Clinical characteristics, prognostic indicators, and survival outcomes in intravascular lymphoma: Mayo Clinic experience (2003–2018)

Abstract

Intravascular lymphoma (IVL) is a rare extranodal non‐Hodgkin lymphoma. We performed a retrospective analysis of 55 IVL patients who were treated at our institution 2003–2018. Median age at diagnosis was 68 years, and 64% were males. The most frequent presenting symptoms were skin rash 43% and weight loss 30%. MRI brain on IVL patients with CNS involvement (CNS‐IVL) showed multifocal involvement in 76% (13/17). 89% (17/19) of non‐CNS‐IVL patients with abnormal FDG‐PET had biopsy of an avid lesion resulting in definitive diagnosis. The top diagnostic biopsy site was the bone marrow (45%). 56% had multiorgan involvement. Based on CNS involvement, 36.5% (20/55) had CNS‐IVL and 63.5% (35/55) had non‐CNS‐IVL. CNS‐IVL group consists of clinically isolated CNS involvement (CNS‐only IVL) (22%;12/55) and mixed clinical CNS and peripheral site involvement (M‐IVL) (14.5%; 8/55). Non‐CNS‐IVL group consists of clinically isolated skin involvement (skin‐only IVL) (9%; 5/55) and peripheral IVL with or without skin involvement (P‐IVL); (54.5%; 30/55). Skin involvement was predominantly in the lower extremities. Pathologically, 89% (48/54) were B‐cell IVL. Rituximab + high‐dose methotrexate‐based regimen were used in 75% (12/16) of CNS‐IVL patients and RCHOP in 60% (17/28) of non‐CNS‐IVL patients. Estimated 5‐year progression free survival (PFS) and overall survival (OS) for the entire cohort were 38.6% and 52%, respectively. Skin‐only IVL was associated with excellent survival. Platelet count <150x109/L, age > 60Y, and treatment without Rituximab were poor prognostic factors. Further research is necessary to identify novel therapies.

Cutaneous Intravascular Hematolymphoid Entities: A Review

“…BCL2 (80-100%) [11], CD10 (13%) [17], and MUM1 (75-100%) positivity [14,16,33], with varied positivity for BCL6 [16,33], were reported. Positivity for MYC (68-71.4%) [16,33] and IgM (91%) [16] was reported. CD3 is importantly negative and distinguishes IVLBCL from T-cell lymphoma [2].…”

“…The tumor cells of IVLBCL express CD20 (99-100%), Pax5 (100%), CD79a (100%), and other B-cell markers such as CD19, BOB.1, and OCT2 [2,14,16,32]. BCL2 (80-100%) [11], CD10 (13%) [17], and MUM1 (75-100%) positivity [14,16,33], with varied positivity for BCL6 [16,33], were reported. Positivity for MYC (68-71.4%) [16,33] and IgM (91%) [16] was reported.…”

“…One potential pitfall is the expression of pan T-cell marker CD5 (38-50%) [11,16,[33][34][35]. The largest review of 342 cases showed 45.1% CD5 positivity [14].…”

“…One study [32] reported a Ki-67 proliferation index above 60% in all 17 cases; when stratified into "lower" (60-79% proliferation index) and "higher" (80-100% proliferation index), the one-and two-year survival rates were significantly different, 83.3% versus 60.0% one-year survival rate and 31.3% versus 0.0% two-year survival rate. One potential pitfall is the expression of pan T-cell marker CD5 (38-50%) [11,16,[33][34][35]. The largest review of 342 cases showed 45.1% CD5 positivity [14].…”

Primary Bone Marrow Lymphoma: De Novo and Transformed Subtypes

“…1,[3][4][5] A diagnosis of PBML also requires exclusion of intravascular lymphoma, leukemias and other aggressive B-cell lymphomas known for primary bone marrow involvement and secondary bone lymphoma which arises from infiltration of the marrow in the setting of disseminated disease. 1,4,5,[9][10][11]16 The majority of reported PBML cases were DLBCLs; however, they were diagnosed before the widespread use of HGBCL as a diagnostic entity. 1,6,8 We have used HGBCL confined to the bone marrow with or without leukemic component as the operational diagnostic criteria irrespective of the underlying lymphoma subtype.…”