SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA |
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SPTL | histologisch beeld |
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histologisch beeld | histologisch beeld |
Subcutaneous panniculitis-like T-cell lymphoma: | ||
---|---|---|
SPTL-AB | SPTL-GD | |
Immunofenotype - T-celreceptor - T-celfenotype - co-expressie CD56 |
βF1+, TCRδ1- CD3+, CD4-, CD8+ afwezig |
βF1-, TCRδ1+ CD3+, CD4-, CD8- frequent (60%) |
Infiltraat | alleen subcutis | subcutis en (epi)dermis |
Kliniek | nodi en plaques zelden ulceratie |
nodi en plaques vaak ulceratie |
Hemofagocytair syndroom |
17% | 50% |
5-jaarsoverleving - zonder HFS - met HFS |
91% 46% |
11% 11% |
1. | Willemse R. Diagnostiek en behandeling van cutane lymfomen. Ned Tijdschr Hematol 2008;5:3-11. |
2. | Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification and prognostic factors. An EORTC Cutaneous Lymphoma Group study of 83 cases. Blood 2008 Jan 15;111(2):838-845. |
31-05-2023 (JRM / EJK) - www.huidziekten.nl |
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ICD10 | C86.3 | Subcutaan T-cel-lymfoom, panniculitis-achtig |
ICD10 | C86.3 | Subcutaneous panniculitis-like T-cell lymphoma |
SNOMED | 404133000 | Subcutaneous panniculitis-like T-cell lymphoma |
DBC | 14 | Maligne dermatosen |