Cutaneous B-Cell Lymphoma - Classification, Causes, and Treatment

What Is Cutaneous B-Cell Lymphoma?

The term cutaneous B-cell lymphoma comprehends a variety of lymphoproliferative disorders due to the clonal proliferation of B-cells primarily involving the skin. The lack of apparent extra-cutaneous illness is required to diagnose cutaneous B-cell lymphoma since they have completely different clinical behavior and prognosis.

What Is the Epidemiology of Cutaneous B-Cell Lymphoma?

Cutaneous B-cell lymphoma accounts for up to 25 percent to 29 percent of total primary cutaneous lymphomas, with an annual incidence of 0.5 to 1 new case per 100,000 population.

Cutaneous B-cell lymphoma is more prevalent in non-Hispanic White men. However, the prevalence of cutaneous B-cell lymphoma increases with age.

What Is the Classification of Cutaneous B-Cell Lymphoma?

The World Health Organization classifies cutaneous B-cell lymphomas as follows:

1. Primary cutaneous marginal zone B-cell lymphoma (PCMZL).

2. Primary cutaneous follicle center lymphoma (PCFCL).

3. Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL).

4. Leg type (LT).

5. Primary cutaneous diffuse large B-cell lymphoma.

What Is the Etiology of Cutaneous B-Cell Lymphoma?

As the prevalence of cutaneous B-cell lymphoma increases with age. Specific factors that may contribute to the development of cutaneous B-cell lymphoma are:

• Chronic inflammation.

• DNA damage.

• Weakened immunity.

What Is Primary Cutaneous Marginal Zone Lymphoma?

Primary cutaneous marginal zone lymphoma is an indolent B-cell lymphoma consisting of small B-cells, lymphoplasmacytic cells, and mature plasma cells. It belongs to the extranodal marginal zone lymphoma (MZL) subtype of mucosa-associated lymphoid tissue (MALT) lymphoma.

Primary cutaneous marginal zone lymphoma shares pathogenic mechanisms and biological similarities with its systemic mucosa-associated lymphoid tissue (MALT) lymphoma.

What Is Primary Cutaneous Follicle Center Lymphoma?

Primary cutaneous follicle center lymphoma accounts for roughly 11 percent to 18 percent of all cutaneous lymphomas. It is the most prevalent variety of cutaneous B-cell lymphoma, accounting for approximately 55 percent of all cutaneous B-Cell lymphoma (CBCL).

What Are Primary Cutaneous Diffuse Large B-Cell Lymphoma And Leg Type?

Primary cutaneous diffuse large B-cell lymphoma and leg type is a kind of cutaneous B-cell lymphoma (CBCL) that is aggressive, with skin lesions mainly on the legs. They have been separated from other cutaneous B-cell lymphomas as they may have large B-cells. Primary cutaneous diffuse large B-cell lymphoma and leg type account for five percent to 10 percent of all cutaneous B-cell lymphoma (CBCL) seen in elderly ladies (male: female 1:3-4), with a peak incidence in the seventh decade of life.

What Are the Clinical Features of Cutaneous B-Cell Lymphoma?

• Primary Cutaneous Marginal Zone Lymphoma - Primary cutaneous marginal zone lymphoma manifests as erythematous-cyanotic single or multifocal, asymptomatic, or mildly irritating papules or nodules with a glossy surface and no desquamation, most commonly found on the arms or trunk. It enlarges slowly and can reach over three centimeters in diameter. In addition, primary cutaneous marginal zone lymphoma may occasionally show many erythematous papules located symmetrically over the face, simulating a granulomatous rosacea pattern.
• Primary Cutaneous Follicle Center Lymphoma - Primary cutaneous follicle center lymphoma often manifests as single or clustered plaques, nodules, or tumors. Presenting with a multifocal cutaneous lesion is uncommon. Lesions are often red to violet in color, with a smooth, lustrous mamillated surface. A typical feature is the appearance of erythematous papules and indurated plaques around the tumor.
• Primary Cutaneous Diffuse Large B-cell Lymphoma And Leg Type - It is a fast developing, red to bluish-red hard tumor on one or both legs, often below the knee. Other areas, such as the trunk, head-neck, and upper arms, are also affected in 10 percent to 15 percent of people. Multiple lesions might spread or cluster, and extracutaneous spread often occurs in lymph nodes, bone marrow, and the neurological system.

What Is the Treatment for Cutaneous B-Cell Lymphoma?

1. Primary Cutaneous Marginal Zone Lymphoma -

Treatment recommendations for primary cutaneous marginal zone lymphoma need a multidisciplinary examination by a dermatologist, medical oncologist, hematologist, and radiotherapist.

• Localized Lesions-

  • The first-line treatments for a single small lesion or multiple localized lesions are radiotherapy or surgical excision.

  • Oral antibiotics such as Cephalosporins or Tetracyclines can be used in selected instances with positive serology for persistent Borrelia burgdorferi infection.

  • In these situations, intralesional (IL) IFN-alpha, IL Rituximab, and IL corticosteroid are all feasible alternatives for second-line therapy.

• Multifocal Lesion-

  • Local radiation, intravenous Rituximab, oral antibiotics, and Chlorambucil are all well-tolerated and safe choices in multifocal illnesses.

  • IL IFN-alpha, IL Rituximab, and topical or IL steroids are among the second-line therapies.

  • A wait-and-see approach with active therapies limited to symptomatic lesions is a viable choice in all situations.

2. Primary Cutaneous Follicle Center Lymphoma -

• Localized radiation in persons with single or isolated skin lesions, a dosage of at least 30 Gy, and a margin of clinically uninvolved skin of at least 1 to 1.5 cm is the treatment of choice.

• However, surgical excision is a viable alternative for well-defined, single lesions. In addition, surgery is an equally effective primary cutaneous follicle center lymphoma (PCFCL) treatment option that avoids problems associated with radiation therapy.

• Second-line options include IL therapy with IFN-alpha or Rituximab.

• Topical treatments such as high-potency steroids, Imiquimod, nitrogen mustard, Bexarotene, and IL steroids are effective in a subset of symptomatic individuals.

• Systemic Rituximab (375 mg/m2 weekly for one to eight weeks) can be used in individuals with prevalent skin lesions, especially when local therapy is ineffective or undesirable.

3. Primary Cutaneous Diffuse Large B-cell Lymphoma And Leg Type -

• Immunochemotherapy with R-CHOP and radiation is the first-line treatment for solitary, localized, or widespread lesions.

• Local radiation or Rituximab as a single treatment is also used as a treatment.

• For relapsed systemic lymphoma, autologous stem cell transplantation is the standard of treatment.

• Oral Lenalidomide monotherapy has significant clinical efficacy, although it is currently being studied since new monoclonal antibodies and tyrosine kinase inhibitors are being developed.

Conclusion:

Cutaneous B-cell lymphoma (CBCL) is a lymphoma that occurs outside of the lymph nodes. After the gastrointestinal system, the skin is the second most prevalent location of extranodal involvement. Therefore, cutaneous B-cell lymphoma (CBCL) originates in the skin, and the absence of systemic illness is required for a cutaneous B-cell lymphoma (CBCL) diagnosis. Most cutaneous B-cell lymphoma (CBCL) is inactive or slow developing. They may show as a reddish rash, lump, or nodule on the skin, and since they form in the dermis or second layer of skin, they may be slightly elevated and smooth. Immediate care must be taken as soon as any symptom arises.