Multiple cutaneous monoclonal B‐cell proliferations as harbingers of systemic angioimmunoblastic T‐cell lymphoma

Abstract

We describe a 66-year-old man initially diagnosed with primary cutaneous marginal zone B-cell lymphoma who developed four additional monoclonal/monotypic B-cell lymphoid proliferations and a systemic angioimmunoblastic T-cell lymphoma over the course of 19 months. Through retrospective analysis, we identified the evolution of a T-cell clone within the background of clinically and pathologically dominant cutaneous B-cell tumors. In terms of clinical practice, this case supports that patients diagnosed with multiple clonal B-cell proliferation need thorough investigation and close clinical follow up to identify a coexistent or evolving systemic lymphoma, in particular, peripheral T-cell lymphomas of follicular T-helper cell type, such as angioimmunoblastic T-cell lymphoma. Biologically, this case offers unique insight into the interactions between B-cell and T-cell lineages in lymphoid neoplasia.

Extranodal Marginal Zone Lymphoma–like Presentations of Angioimmunoblastic T-Cell Lymphoma

“…The most typical and recurrent histologic skin findings seen in AITL are: 1) perivascular infiltrate (47% of cases), 2) vascular hyperplasia or proliferations (44% of cases), and 3) vasculitis (27% of cases) 10,38 . Clonal populations of T-cells are seen in 87% of cases and clonal B-cells can be found in up to 40–80% of cases (with only 5 cases tested from previous reports) 10,39 . Uncommon histologic presentations include granulomatous dermatitides 4,40 , an atypical linear IgA dermatosis form 40 , prurigo-like eruptions 41 , and rarely an epidermotropic form mimicking mycosis fungoides (MF) 42 .…”

“…Although not typical, EBV reactivity has been described in the setting of MALT, particularly in immunosuppressed individuals 44,45 . Bayer et al 39 described a case of an individual diagnosed with MALT lymphoma who went on to developcutaneous EBV positive diffuse large B-cell lymphoma followed by AITL. The authors suggested that development of multiple clonal populations of B-cells at cutaneous sites should warrant further evaluation for a T-cell lymphoma.…”

“…In fact, the development of DLBCL in the setting of AITL has been well-documented 27,28 , including the isolated case report at a cutaneous site 46 . It’s been shown the delay of the diagnosis of AITL from the development of cutaneous non-specific lesions can take up to 2 years 9,39 . La-chenal et al 9 has shown that at the time of diagnosis of AITL patients have adenopathy in 99% of cases and cutaneous lesions in nearly half of the individuals.…”

Primary Cutaneous, Composite, Epstein-Barr Virus-Associated, Diffuse Large B-cell Lymphoma and Peripheral T-cell Lymphoma

“…Cases falling under this category with features similar to ours have been described; however, development of DLBL occurred late in the course of the disease and not as an initial manifestation of the B-cell lymphoma as in our patient. 32 …”

Cutaneous B-Cell Lymphoproliferative Disorders

“…5,27 The workshop included 1 previously reported case in which a patient with an initial skin lesion considered to be a cutaneous MALT lymphoma was eventually found to have an angioimmunoblastic T-cell lymphoma. 28 Perhaps a less problematic cloud (in most circumstances at least) is the observation that individual patients can have both κ-and l-positive cutaneous MALT lymphomas. 19,20 Whether these populations with different light chains are clonally related is uncertain.…”