As non-Hodgkin lymphoma (NHL; abdominal lymphoma; axillary lymphoma; choroidal lymphoma; intestinal lymphoma; small bowel lymphoma; follicular germinal center lymphoma; non-follicular lymphoma; hilar lymphoma; Conjunctival lymphoma; eyelid lymphoma; lymphadenoma; lymphoma; lymphomata; lymphoma of the mediastinum ; gastric lymphoma; malignant lymphoma; marginal zone lymphoma; mycosis fungoides; NHL [non-Hodgkin lymphoma] – s. a. non-Hodgkin’s lymphoma; non-Hodgkin’s lymphoma n.d.; orbital lymphoma; primary gastric lymphoma; Sézary syndrome; cerebral lymphoma; cerebral non-Hodgkin’s lymphoma; ICD-10-GM codes: ICD-10-GM C82: follicular lymphoma; ICD-10-GM C83: non-follicular lymphoma; ICD-10-GM C84: mature T/NK cell lymphoma; ICD-10-GM C85: Other and unspecified types of non-Hodgkin’s lymphoma; ICD-10-GM C86: Other specified T/NK cell lymphomas) are used to refer to all malignant (malignant) lymphomas that are not Hodgkin’s lymphoma. These highly heterogeneous diseases (see classifications below) can vary widely in histology (fine tissue structure) and disease progression.
NHL can occur nodally or, less commonly, extranodally (“outside a lymph node”: e.g., organs, skin, etc.).
Extranodal lymphomas include MALT (“mucosa associated lymphoid tissue”) lymphomas and primary cutaneous lymphomas (skin lymphomas). In addition to primary cutaneous lymphomas, there are secondary cutaneous lymphomas that can occur as a result of primary nodal NHL.
NHLs are divided into:
- B-cell lymphomas (B-lymphoid cell; approximately 80% of all NHL; approximately 70% of all primary cutaneous lymphomas).
- T-cell lymphoma (T-lymphatic cell; 20% of all NHL; approximately 25% of all primary cutaneous lymphomas).
- NK cell lymphoma (NK cell; very rare).
Cutaneous B-cell and T-cell lymphomas (ICD-10-GM C84.-: mature T/NK-cell lymphomas) are part of non-melanoma skin cancer (NMSC).
Typical primary cutaneous lymphomas are:
- Cutaneous T-cell lymphomas (approximately 70% of all primary cutaneous lymphomas).
- Mycosis fungoides (MF) (ICD-10-GM C84.0: Mycosis fungoides))
- Sézary syndrome (ICD-10-GM: C84.1: Sézary syndrome).
- Cutaneous B-cell lymphomas (approximately 25% of all primary cutaneous lymphomas).
- Primary cutaneous follicular germinal center lymphoma, English PCFCL; is a variant of follicular lymphoma (synonyms: Follicular center lymphoma or follicular germinal center lymphoma, sometimes abbreviated FCL or FL, English follicular lymphoma or follicle center lymphoma) (ICD-10-GM C82.-: Follicular lymphoma).
- Marginal zone lymphoma (PCMCL) (ICD-10-GM C83.0: Small B-cell lymphoma).
Sex ratio: males to females is 1.5: 1. The sex ratio otherwise varies widely depending on the form of non-Hodgkin’s lymphoma in question.
Peak incidence: the maximum incidence of non-Hodgkin’s lymphoma (NHL) is in older age. NHL can occur at any age. The median age of onset is just over 60 years of age.
The incidence (frequency of new cases) is approximately 12 cases per 100,000 population per year. The incidence of primary cutaneous lymphomas, which belong to the non-Hodgkin’s lymphomas, is 1: 100,000 inhabitants per year. The disease is increasing in frequency.
Course and prognosis: The course and prognosis of non-Hodgkin’s lymphoma depends largely on the type of disease (see classification below).