Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature

Baum, Christian L.; Link, Brian K.; Neppalli, Vishala T.; Swick, Brian L.; Liu, Vincent

doi:10.1016/j.jaad.2010.07.028

Cited by 67 publications

(73 citation statements)

References 14 publications

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“…10,12,15,18,19,22 Notably, secondary lymphoid follicles have not been described in association with this lymphoma. 17,21,22 A subset of large, atypical, pleomorphic cells may be present, but they make up less than 30% of the infiltrate by definition.…”

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

“…7,8 Although it affects individuals with a wide age range, including children, 10,12,15À18 most patients are older adults with an average age in the 50s to 60s and without a clear sex predilection. 3,4,7,10,12,[15][16][17][18][19][20][21] Patients typically present with a solitary, asymptomatic lesion on the face, neck, upper trunk, or upper extremities, which may appear as a violaceous papule, plaque, nodule, or tumor. * Some early series, before PC-SMTCL was a well-defined entity, reported a relatively high frequency (50%-74%) of multiple lesions in a localized or generalized distribution.…”

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

“…Frequently, the infiltrate extends into the subcutaneous fibroadipose tissue, and it may also involve skeletal muscle, vessels, and adnexal structures, sometimes with invasion and destruction of vessels and adnexae. 4,6,7,10,[15][16][17][18][19][20][21][22]26 Lymphoid cells are most often separated from the epidermis by a grenz zone, but focal epidermotropism may be seen. 3,4,7,8,19,20,22 Notably, prominent epidermotropism should prompt consideration of a diagnosis of MF.…”

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

“…8,17,20 CD8 þ T cells generally make up a substantial minority of cells (5%-47%). 10,15,17,18,[20][21][22][23] Scattered CD30 þ immunoblasts are usually identified. 10,12,15,18,21,22 Thus, immunophenotypic studies confirm the mixed hematolymphoid infiltrate seen morphologically in this lymphoma.…”

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

“…10,15,17,18,[20][21][22][23] Scattered CD30 þ immunoblasts are usually identified. 10,12,15,18,21,22 Thus, immunophenotypic studies confirm the mixed hematolymphoid infiltrate seen morphologically in this lymphoma. Although PC-SMTCL generally has a low Ki-67 proliferation index, usually ranging from 5% to 30%, 10,17,18,20,21,23,26 in some cases it may be as high as 50% to 70%.…”

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

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Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

Lan

Brown

Hristov

2014

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Primary cutaneous CD4+ small/medium T-cell lymphoma is a provisional and controversial entity with a broad differential diagnosis. Despite being an uncommon lymphoma, it is a frequent diagnostic consideration in cutaneous biopsies with a dense lymphoid infiltrate because it shows overlapping features with reactive lymphoid hyperplasia (pseudolymphoma) and a variety of other primary cutaneous and systemic lymphomas. However, proper classification of this process is important for determining patient prognosis and treatment options. Objective.—To review the clinical, morphologic, immunophenotypic, and genetic features of primary cutaneous CD4+ small/medium T-cell lymphoma and contrast those features with entities in the differential diagnosis. Data Sources.—Applicable literature will be reviewed with emphasis on current controversies and distinguishing characteristics. Conclusions.—Although many consider primary cutaneous CD4+ small/medium T-cell lymphoma to be indistinguishable from reactive lymphoid hyperplasia/pseudolymphoma, it can be differentiated from other primary cutaneous and systemic lymphomas. Patients with solitary lesions of primary cutaneous CD4+ small/medium T-cell lymphoma generally have an excellent prognosis. Nevertheless, a subset of patients who have been reported to meet criteria for this lymphoma have followed a more-aggressive course; however, those patients show some differing clinical, morphologic, and immunophenotypic features.

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Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

Section: Clinical Morphologic and Immunophenotypic Features Primarymentioning

confidence: 99%

See 3 more Smart Citations

Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

Lan

Brown

Hristov

2014

Archives of Pathology &Amp; Laboratory Medicine

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Intertriginous Mycosis Fungoides

Gammon¹,

Guitart²

2012

Arch Dermatol

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Background: Follicular helper T cells are a subset of helper T cells that facilitate B-cell recruitment and maturation. Rare cases of cutaneous T-cell lymphoma manifesting as de novo tumor lesions in intertriginous skin contain an infiltrate rich in B cells. These cases may represent malignant counterparts of skin-homing follicular helper T cells. Observations: Two men and 1 woman (age range, 35-58 years) were seen with predominantly intertriginous tumorstage cutaneous T-cell lymphoma lesions characterized by the absence of epidermotropism and the presence of a mixed infiltrate rich in B cells. Two of the patients died of the disease less than 3 years from the initial diagnosis. The surviving patient has aggressive disease and underwent hematopoietic stem cell transplantation. Two of the patients had a prominent CXCL13 ϩ , Bcl6/CD3 ϩ , and programmed death protein 1-positive follicular helper T-cell population. Conclusions: The intertriginous tumor variant of cutaneous T-cell lymphoma is heterogeneous but may be associated in some cases with a follicular helper T-cell immunophenotype. These patients may follow an aggressive clinical course. Tumor progression in sanctuary sites on patients receiving phototherapy may manifest as a similar clinical phenotype. Further characterization of the disease process is needed to confirm this observation.

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Cutaneous Lymphomas in Children and Adolescents

2014

Skin Lymphoma

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Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature

Cited by 67 publications

References 14 publications

Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

Intertriginous Mycosis Fungoides

Cutaneous Lymphomas in Children and Adolescents

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