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HAEM4:Splenic Diffuse Red Pulp Small B-cell Lymphoma (view source)
Revision as of 16:33, 4 December 2023
, 16:33, 4 December 2023no edit summary
==Cancer Category/Type==
==Cancer Category/Type==
*[[Mature B-Cell Neoplasms|Mature B-Cell Neoplasm]]
*[[HAEM4:Mature B-Cell Neoplasms|Mature B-Cell Neoplasm]]
==Cancer Sub-Classification / Subtype==
==Cancer Sub-Classification / Subtype==
*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
==Definition / Description of Disease==
==Definition / Description of Disease==
*SDRPL is a provisional WHO entity and definitive diagnostic criteria have not been determined
*SDRPL is a provisional WHO entity and definitive diagnostic criteria have not been determined
**Diagnosis of exclusion
**Diagnosis of exclusion
***Differential of splenic lymphomas with cytoplasmic projections: [[Hairy Cell Leukemia|HCL]], [[Hairy Cell Leukemia Variant|HCL-v]], [[Splenic Marginal Zone Lymphoma|SMZL]]
***Differential of splenic lymphomas with cytoplasmic projections: [[HAEM5:Hairy cell leukaemia|HCL]], [[HAEM5:Hairy cell leukaemia Variant|HCL-v]], [[HAEM5:Splenic marginal zone lymphoma|SMZL]]
**Distinction is by clinical history, morphology, and immunohistochemistry
**Distinction is by clinical history, morphology, and immunohistochemistry
***No pathognomonic diagnostic markers (molecular or otherwise)
***No pathognomonic diagnostic markers (molecular or otherwise)
|BRAF p.Val600Glu
|BRAF p.Val600Glu
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Hairy Cell Leukemia|HCL]] but rare/absent in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Hairy cell leukaemia|HCL]] but rare/absent in SDRPL<ref name=":3" />
|-
|-
|MAP2K1
|MAP2K1
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Hairy Cell Leukemia Variant|HCL-v]] and a subset of cases classified as [[Hairy Cell Leukemia|HCL]] but uncommon in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Hairy cell leukaemia Variant|HCL-v]] and a subset of cases classified as [[HAEM5:Hairy cell leukaemia|HCL]] but uncommon in SDRPL<ref name=":3" />
|-
|-
|KLF2 and TNFAIP3
|KLF2 and TNFAIP3
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Splenic Marginal Zone Lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Splenic marginal zone lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|-
|-
|MYD88
|MYD88
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Lymphoplasmacytic Lymphoma|LPL]] and [[Splenic Marginal Zone Lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Lymphoplasmacytic lymphoma|LPL]] and [[HAEM5:Splenic marginal zone lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|-
|-
|BCOR
|BCOR
|Possible role in diagnosis (inclusion)
|Possible role in diagnosis (inclusion)
|Prevalent in SDRPL but rare/absent in [[Hairy Cell Leukemia|HCL]], [[Hairy Cell Leukemia Variant|HCL-v]], and [[Splenic Marginal Zone Lymphoma|SMZL]]<ref name=":3" />
|Prevalent in SDRPL but rare/absent in [[HAEM5:Hairy cell leukaemia|HCL]], [[HAEM5:Hairy cell leukaemia Variant|HCL-v]], and [[HAEM5:Splenic marginal zone lymphoma|SMZL]]<ref name=":3" />
|}
|}
==Links==
==Links==
*[[Hairy Cell Leukemia]]
*[[HAEM5:Hairy cell leukaemia]]
*[[Hairy Cell Leukemia Variant]]
*[[HAEM5:Hairy cell leukaemia Variant]]
*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[Splenic Marginal Zone Lymphoma]]
*[[HAEM5:Splenic marginal zone lymphoma]]
==References==
==References==