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Primary cutaneous B-cell lymphoma (low-grade, non large cell)

  • Author(s): Moore, Megan M
  • Kovich, Olympia I
  • Brown, Lance H
  • et al.
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Primary cutaneous B-cell lymphoma (low-grade, non large cell)
Megan M Moore MD, Olympia I Kovich MD, Lance H Brown MD
Dermatology Online Journal 13 (1): 8

New York University Department of Dermatology

Abstract

A 43-year-old man presented with erythematous, indurated plaques on the scalp in the setting of a 16-year history of recurrent cutaneous tumors of the head and trunk. Clinical and histopathologic findings were consistent with a diagnosis of primary cutaneous B-cell lymphoma. Laboratory data and computed tomography imaging of the chest, abdomen, and pelvis failed to show an associated systemic lymphoma. Primary cutaneous B-cell lymphomas are a heterogenous group of lymphomas that primarily involve the skin but have variable clinical, histopathologic, and immunologic phenotypes. Successful treatment for most localized subtypes consists of surgical excision and radiation therapy. Rituximab, a chimeric monoclonal antibody that binds the B-cell-specific antigen CD20, has shown promise in treating a number of primary cutaneous B-cell lymphomas.



Clinical synopsis

A 43-year-old man was referred with a history of recurrent cutaneous tumors. The initial diagnosis of B-cell lymphoma was made in 1989 at an outside institution. The first lesion presented on the back and was treated with radiation therapy. Subsequent lesions developed on the temple, face, and left ear in 1992, 1996, 2000, and 2001. These lesions had the same histopathologic features as the initial lesion and also were successfully treated with radiation therapy. Over the 6 months prior to the current presentation, the patient noted several new lesions on the scalp. The lesions waxed and waned and were pruritic and painful. The patient had no relevant past medical history and took no medications. Review of symptoms was negative.

Three, 2-3 cm, firm, indurated, shiny, erythematous plaques were present on the left temporal scalp. There was no cervical, supraclavicular, or axillary lymphadenopathy and no hepatosplenomegaly


Figure 1Figure 2

A complete blood count, creatinine, lactate dehydrogenase, serum immunofixation, flow cytometric analysis of blood, Lyme serologies, and a bone-marrow biopsy were normal. A computed tomography scan of the chest, abdomen, and pelvis was normal.

Histopathology reveals a superficial and deep perivascular, nodular and interstitial, bottom-heavy infiltrate compromised predominantly of lymphocytes, which show a marked crush artifact. There are some preserved lymphocytes with enlarged, slightly hyperchromatic nuclei with basophilic nuclei. The epidermis and adnexal structures are spared.

Immunostains show that the infiltrate is mixed with a predominance of B-cells, which react for L-26 and CD79a. A minority of T-cells react for CD3 and UCHL-1. Approximately 80 percent of the B-cells react positively for bcl-2.


Comment

Cutaneous B-cell lymphomas may represent either primary or secondary lesions that occur in the setting of a systemic lymphoma [1]. Primary cutaneous B-cell lymphomas (PCBLS) are a heterogenous group of lymphomas that primarily involve the skin but may have variable clinical, histopathologic, and immunologic phenotypes. They represent approximately 5 percent of cutaneous lymphomas in the United States [2]. PCBLS generally follow an indolent course [3].

The World Health Organization and the European Organization for Research and Treatment of Cancer (WHO-EORTC) have agreed on a new classification system [4]. The cutaneous B-cell lymphoma classifications designated by the WHO-EORTC include: primary cutaneous marginal zone B-cell lymphoma; primary cutaneous follicle centre lymphoma; primary cutaneous diffuse large B-cell lymphoma, leg type; and primary cutaneous diffuse large B-cell lymphoma, other.

Primary cutaneous marginal zone B-cell lymphoma (PCMZL), which includes the previously designated entities of immunocytoma, plasmacytoma, and extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoma tissue (MALT lymphoma), is a neoplasm of bcl-2+, CD10- and bcl-6- lymphocytes [3]. This immunophenotype facilitates differentiation of PCMZL from primary cutaneous follicular-center lymphomas and from cutaneous lymphoid hyperplasias. Lesions often involve the trunk and arms and are multifocal in approximately 75 percent of cases. Cutaneous relapses after remission have been reported in nearly one-half of patients and are more common in the setting of multifocal disease; [5] however, 5-year survival with PCMZL approaches 100 percent. Isolated lesions may be treated effectively with surgical excision or with radiotherapy; chlorambucil therapy and radiotherapy can effectively treat multifocal lesions. In chronically relapsing disease, the risks and benefits of treatment versus expectant management must be weighed, and treatment goals may shift toward palliation.

Primary cutaneous follicular center lymphoma (PCFCL) is a neoplasm that is composed of follicular-center, small or large centrocytes. Diagnosis is primarily based on the histopathology of cell types. The neoplastic cells are bcl-6+, with variable amounts of bcl-2 and CD10 positivity [3]. Patients present with solitary or multiple lesions on the scalp, forehead, or trunk. Relapses occur in approximately 10 percent of patients, but overall prognosis is excellent, with a 5-year survival of greater than 95 percent. Limited lesions may be treated surgically or with radiotherapy; anthracyclin-based chemotherapy is recommended in cases of extensive cutaneous involvement or extracutaneous disease.

Primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, is a more aggressive cutaneous lymphoma that occurs on the lower legs of elderly women. Histopathologic features include sheets of medium-to-large cells that are bcl-2+ and CD10-. While patients presenting with single, small lesions have an improved prognosis, the overall prognosis for patients with PCLBCL is poor, with a 5-year survival rate of 55 percent and frequent extracutaneous spread. These cutaneous lymphomas should be treated as systemic diffuse large B-cell lymphomas with anthracycline-based chemotherapy.

Primary cutaneous diffuse large B-cell lymphoma, other, includes morphologic variants of diffuse large B-cell lymphoma that are often observed as cutaneous manifestations of nodal lymphomas or in the setting of human immunodeficiency virus infection [3].

Intralesional or systemic treatment with rituximab, a chimeric monoclonal antibody that binds the B-cell-specific antigen CD20, has shown promise in the treatment of a number of primary cutaneous B-cell lymphomas, which include PCMZL, PCFCL, and PCLBCL of the leg [6]. A number of treatment regimens have been described that range from four to eight cycles of intravenous infusions with to two-to-four times weekly intralesional therapy. The treatment is generally well-tolerated and has resulted in prolonged remissions; however, recurrent cutaneous lymphomas with the loss of CD20 expression have been reported [7]. An association of Borrelia burgdorferi infection with PCMZL as well as other PCBCL types has been reported, particularly in endemic areas of Europe [1, 5]. Helicobater pylori infection, which has been associated with MALT lymphomas of the gastric mucosa and intestine, has been noted in a subset of patients with PCBCL. There have been isolated reports of complete remission or near-complete remission obtained in PCBCL patients seropositive for B burgdorferi and/or H pylori treated with antibiotic therapy [1]. Chronic antigenic stimulation by ultraviolet radiation in the context of actinic prurigo has also been reported as causal in the development of PCBCL in isolated reports [8].

References

1. Bogle MA, et al. Primary cutaneous B-cell lymphoma. J Am Acad Dermatol 2005;53:479

2. Zackheim HS, et al. Relative frequency of various forms of primary cutaneous lymphomas. J Am Acad Dermatol 2000;43:793

3. Slater DN. The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants. Br J Dermatol 2005;153:874

4. Willemze R, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768

5. Hoefnagel JJ, et al. Primary cutaneous marginzal zone B-cell lymphoma: clinical and therapeutic features in 50 cases. Arch Dermatol 2005;141:1139

6. Gellrich S, et al. Systemic eight-cycle anti-DC20 monoclonal antibody (rituximab) therapy in primary cutaneous B-cell lymphomas - an applicational observation. Br J Dermatol 2005;153:167

7. Rawal YB, et al. The absence of CD20 messenger RNA in recurrent cutaneous B-cell lymphoma following rituximab therapy. J Cutan Pathol 2005;32:616

8. Perrett CM, et al. Primary cutaneous B-cell lymphoma associated with actinic prurigo. Br J Dermatol 2005;153:186

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