Primair cutaan marginale zone B-cel lymfoom

PRIMAIR CUTAAN MARGINALE ZONE B-CEL LYMFOOM

ICD10: C83.0

Bij een primair cutaan marginale zone B-cel lymfoom ziet men meestal solitaire of gegeneraliseerde papels, plaques of nodi, meestal op de romp en extremiteiten.


PCMZL	PCMZL


PCMZL	PCMZL

PA: nodulair of diffuus infiltraat van kleine B-cellen, waaronder marginale zone cellen, lymfoplasmacytoïde cellen en plasmacellen.

Therapie:
Radiotherapie of excisie bij solitaire laesies. Bij uitgebreide huidafwijkingen chloorambucil. Bij 50% treden cutane recidieven op, extracutane uitbreiding is zeldzaam. Goede prognose (5-jaars overleving > 95%).

Primair cutane B-cel lymfomen:
*Primary cutaneous B-cell lymphoma* - Primary cutaneous diffuse large cell B-cell lymphoma - Angioendotheliomatosis - Primary cutaneous anaplastic large cell B-cell lymphoma - Primary cutaneous diffuse large cell B-cell lymphoma of lower extremity - Primary cutaneous follicular centre B-cell lymphoma - Primary cutaneous marginal zone B-cell lymphoma - Primary cutaneous immunocytoma

Primair cutane B-cel lymfomen:

Primary cutaneous B-cell lymphoma
- Primary cutaneous diffuse large cell B-cell lymphoma
     - Angioendotheliomatosis
     - Primary cutaneous anaplastic large cell B-cell lymphoma
     - Primary cutaneous diffuse large cell B-cell lymphoma of lower extremity
- Primary cutaneous follicular centre B-cell lymphoma
- Primary cutaneous marginal zone B-cell lymphoma
     - Primary cutaneous immunocytoma

Author(s):
dr. Jan R. Mekkes. Dermatologist, Amsterdam UMC.

31-05-2023 (JRM) - www.skin-diseases.eu

Diagnosis codes:

ICD10	C83.0	Small cell B-cell lymfoom: primair cutaan marginale zone B-cell lymfoom
ICD10	C83.0	Small cell B-cell lymphoma: primary cutaneous marginal zone B-cell lymphoma
SNOMED	404140004	Primary cutaneous marginal zone B-cell lymphoma
DBC	14	Maligne dermatosen