Primary cutaneous B-cell lymphoma: A unique type of low-grade lymphoma. Clinicopathologic and immunologic study of 83 cases

Abstract

The clinical presentation and course, and the morphoimmunologic features of primary cutaneous B-cell lymphoma (CBCL) were investigated in a series of 83 patients. Fifty-one patients were male and 32 were female (male-to-female ratio of 1,6:1]; CBCL primarily involved the elderly (median age, 58 years). A locoregional extension of the disease was quite frequent (86.7%). The neoplastic cells showed a range of appearances reminiscent of the whole spectrum of follicular/ parafollicular cells. The antigenic phenotype of tumor cells (CD19+, CD20+, CD22+, CD28+, CD10-, CD5-, MB2+, CD74+/-, CDw75+/-, MT2+/-, surface immunoglobulin+ monoclonal/-) plus the presence of admixed CD14-dendritic reticulum cells suggest a mantle-zone nature for CBCL. The nonaggressive clinical behavior with a substantial tendency to remain localized to a limited area of the skin, the quite good response to nonaggressive treatment, and the dichotomy existing between the enhancement of morphoimmunologic atypism-which parallels the increasing age and growth rate of lesions-and the constant benign overall prognosis on long-term follow-up make CBCL a unique type of lymphoma of low-grade malignancy. Proper recognition of CBCL is mandatory to avoid possible undertreatment or overtreatment of the patients affected. Cancer 672311-2326,1991. HE CUTANEOUS INVOLVEMENT of a B-cell lymphoma

CLIPI: a new prognostic index for indolent cutaneous B cell lymphoma proposed by the International Extranodal Lymphoma Study Group (IELSG 11)

“…However, these lymphomas have a relapse rate between 20% and 60% according to different studies [5][6][7][8][9][10][11]. Nevertheless, since relapses are mainly confined to the skin and they do not constitute a dissemination of disease, the standard of care in patients without extensive cutaneous disease or evidences of Borrelia burgdorferi infection, consists of local treatments, namely surgical excision (SX) or radiotherapy (RT) [4].…”

Primary cutaneous B-cell lymphoma: A clinically homogeneous entity?

“…The only reliable tool is the demonstration of B-cell monoclonality by immunohistochemistry and/or molecular analysis by Southern blotting and the polymerase chain reaction. 18,[22][23][24][25][26][27][28] The conclusion that most primary and rare/undefined types. 33 Primary cutaneous FCC lymphoma and IC, which affect more than 90% of all patients with a definite diagnosis of primary CBCL, share a good response to radiotherapy and a generally excellent prognosis that is much better than morphologically similar lymphomas in lymph nodes.…”

BCA-1, A B-cell chemoattractant signal, is constantly expressed in cutaneous lymphoproliferative B-cell disorders

“…In low grade CBCL, conversely, we usually found CD35+ follicular dendritic cells, with a stronger staining in cryostat sections, while the expression of CD21 is AE in cryostat sections and often lacking in paraffin sections. Indeed, we have previously stressed this ''aberrancy'' of dendritic cells in neoplastic follicle-like clusters of classic CBCL [15][16][17][18]29,30]. Therefore, it is not unlikely that immunologically aberrant follicular dendritic cells have a substantially downregulated expression of BCA-1 in both low grade CBCL and low grade gastric lymphoma of the MALT type.…”

“…The clinico-pathological features of primary cutaneous B-cell lymphomas (CBCL) are extensively dealt with elsewhere in Refs. [15][16][17][18]. In short, CBCL are mostly characterised by a homogeneous clinical presentation and behaviour, with a good response to local radiotherapy, low tendency to extracutaneous spread and excellent prognosis.…”

“…An alternative possibility of a unitary interpretation of low grade CBCL has been suggested. In fact, we have documented that the histological pattern in CBCL is largely related to the age and growth rates of the skin lesions [15][16][17][18], with a characteristic multiphasic progression of the histological/immunohistological pattern from ''early'' (patchy/nodular infiltrate, with distinct compartmentalisation of neoplastic, small-medium centrocyte-like B-cells and reactive T-cells, that are often prominent) to ''late'' phases (diffuse infiltrate of neoplastic, large transformed centrocyte-like B-cells, with few admixed reactive T-cells). These multiphasic histological features have no correlation with either the clinical course or the prognosis of the disease.…”