Cutaneous B-cell lymphoma - Immunocompromised, HIV and AIDS
There is still controversy regarding precise classification, which requires a synthesis of clinical, histopathologic, immunophenotypic, and molecular features. The World Health Organization-European Organization of Research and Treatment of Cancer (WHO-EORTC) classifies disease as the following: follicle center cell (Crosti's) lymphoma, marginal zone lymphoma (immunocytoma), large B-cell lymphoma, cutaneous plasmacytoma, and intravascular large B-cell lymphoma (which, in contrast to the others, is usually systemic and previously known as malignant angioendotheliomatosis).
Lymphomatoid granulomatosis is another angiocentric and angiodestructive Epstein-Barr virus (EBV) associated extranodal lymphoma. Lymphomatoid granulomatosis typically involves the lungs and may involve the skin. CBCL may occur more frequently in some areas of the world; it represents only 4.5% of all cases of primary cutaneous lymphoma is the US versus up to 26% in Europe. Disease in Europe has been associated with Borrelia spp. infection, but the pathogenesis of most cases is unknown. Immune dysregulation may play a role, as cutaneous lesions due to non-Hodgkin lymphoma may occur in HIV-infected and immunocompromised patients. HIV-infected patients with CBCL were often characterized (in contrast to immunocompetent patients with CBCL) by CD 30+, EBV+ phenotypes.
CBCL is usually asymptomatic when confined to the skin. When systemic disease exists, symptoms may include weight loss, fever, fatigue, malaise, and night sweats; lymphadenopathy may be present.
Patients receiving chemotherapy and radiotherapy are at increased risk of non-Hodgkin lymphoma as well as those with immunodeficiency states and those with connective tissue disease. EBV-associated lymphoma occurs more often in immunocompromised patients.
Most CBCLs are low-grade malignancy with indolent behavior and a good prognosis, except for large B-cell lymphomas, B-cell lymphoblastic lymphoma, and lymphomatoid granulomatosis, which are considered systemic diseases.
In primary cutaneous large B-cell lymphoma there are very common (76%) MYD88 somatic mutations that can be useful criteria for diagnosis.
Related Topic: Non-Hodgkin lymphoma
C85.10 – Unspecified B-cell lymphoma, unspecified site
402881008 – Cutaneous B-cell lymphoma
- Cutaneous pseudolymphoma (parapsoriasis, actinic reticuloid [see chronic actinic dermatitis], Jessner's benign lymphocytic infiltrate)
- Lymphomatoid papulosis
- Cutaneous T-cell lymphoma (Sezary syndrome)
- Leukemia cutis
- Metastatic malignancy
- Mycobacterial infection
- Mycobacterium marinum
- Erythema nodosum
- Tertiary syphilis
- Bacillary angiomatosis
- Kaposi sarcoma
- B-cell chronic lymphocytic leukemia