eMedicine Specialties > Dermatology > Lymphoma and Related Processes
Cutaneous B-Cell Lymphoma
Updated: Jan 15, 2010
Introduction
Background
Cutaneous lymphomas represent a unique group of lymphomas and are the second most frequent extranodal lymphomas.1 They can be defined as lymphoproliferative skin infiltrates of T-cell, B-cell, or natural killer cell lineage, which primarily occur in and remain confined to the skin in most patients, without detectable extracutaneous manifestations at diagnosis.
B-cell lymphomas account for the majority of nodal lymphomas, whereas in the skin, primary cutaneous B-cell lymphomas (CBCLs) represent 20-25% of all cutaneous lymphomas. Because cutaneous B-cell lymphomas have an overall favorable prognosis, proper recognition is vital for appropriate therapy and to avoid overtreatment in most cases. The tumor type and the extent of cutaneous involvement are the 2 most relevant prognostic factors in primary cutaneous B-cell lymphoma.2
Pathophysiology
The etiology and the exact steps in the pathogenesis of cutaneous lymphomas are only partially understood. Most probably, lymphomagenesis of cutaneous lymphoma represents a multifactorial and multistep process, owing to the impact of various etiologic factors occurring over a long period. The disease likely begins as a hyperreactive inflammatory process. Deficits in cell proliferation regulation and defective oncogene and/or suppressor gene expression later promote transition from preneoplastic conditions to neoplasia. The most important factors initiating cutaneous B-cell lymphomas are immunodeficiency disorders, infections with oncogenic viruses (eg, Epstein-Barr virus [EBV], human herpesvirus type 8/Kaposi sarcoma–associated herpesvirus, hepatitis C virus), and bacteria (eg, Helicobacter pylori in mucosa-associated lymphoid tissue [MALT] lymphomas, Borrelia burgdorferi in cutaneous B-cell lymphomas).3
Demographic features
The frequency of cutaneous lymphomas is 0.3 case per 100,000 population per year, with 10% (in the United States) to 20% (in Europe) being cutaneous B-cell lymphomas, marginal zone lymphomas, or follicle center lymphoma (FCL) in most cases
The 5-year overall survival rate for most cases of cutaneous B-cell lymphoma is greater than 90%, except in diffuse large B-cell lymphoma (DLBCL), for which the 5-year survival rate is 20-50%.
No significant statistical data are available for cutaneous B-cell lymphoma with regard to any predisposition based on race. However, with regard to sex and age, DLBCL is predominantly seen in elderly women.
Classification
Diseases formerly designated as reticulosis or reticulosarcomatosis today are classified according to the World Health Organization (WHO)/European Organization for Research and Treatment of Cancer (EORTC) classification (WHO-EORTC classification) for primary cutaneous lymphomas and the WHO classification.4,5 6,7,8 Cutaneous T-cell lymphomas (CTCLs) can be stratified in prognostically relevant clinical stages, but no generally accepted staging classification exists for primary cutaneous B-cell lymphoma. In 2007, a new TNM staging system for non–mycosis fungoides/Sézary syndrome has been developed.9 The value of this scheme has been proven by clinicopathological studies.10,11
WHO/EORTC classification of cutaneous B-cell lymphomas4,5,6,7 is as follows:
Primary cutaneous marginal zone B-cell lymphoma (MALT-type)
Variant
- Immunocytoma
- Primary cutaneous plasmacytoma
Variants (according to growth pattern)
- Follicular
- Follicular and diffuse
- Diffuse
Variants
- Leg type
- Others
Lymphomatoid granulomatosis*
Chronic lymphocytic leukemia*
Mantle cell lymphoma*
Burkitt lymphoma*
*Not primarily in the skin; only secondary skin involvement.
Nosologic Entities of Cutaneous B-Cell Lymphoma
Primary cutaneous marginal zone B-cell lymphoma (MALT-type)
Marginal zone B-cell lymphoma (MZL) (MALT-type) is an indolent cutaneous lymphoma, accounting for approximately 10% of all cutaneous lymphomas. The prognosis is excellent, with a 5-year survival rate of greater than 95%. See the images below:
Solitary nodule in marginal zone lymphoma.
Monocytoid B cells and plasmacytoid cells in marginal zone lymphoma.
Marginal zone lymphoma, CD20.
Intranuclear periodic acid-Schiff–positive Dutcher bodies in immunocytoma (marginal zone lymphoma).
Synonyms based on classification are as follows:
- WHO classification (2008) - Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
- WHO/EORTC classification (2005) - Primary cutaneous MZL
- WHO classification (2001) - Extranodal marginal zone lymphoma of MALT type
- Revised European American Lymphoma (REAL) classification (1997) - Extranodal MZL
- EORTC classification (1997) - Primary cutaneous MZL
Extranodal MZL may develop from reactive infiltrates that represent immune responses to external factors or autoantigens.15 An etiopathological relationship to B burgdorferi has been demonstrated in some cases.16,17
Clinically, MZL manifests as solitary or multiple reddish, dome-shaped papules, nodules, or erythematous plaques, frequently located on the trunk and extremities and, to a lesser extent, on the head and neck area.18,19
Histologic findings include a nodular or diffuse nonepidermotropic infiltrate composed of small to medium-sized lymphoid cells with indented nuclei and abundant, pale cytoplasm (marginal zone cells, monocytoid B-cells) or lymphoplasmacytoid cells.20 Darker chromatin-rich cells are surrounded by pale-staining cells, resulting in a characteristic inverse pattern. Remnants of reactive germinal centers are present, colonized by tumor cells and reactive T cells.21
The tumor cells express the following immunophenotype: CD19+, CD20+, CD22+, CD43+, CD79a+, CD5-, CD10-, CD23-, bcl-6-, bcl-2+, KiM1p+ (monocytoid B-cell–related antibody). Plasma cells, often located at the periphery of the infiltrates, show monotypic expression of immunoglobulin light chain kappa more frequently than lambda. Plasmacytoid cells may be present in confluent aggregates. A variable number of reactive CD3+ T cells are present. Clusters of CD123-positive plasmacytoid dendritic cells are present in MZL, but found to a lesser extent or absent in other cutaneous B-cell lymphoma forms.22
Molecular analysis has shown that immunoglobulin H (IgH) genes are clonally rearranged in most cases.23 Translocation of t(11;18), as is seen in the nodal counterpart, is usually not found in primary cutaneous MZL.
Primary cutaneous MZL with high numbers of monotypic plasma cells and lymphoplasmacytoid cells showing intranuclear periodic acid-Schiff–positive globular inclusions (Dutcher bodies) was previously referred to as cutaneous immunocytoma.16,24
Primary cutaneous FCL
Primary cutaneous FCL is an indolent lymphoma of follicle center cells (predominantly centrocytes with an admixture of centroblasts). The prevalence rate is approximately 12%, and the 5-year survival rate is greater than 90-95%, indicating the excellent prognosis in comparison to its nodal counterpart. See the images below:
Follicle center lymphoma. Multiple skin tumors.
Small and large follicle center cells.
Follicle center cell lymphoma. Irregular pattern of CD21-positive dendritic cells.
Synonyms based on classification are as follows:
- WHO classification (2008) - Primary cutaneous FCL
- WHO/EORTC classification (2005) - Primary cutaneous FCL
- WHO classification (2001) - Cutaneous FCL
- REAL classification (1997) - FCL, follicular
- EORTC classification (1997) - Primary cutaneous follicle center cell lymphoma
Clinically, nodules and tumors are found most frequently in the head and neck area, but they are also found in other locations of the body.26,10 A firm consistency without ulceration is very typical. Primary cutaneous FCL has an excellent prognosis, with a 5-year survival rate of greater than 90%.10,27 PFCL recurs in up to 40% of patients. Extracutaneous spread occurs very rarely. PCFCL arising at the legs and those cases with expression of FOX-P1 appear to have a worse prognosis and should be treated more aggressively, similar to DLBCL, leg type.11
Histologically, 3 growth patterns can be differentiated: follicular, follicular and diffuse, and diffuse. The diffuse pattern is, in fact, the most common seen in primary cutaneous FCL. The infiltrates are composed mainly of centrocytelike cells with intermingled centroblasts and immunoblasts. A subepidermal grenz zone is present in most cases. Mitoses, tingible body macrophages, and starry-sky features, typically found in reactive lymph follicles,28 are rare or absent in FCL.
Immunophenotypically, the cells in FCL express CD19+, CD20+, CD22+, CD43+, CD79a+, CD5-, CD23+/-, CD43, bcl-6+, and bcl-2-. CD10+ is expressed in cases with a follicular growth pattern. Follicular dendritic cells (CD21+) are arranged in an irregular network. The MUM/IRF4 antigen, which is positive in DLBCL, is not expressed in FCL and may be helpful in differentiating FCL with a diffuse growth pattern from DLBCL.
Molecular analysis shows clonal rearrangement of immunoglobulin genes. In contrast to secondary skin involvement in FCL, chromosomal translocation of t(14;18) and BCL2 gene rearrangement are absent in most cases of primary cutaneous FCL.29,30
Studies on gene expression profiles have shown distinct patterns in various types of B-cell lymphoma of the skin.31,32
Primary cutaneous diffuse large B-cell lymphoma
Primary cutaneous DLBCL is an aggressive cutaneous B-cell lymphoma, accounting for approximately 6% of all cutaneous lymphomas. It is associated with a relatively poor prognosis compared with other primary cutaneous B-cell lymphomas, with a 5-year survival rate of 20-55%, and tends to spread to lymph nodes and extracutaneous sites.33,34
Two groups have been differentiated. The first is the leg type and the second is DLBCL, other. The leg type usually occurs on the lower legs of elderly women. The term is similar to terms used in the classification of other extranodal lymphomas (eg, nasal type). It manifests with a distinct phenotype and can also be present in other locations of the body (with a similar bad prognosis). DLBCL, others, includes T-cell/histiocyte-rich DLBCL, plasmablastic lymphoma, and other types that do not fulfill the criteria for a DLBCL, leg type. See the images below:
Tumorous lesions on the legs in a patient with diffuse large B-cell lymphoma.
Large lymphoid cells of diffuse large B-cell lymphoma, sparing a subepidermal grenz zone.
Neoplastic cells in diffuse large B-cell lymphoma with strong positivity for bcl-2.
Synonyms based on classification are as follows:
- WHO classification (2008) - Primary cutaneous DLBCL, leg type
- WHO/EORTC classification (2005) - Primary cutaneous DLBCL
- DLBCL, leg type
- DLBCL, other
- WHO classification (2001) - DLBCL
- REAL classification (1997) - DLBCL
- EORTC classification (1997) - Primary cutaneous large B-cell lymphoma of the leg
The histological features are characterized by a diffuse infiltrate sparing a thin subepidermal grenz zone in most cases, covering the entire dermis, destroying adnexal structures, and extending into the subcutaneous tissue. The tumor cells are large B cells, formally referred to as centroblasts and immunoblasts.35 Centrocytes, which are typically seen in FCL, are minimal or absent in DLBCL.
The immunophenotype of the neoplastic cells is CD19+, CD20+, CD22+, CD5-, CD10-, CD79a+, bcl-2++, bcl-6-/+, MUM1+, CD138-, cyclin D1-. The strong positivity for Bcl-2 protein and MUM-1/IRF-436 allows differentiation of FCL with a diffuse growth pattern from DLBCL. Networks of CD21-positive follicular dendritic cells are not found in DLBCL.
Molecular analysis shows clonal rearrangement of immunoglobulin genes. In primary cutaneous DLBCL, t(14; 18) and the bcl-2/JH translocation cannot be detected.37 Gene expression profiles of DLBCL and FCL with a diffuse growth pattern are different.31,32,38
DLBCL, other
The DLBCL, other subcategory includes large B-cell lymphomas with distinct growth patterns, such as T-cell rich/histiocytic DLBCL39 and intravascular large B-cell lymphoma.40,41
B-cell lymphomas with common secondary cutaneous involvement
Skin involvement in mantle cell lymphoma is rare and usually secondary.42 Cyclin D1 is a useful marker for the small tumor cells derived from mantle cells, as in mantle cell lymphoma.
Lymphomatoid granulomatosis is a rare multisystemic, angiocentric, and angiodestructive B-cell lymphoproliferative disease that involves extranodal sites, especially the lungs, skin, and nervous system. It is associated with EBV infection and may progress to DLBCL.
Burkitt lymphoma occurs endemically in children in the so-called lymphoma belt of Central Africa and is associated with EBV infection in most cases. Sporadic EBV-negative cases may be seen in association with HIV-induced immunodeficiency.43
Waldenström macroglobulinemia is characterized by a clonal expansion of lymphocytes with plasmacytoid features that produce a monoclonal immunoglobulin M protein and infiltrate bone marrow, lymph nodes, and the spleen. Cutaneous involvement usually is nonspecific, showing urticarial and purpuric eruptions, ulcers, bullous lesions, and vasculitis.
Multiple myeloma with monoclonal gammopathy typically induces hyperkeratotic spicules, preferentially on the face.44
Differential Diagnoses of Cutaneous B-Cell Lymphoma
Differentiation of cutaneous B-cell lymphoma from CTCL
The history in CTCL usually is long with a prediagnostic-preneoplastic phase over several years, during which differentiation from dermatitis may be challenging or even impossible. In contrast, cutaneous B-cell lymphoma lesions evolve quickly, usually within a few weeks.
Most CTCLs show typical clinical features. The prototype, mycosis fungoides, starts with eczematous patches, slowly developing into plaques over years or sometimes over decades, and finally—in a subset of patients—evolving into exophytic tumors with ulceration.
The most significant discriminating histological feature between T- and B-cell lymphomas of the skin is the growth pattern,5,45,46 which is horizontal, disk-like, and epidermotropic in CTCL and is ball-like, spherical, and nonepidermotropic in cutaneous B-cell lymphoma. Moreover, infiltrating cells in CTCL tend to be small and irregularly shaped with convoluted and indented nuclei. Cells in cutaneous B-cell lymphoma usually are oval or roundish, reflecting the shapes of large follicular center cells, immunoblasts, or plasma cells.
The immunophenotypical features are clearly different and correspond to the T- or B-cell lineage from which they originate. Genotyping reveals clonal rearrangement of immunoglobulin genes and of T-cell receptor genes, respectively.
Differentiation of cutaneous B-cell lymphoma and B-cell pseudolymphoma
The most important differential diagnosis of cutaneous B-cell lymphoma is from B-cell pseudolymphoma (PSL) (lymphoid hyperplasia) of the skin.24,47,48,49
Clinically, PSL is usually located as a single nodule on the face, neck, mammillae, or scrotum following B burgdorferi infection through a tick bite, tattooing, or other mechanical or infectious irritants. The consistency is soft, in contrast to the nodules of cutaneous B-cell lymphoma, which are firm or even hard.
Histologically, the infiltrate in cutaneous B-cell lymphoma is usually dome shaped with a convex border of infiltrative nodules. In PSL, it is wedge shaped, showing concave borders of the infiltrate. The latter may show regular germinal center formation, as is seen in reactive lymph nodes, with many starry-sky macrophages carrying ingested nuclear dust. Eosinophils, polyclonal plasma cells, and many T cells are present in the periphery and within the follicular area.
Immunophenotypically, the expression presence of both kappa- and lambda-positive cells in the infiltrate argues in favor of PSL. The networks of CD21+ dendritic cells are regular, round, or oval in PSL but are irregular, ringlike, or bizarre in cutaneous B-cell lymphoma, if present at all.
Genotyping reveals clonal rearrangement of immunoglobulin genes in most cases of cutaneous B-cell lymphoma and lacks clonality in PSL.
Differentiation of various types of cutaneous B-cell lymphoma
In contrast to primary CTCLs, primary cutaneous B-cell lymphomas appear clinically quite similar. However, a few criteria are seen more frequently in one or the other type. Nodules and tumors of MZL and of FCL demonstrate a hard consistency and are seen more frequently in the head and neck area (FCL) and on the trunk or arms (MZL), whereas single or grouped tumors on the lower legs typically appear in DLBCL, leg type, in elderly patients.10,11
The histological growth pattern is nodular with convex borders. The infiltrating cells correspond in morphology to their normal counterparts, which are small lymphocytes and large follicle center cells, immunoblasts, and smaller mantle cells or marginal zone monocytoid or plasmacytoid B cells.
The immunophenotypical pattern is most important in the differentiation of various entities of cutaneous B-cell lymphoma. The typical differential features are given in the Table.
Differential Phenotyping of Cutaneous B-Cell Lymphoma
Open table in new window
Table
| Bcl-2 | Bcl-6 | CD10 | t(14;18) | MUM1/IRF4 | |
| MZL/Immunocytoma | + | - | - | - | - |
| FCL | - | + | +/- | - | - |
| Secondary FCL | + | + | + | + | - |
| DLBCL | ++ | + | - | -/+ | + |
| PSL | - | + | + | - | - |
| Bcl-2 | Bcl-6 | CD10 | t(14;18) | MUM1/IRF4 | |
| MZL/Immunocytoma | + | - | - | - | - |
| FCL | - | + | +/- | - | - |
| Secondary FCL | + | + | + | + | - |
| DLBCL | ++ | + | - | -/+ | + |
| PSL | - | + | + | - | - |
Gene rearrangement studies are helpful adjunctive diagnostic markers in the differentiation of cutaneous B-cell lymphoma from CTCL and B-cell PSL, but they do not allow discrimination of cutaneous B-cell lymphoma subtypes.
Gene expression profiles may be helpful for discriminating DLBCL from FCL and other subtypes of cutaneous B-cell lymphoma.31,32,38
Workup in Cutaneous B-Cell Lymphoma
Mycosis fungoides, the prototype of CTCL, usually evolves from erythematous patches, to plaques, and then to tumoral lesions over a period of years and decades, whereas cutaneous B-cell lymphoma has a relatively brief history of nodules and tumors growing within a few weeks or months, preferentially in the head and neck area or trunk; these nodules and tumors may be surrounded by monomorphous papules or dermal plaques.
Histomorphology and cytomorphology
Cutaneous B-cell lymphoma exhibits a typical growth pattern, referred to as a B-cell pattern.45 It is characterized by a (often well-demarcated) nodular infiltrate of densely packed lymphoid cells in the dermis with convex margins, without significant interstitial infiltration and without epidermotropism. The subepidermal grenz zone is free of lymphoid cells. Whereas inflammatory infiltrates usually comprise a mixture of various cell types, lymphoproliferative disorders, especially FCL (diffuse type) and DLBCL, mostly show a predominance of one cell type.
Cytomorphologically, the infiltrating cells in cutaneous B-cell lymphoma correspond to small lymphocytes or to the cellular components of the lymph follicle (ie, centrocytes, centroblasts, mantle cells, monocytoid B cells of the marginal zone, plasma cells, or immunoblasts).
Immunohistochemistry and phenotypic features
Immunohistochemical identification of the tumor cell phenotype plays a crucial role in the diagnostic workup, especially of cutaneous B-cell lymphomas. The most important antibodies for cutaneous B-cell lymphoma are CD5 (expressed in B-cell chronic lymphatic leukemia), CD20, CD79a, CD21 (marker for follicular dendritic cells), CD10, Mum-1/IRF4, bcl-2, bcl-6, and kappa- and lambda light chains. Classic T-cell markers (ie, CD2, CD3, CD4, CD8, CD7, CD43 [also stains blastic B cells and a variety of histiocytic cells], CD30) should be negative. However, be aware of aberrant expression of T-cell markers (ie, CD43, CD5) or of CD30 in cutaneous B-cell lymphoma.
Translocation of the BCL2 gene (t14;18),29 although regularly seen in nodal follicular lymphomas, is usually negative in cutaneous B-cell lymphoma and thus cannot be regarded as a helpful diagnostic tool in these cases.
Genotyping and cytogenetic studies in cutaneous B-cell lymphomas
The tumor cells show clonal rearrangement of immunoglobulin genes in 60-70% of the cases in cutaneous B-cell lymphoma. Cases of cutaneous lymphoid hyperplasia with monotypic plasma cells have been reported, which have been described to be a biologically distinct clinicopathological entity.49
The genetic defects in cutaneous lymphoma are heterogeneous.5 Thus, to date, the detection of chromosomal abnormalities has limited diagnostic or prognostic value in CTCL and cutaneous B-cell lymphoma. Nodal follicular lymphoma is determined by the presence of a unique translocation between chromosomes 14 and 18, t(14;18)(q32;q21), BCL2-JH gene rearrangement that is not present in primary cutaneous FCL. Genetic alterations have been detected in intravascular lymphoma, possibly delineating the critical mutations associated with the initiation and progression of this disease, but without any diagnostic relevance.50
Cytogenetic analysis using fluorescence in situ hybridization and other techniques has shown that recurrent chromosomal abnormalities known in systemic MZL of the MALT type and in nodal FCL rarely occur in primary cutaneous B-cell lymphoma of these types.51 A reciprocal translocation between the long arms of chromosomes 12 and 21, t(12;21)(q13;q22), has been found in a patient with primary cutaneous FCL,52 which again is of some interest in view of the potential pathogenetic pathway of these tumors, but so far has no practical diagnostic relevance.
The modern standard procedure to elucidate and define the normal and pathologic state of a cell or tissue type is the analysis of tissue-specific gene expression and functional profiling by microarray technology.31,32
Blood tests, laboratory tests, and other investigations
A routine blood cell count is performed in order to exclude leukemic spread to tumor cells, which is unlikely if palpable enlargement of the lymph nodes is absent. In primary cutaneous B-cell lymphoma, abnormalities in routine laboratory investigations are not expected.
The likelihood of significant node involvement in patients with nonpalpable nodes is low; therefore, blind lymph node biopsy is not indicated. However, a lymph node biopsy should be performed if lymph nodes are enlarged.
Additional investigations include chest radiography, ultrasonography, and CT scanning of the abdomen and peripheral lymph nodes. CT scanning of the abdomen and peripheral lymph nodes is useful in patients with advanced skin disease and palpable lymphadenopathy for an accurate baseline assessment and to document disease progression. A bone marrow biopsy should be considered in persons with FCL/DLBCL, in order to confirm the primary cutaneous origin and to exclude extracutaneous origin of the lymphoproliferative disorder. It is, however, optional in patients with MZL.53
Treatment
Overtreatment in FCL and MZL must be avoided because cutaneous B-cell lymphomas have a much better prognosis than their nodal counterparts. Aggressive systemic treatment regimens are suitable only for DLBCL and for patients with extracutaneous spread. Treatment follows standard regimens for primary nodal lymphomas in these patients, as in B-cell lymphoma.
The treatment of primary cutaneous lymphomas associated with an excellent prognosis (ie, MZL [including immunocytoma] and FCL)54,55,56,57,58 is as follows:
- First-line treatments for solitary lesions
- Surgical excision
- Antibiotics - Doxycycline at 100 mg twice daily for 3 weeks59 or pulse therapy with cefotaxime55,60 in Borrelia -associated cases
- Radiotherapy - 20-100 kV Orthovolt therapy 1-4 times per week, to a total dose of up to 15-20 Gy61 ; extended-field irradiation at 30 Gy (4 fractions of 2.5 Gy/wk); localized-field irradiation as either a boost after extended-field irradiation or as definitive treatment; energies of 45 kV x-ray filtered by 0.55 aluminium, for small depth lesions; higher energies (≤100 kV) for deeper lesions; radiation dose varies from 30-40 Gy (fractions of 2 Gy/wk)14,62,63,64
- Second-line treatments for solitary lesions
- Intralesional glucocorticosteroids
- Intralesional rituximab - Injections 2-3 times weekly; single doses of 1-3 mL of stock solution (10 mg mL-1)65 or 3 times a week with 3 mL of stem solution (at 10 mg/mL); therapy repeated 6 times every 26 days66
- Intralesional interferon alfa - 3 and 9 million U subcutaneously (weekly basis) or 9 million U 3 times a week60,67
- First-line treatments for multiple lesions
- Antibiotics - Doxycycline at 100 mg twice daily for 3 weeks59 or pulse therapy with cefotaxime55,60 in Borrelia -associated cases
- Radiotherapy - 20-100 kV Orthovolt therapy 1-4 times per week, to a total dose of up to 15-20 Gy61 ; extended-field irradiation at 30 Gy (4 fractions of 2.5 Gy/wk); localized-field irradiation as either a boost after extended-field irradiation or as definitive treatment; energies of 45 kV x-ray filtered by 0.55 aluminium, for small depth lesions; higher energies (≤100 kV) for deeper lesions; radiation dose varies from 30-40 Gy (fractions of 2 Gy/wk)14,62,63,64
- Second-line treatments for multiple lesions
- Intralesional interferon alfa - 3 and 9 million U subcutaneously (weekly basis) or 9 million U 3 times a week67,60
- Intralesional rituximab - Injections 2-3 times weekly; single doses of 1-3 mL of stock solution (10 mg mL-1)65 or 3 times a week with 3 mL of stem solution (at 10 mg/mL); therapy repeated 6 times every 26 days66
- Intravenous rituximab - 375 mg/m2 weekly for 4 weeks68,69
- First-line treatment for isolated or grouped lesions
- Radiotherapy - 20-100 kV Orthovolt therapy 1-4 times per week, to a total dose of up to 15-20 Gy61 ; extended-field irradiation at 30 Gy (4 fractions of 2.5 Gy/wk); localized-field irradiation as either a boost after extended-field irradiation or as definitive treatment; energies of 45 kV x-ray filtered by 0.55 aluminium, for small depth lesions; higher energies (≤100 kV) for deeper lesions; radiation dose varies from 30-40 Gy (fractions of 2 Gy/wk)14,62,63,64
- Surgical excisions
- First-line therapy for multiple lesions
- Second-line therapy for multiple lesions - Polychemotherapy (as above) plus rituximab
Follow Up
Patients should be seen in an outpatient setting at least every 6 months for a clinical workup; they do not require any additional laboratory workup unless enlargement of the regional lymph nodes has occurred.
Multimedia
 | Media file 1: Solitary nodule in marginal zone lymphoma. |
 | Media file 2: Monocytoid B cells and plasmacytoid cells in marginal zone lymphoma. |
 | Media file 3: Marginal zone lymphoma, CD20. |
 | Media file 4: Intranuclear periodic acid-Schiff–positive Dutcher bodies in immunocytoma (marginal zone lymphoma). |
 | Media file 5: Follicle center lymphoma. Multiple skin tumors. |
 | Media file 6: Small and large follicle center cells. |
 | Media file 7: Follicle center cell lymphoma. Irregular pattern of CD21-positive dendritic cells. |
 | Media file 8: Tumorous lesions on the legs in a patient with diffuse large B-cell lymphoma. |
 | Media file 9: Large lymphoid cells of diffuse large B-cell lymphoma, sparing a subepidermal grenz zone. |
 | Media file 10: Neoplastic cells in diffuse large B-cell lymphoma with strong positivity for bcl-2. |
Keywords
cutaneous B-cell lymphoma, B-cell lymphoma, reticulosis of the skin, skin reticulosis, monomorphous reticulosis, lymphocytoma, reticulohistiocytosis of the back, Crosti disease, Crosti’s disease, reticulosarcoma, reticulosarcomatosis, angioendotheliomatosis, cutaneous marginal zone B-cell lymphoma, MZL, primary cutaneous follicle center lymphoma, FCL, cutaneous diffuse large B-cell lymphoma, DLBCL, mantle cell lymphoma, CBCL
More on Cutaneous B-Cell Lymphoma |
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Further Reading
Keywords
cutaneous B-cell lymphoma, B-cell lymphoma, reticulosis of the skin, skin reticulosis, monomorphous reticulosis, lymphocytoma, reticulohistiocytosis of the back, Crosti disease, Crosti’s disease, reticulosarcoma, reticulosarcomatosis, angioendotheliomatosis, cutaneous marginal zone B-cell lymphoma, MZL, primary cutaneous follicle center lymphoma, FCL, cutaneous diffuse large B-cell lymphoma, DLBCL, mantle cell lymphoma, CBCL
