:Hairy cell leukaemia]]


==Additional Information==
==Additional Information==