Marrow involvement in cutaneous T-cell lymphoma: a clinicopathologic study of 60 cases

Cutaneous T-cell lymphomas (CTCLs), including Sézary syndrome, are generally assumed to spare the marrow until the disease is far advanced. The authors reviewed marrow sections from 60 patients with CTCL, looking carefully for aggregates of cerebriform cells, and found marrow involvement in 13 patients (21.7%); another 19 had abnormal lymphoid nodules that were not diagnostic of involvement, and 28 had marrows with negative results. Involved marrows had nodules or infiltrates of dysplastic cerebriform cells that were often subtle, without a significant increase in cellularity; only one case showed massive involvement. Patients with CTCL with an infiltrative component of marrow involvement had associated peripheral blood involvement (eight of eight), generalized erythroderma (six of eight), lymph node involvement (five of eight), visceral progression (five of eight), and significantly shortened median survival compared with patients with CTCL with negative marrows (11 months and 70 months, respectively; P = 0.007). In contrast, five patients with nodules of tumor in the marrow but lacking an infiltrative component did not have peripheral blood involvement; only one patient had adenopathy or visceral progression develop, and two patients have died. Significant hematologic abnormalities were generally absent. Eight of 13 patients with marrow involvement had advanced skin disease, but skin disease was limited to plaques in five patients. Eight patients had marrow involvement develop within three months of initial diagnosis. Thus, marrow involvement occurs in approximately 20% of patients with CTCL, is often present at initial diagnosis, and is associated with widespread dissemination and shortened survival time when an infiltrative component is present.