Composite Cutaneous Lymphoma in a Patient With Rheumatoid Arthritis Treated With Methotrexate

Abstract

Patients with rheumatoid arthritis, whether treated or not with immunosuppressive agents including methotrexate, have an increased risk of lymphoproliferative disorders. Termed "iatrogenic immunodeficiency-associated lymphoproliferative disorders" in the 2008 World Health Organization classification of lymphoid neoplasms, they include Hodgkin and non-Hodgkin lymphomas. Composite lymphomas are rare, particularly in skin, with none reported in immunodeficiency states. We report the case of a 67 year-old woman with a long history of rheumatoid arthritis, on methotrexate treatment, who developed multiple skin lesions exhibiting a malignant infiltrate displaying both B- and T-cell phenotypes and dual clonal gene rearrangement by polymerase chain reaction, consistent with a cutaneous composite lymphoma. The patient received chemotherapy including rituximab with partial response, but the T-cell component recurred. To the best of our knowledge, this is the first case report of a cutaneous composite lymphoma in a patient with an iatrogenic immunodeficiency representing a dual challenge, diagnostic for the pathologist and therapeutic for the hematologist.

Primary cutaneous follicular helper T‐cell lymphoma: diagnostic pitfalls of this new lymphoma subtype

“…Likewise, three of five patients reported by Battistella et al were initially misdiagnosed as CFCL . Cases of cutaneous composite lymphoma should also be reviewed carefully for possible CT FH CL as clinical courses in some cases resemble ours and the other yet reported cases of CT FH CL . Importantly, as exemplified by our case, CT FH CL might lose PD‐1 while retaining CD10 expression, which might represent a clue to diagnosis in later stages.…”

Methotrexate-associated orbital lymphoproliferative disorder in a patient with rheumatoid arthritis: a case report

“…The histopathology also varies, with diffuse large B-cell lymphoma (DLBCL) accounting for 35-60 % of cases, Hodgkin lymphoma for 12-25 % of cases, follicular lymphoma for 5-10 % of cases, Burkitt lymphoma for 4 % of cases, and peripheral T-cell lymphoma for 4 % of cases [4]. As far as we are aware, no report of orbital MTX-LPD has been published to date.…”

“…About half of patients with primary MTX-LPD manifest lymph node lesions and about half have lesions outside the lymph nodes, such as in the gastrointestinal tract, skin, lungs, and soft tissues [4]. The histopathology also varies, with diffuse large B-cell lymphoma (DLBCL) accounting for 35-60 % of cases, Hodgkin lymphoma for 12-25 % of cases, follicular lymphoma for 5-10 % of cases, Burkitt lymphoma for 4 % of cases, and peripheral T-cell lymphoma for 4 % of cases [4].…”

Cutaneous Manifestations of Methotrexate-associated Lymphoproliferative Disorders: Report of Two Cases and a Review of the Literature

“…Histologically, 12 cases (63%) were B-cell lymphoma, of which 8 cases were DLBCL. In addition, there was 1 case of T-cell lymphoma (7), 1 of composite lymphoma (8), and 5 whose diagnosis was "LPD". Expression of EBV in tumour cells was confirmed in 13 of 18 cases (72%) by immunohistochemistry and/ or EBER in situ hybridization.…”