Abstract

Composite lymphomas have been defined as 2 distinct subtypes of lymphoma occurring at a single anatomic site. Composite lymphomas limited to the skin are a rare occurrence and pose a unique challenge. Many reported cases within the skin are combined B-cell and T-cell lymphomas, typically mycosis fungoides and a low-grade B-cell lymphoma. These cases are challenging to recognize because lymphoid infiltrates within the skin often include a mixed population of B cells and T cells. In particular, reactive lymphoid proliferations (pseudolymphomas), primary cutaneous low-grade B-cell lymphomas, and primary cutaneous CD4+ T-cell lymphoproliferative disorder may show nearly equal numbers of B cells and T cells. In order to exclude these possibilities, overwhelming evidence in support of each lymphoma is helpful, including abnormal architecture, cytology, and immunophenotype, as well as molecular genetic evidence of clonality.

Highlights

  • Man age 70 y with a 10-y history of papular mycosis fungoides (MF) developed a subcutaneous nodule on his right arm that showed a primary cutaneous Composite Lymphomas (CLs)

  • Woman age 67 y with rheumatoid arthritis (RA) treated for 6 y with MTX and variably with prednisone had a 2-y history of plaques

  • Man age 73 y with a 2-y history of patch/plaque MF developed plaquelike to nodular lesions on his scalp

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Summary

Primary Cutaneous Composite Lymphomas

Composite lymphomas have been defined as 2 distinct subtypes of lymphoma occurring at a single anatomic site. Many reported cases within the skin are combined B-cell and T-cell lymphomas, typically mycosis fungoides and a low-grade B-cell lymphoma. These cases are challenging to recognize because lymphoid infiltrates within the skin often include a mixed population of B cells and T cells. 1352 Arch Pathol Lab Med—Vol 142, November 2018 same neoplastic clone (eg, follicular lymphoma transforming to diffuse large B-cell lymphoma).[5–9]. Some authors excluded these latter cases from the term CL, emphasizing the coexistence of 2 ‘‘separate and distinct lymphomas’’ with different cells of origin.[9–11]. 1352 Arch Pathol Lab Med—Vol 142, November 2018 same neoplastic clone (eg, follicular lymphoma transforming to diffuse large B-cell lymphoma).[5–9] Some authors excluded these latter cases from the term CL, emphasizing the coexistence of 2 ‘‘separate and distinct lymphomas’’ with different cells of origin.[9–11] This later definition has gained more support with time

PRIMARY CUTANEOUS CL
DIFFERENTIAL DIAGNOSIS
Additional Features
Papular MF and PCMZL
Indolent course
CLINICAL IMPLICATIONS
Findings
CONCLUSIONS
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