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Cutaneous B-cell lymphoma - Immunocompromised, HIV and AIDS
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Cutaneous B-cell lymphoma - Immunocompromised, HIV and AIDS

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Contributors: Marvin Turck MD
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Synopsis

Cutaneous B-cell lymphoma (CBCL) represents a group of malignant lymphomas derived from B cells in different stages of differentiation with primary location in the skin. Other types of systemic non-Hodgkin B-cell lymphoma may be manifest secondarily in the skin (such as small cell lymphocytic lymphoma and mantle cell lymphoma). It is essential to differentiate between primary and secondary skin involvement, as prognosis and therapy differ significantly.

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

Codes

ICD10CM:
C85.10 – Unspecified B-cell lymphoma, unspecified site

SNOMEDCT:
402881008 – Cutaneous B-cell lymphoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 10/19/2017
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Cutaneous B-cell lymphoma - Immunocompromised, HIV and AIDS
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Cutaneous B-cell lymphoma : Fatigue, Fever, Night sweats, Scattered few, Scattered many, Smooth nodule, Smooth plaque, Weight loss
Clinical image of Cutaneous B-cell lymphoma
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