Association of systemic mastocytosis with primary cutaneous marginal zone lymphoma; first case

Primary cutaneous lymphoma in a patient with mastocytosis: Is there an association?

“… 1 Subsequent to this experimental evidence, there are 3 additional cases reporting a co-occurrence of PCL in patients with mastocytosis, all of which were B-cell type ( Table I ). 2 , 6 , 7 In conjunction with the present case, we contemplate whether these PCL cases represent a previously underrecognized connection. While PCLs are a heterogenous group of neoplasms with varied presentation, the few described cases in patients with mastocytosis appear to have some common clinical features.…”

“…But given the distinct clonal origins of mast cells and lymphocytes, this association remains unclear. 2 …”

“… 5 Historically, the lymphoproliferative disorders reported in association with mastocytosis have traditionally been systemic B-cell lymphomas. 2 The question this case raises is the possibility of whether skin-residing mast cells also contribute in a similar fashion to promoting the growth of certain cutaneous neoplasms. In strong support of this, recent studies have reported the presence of mast cells, even in increased numbers, in both B- and T-cell types of PCL neoplasms.…”

“… 1 Patients with mastocytosis are at an increased risk for myeloid malignancies, with emerging evidence of a co-occurrence with a subset of lymphoproliferative disorders, primary cutaneous lymphomas (PCL). 2 Because the presentation of PCL lesions can be asymptomatic and subtle, we aim to alert dermatologists of the clinical clues in patients with longstanding mastocytosis.…”