淋巴瘤常见类型的一些罕见病理特征-(李小秋)
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Unusual examples of some commonly seen non-Hodgkin
lymphoma subtypes
Xiao-Qiu Li M.D., Ph.D.
Fudan University Shanghai Cancer Center
April 17, Fuzhou
Unusual categories of non-Hodgkin lymphoma (NHL)
Uncommon clinical features (e.g., location, age, etc.)
Unusual morphologic features
Distribution/growth pattern Cytology of the neoplastic cells Reactive cellular component Stromal changes
CD20
FL
RFH
Βιβλιοθήκη Baidu
CD20
CD3
CD10
FL
BCL2
FL, floral variant
CD10
BCL2
CD3
Follicular variant of PTCL, NOS (PTCL-F)
A rare morphological variant of PTCL characterized by a nodular growth pattern
Diverse patterns: intrafollicular, PTGC/NLPHL-like, perifollicular/MZL-like, or mixed ones
Uniform, small to medium-sized neoplastic clear cells Limited foci of paracortical hyperplasia (HEVs,
May histologically mimic reactive lymphoid hyperplasia due to the intact architecture: GCs are usually preserved or hyperplastic and are surrounded by a normal or mildly thickened, eccentric mantle zones
Rare but well-defined categories in the WHO classification
Newly described diseases/entities not included in the WHO classification yet
Composite lymphoma Grey zone lymphoma
Some conventional NHL subtypes may display unusual
clinicopathologic features as well
Unusual morphologic variants /patterns
Aberrant phenotypes or genetic changes
PTCL-F may represent a peculiar stage of AITL, featuring the follicular distribution of tumour cells A. Some similar or overlapping features are present - Perifollicular growth pattern - Medium-sized cells with abundant cytoplasm and round nuclei, admixed with occasional EBV+ IBs - TFH derivation B. Composite lesions or metachronous lymphomas C. Similar genetic changes have been found in AITL, too
SYK protein has transforming properties in vitro Usually corresponds to early stage disease
CD21
CD3
CD10
CD4
CD8
EBER&CD20
A possible relationship between PTCL-F and AITL
Cellular atypia is present, but not easily appreciable, and a definite diagnosis relies more on cyclin D1 staining
plasma cells, eosinophils) is always appreciable A characteristic TFH phenotype: CD4+, CD10+,
BCL6+, CD57+, PD1+, CXCL13+, ICOS+ < 20% of cases contain t(5;9)(q33;q22)/ITK-SYK, ITK-
EBER
CD20 CD30
t(11;14)(q13;q32)
/BCL1-IGH
cyclin D1
“In situ MCL” lesions
Refers to rare cases which show in situ involvement by MCL-like cells, which almost exclusively restricted to the inner mantle zones
Hence the category of PTCL-F will be placed together with AITL rather than PTCL, NOS in the 5th edition of the WHO classification
ALCL, ALK+
CD30
ALK1
CD56
lymphoma subtypes
Xiao-Qiu Li M.D., Ph.D.
Fudan University Shanghai Cancer Center
April 17, Fuzhou
Unusual categories of non-Hodgkin lymphoma (NHL)
Uncommon clinical features (e.g., location, age, etc.)
Unusual morphologic features
Distribution/growth pattern Cytology of the neoplastic cells Reactive cellular component Stromal changes
CD20
FL
RFH
Βιβλιοθήκη Baidu
CD20
CD3
CD10
FL
BCL2
FL, floral variant
CD10
BCL2
CD3
Follicular variant of PTCL, NOS (PTCL-F)
A rare morphological variant of PTCL characterized by a nodular growth pattern
Diverse patterns: intrafollicular, PTGC/NLPHL-like, perifollicular/MZL-like, or mixed ones
Uniform, small to medium-sized neoplastic clear cells Limited foci of paracortical hyperplasia (HEVs,
May histologically mimic reactive lymphoid hyperplasia due to the intact architecture: GCs are usually preserved or hyperplastic and are surrounded by a normal or mildly thickened, eccentric mantle zones
Rare but well-defined categories in the WHO classification
Newly described diseases/entities not included in the WHO classification yet
Composite lymphoma Grey zone lymphoma
Some conventional NHL subtypes may display unusual
clinicopathologic features as well
Unusual morphologic variants /patterns
Aberrant phenotypes or genetic changes
PTCL-F may represent a peculiar stage of AITL, featuring the follicular distribution of tumour cells A. Some similar or overlapping features are present - Perifollicular growth pattern - Medium-sized cells with abundant cytoplasm and round nuclei, admixed with occasional EBV+ IBs - TFH derivation B. Composite lesions or metachronous lymphomas C. Similar genetic changes have been found in AITL, too
SYK protein has transforming properties in vitro Usually corresponds to early stage disease
CD21
CD3
CD10
CD4
CD8
EBER&CD20
A possible relationship between PTCL-F and AITL
Cellular atypia is present, but not easily appreciable, and a definite diagnosis relies more on cyclin D1 staining
plasma cells, eosinophils) is always appreciable A characteristic TFH phenotype: CD4+, CD10+,
BCL6+, CD57+, PD1+, CXCL13+, ICOS+ < 20% of cases contain t(5;9)(q33;q22)/ITK-SYK, ITK-
EBER
CD20 CD30
t(11;14)(q13;q32)
/BCL1-IGH
cyclin D1
“In situ MCL” lesions
Refers to rare cases which show in situ involvement by MCL-like cells, which almost exclusively restricted to the inner mantle zones
Hence the category of PTCL-F will be placed together with AITL rather than PTCL, NOS in the 5th edition of the WHO classification
ALCL, ALK+
CD30
ALK1
CD56