Should CLIPPERS Be Considered a Prelymphoma State or a New Inflammatory Disease?—Reply

Peripheral T cell lymphoma after chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report

“…[3][4][5][6] However, whether CLIPPER is an independent, new disease or a pre-lymphoma state remains unclear. [7] Now, we describe one more patient clinically diagnosed as CLIPPERS developed peripheral T cell lymphoma, not otherwise specified 3 (PTCL-NOS), which was confirmed by extra-brain biopsy.…”

Case 259: Primary Central Nervous System Lymphomatoid Granulomatosis Mimicking Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

“…The pathogenesis of CLIPPERS is unknown. The present case may suggest that CLIPPERS might be a prelymphoma state (9).Thus, patients with newly diagnosed CLIPPERS, even those with pathologic confirmation, should be closely observed for at least 2-3 years. Follow-up should include repeated clinical examination, contrast-enhanced head MRI, and pathologic analysis of brain tissue, if necessary.…”

“…Serum analysis was also negative for human immunodeficiency virus type 1 and 2 RNA, hepatitis B and C antigen or antibody profile, and fluorescent treponemal antibody absorption. Cerebrospinal fluid (CSF) analysis revealed clear fluid, a normal glucose level, an elevated protein level (45 mg/dL; normal range, 20-40 mg/dL), and an elevated white blood cell count (10/mm 3 [0.01 ×10 9 /L]; normal range, 0-8/mm 3 [{0-0.008} ×10 9 /L]; 81% lymphocytes, 19% monocytes). No CSF-specific oligoclonal bands were detected.…”

Peripheral T cell lymphoma after chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report

“…However, only two patients of CLIPPERS associated with T cell lymphoma have been reported. [5,6] One case of cutaneous T-cell lymphoma (Mycosis fungoides) [5] and one case of PTCL-NOS, [6] were both presenting eventually with CLIPPERS features. We reported the first case presenting initially with CLIPPERS of clinical and radiological features progressed eventually to PTCL-NOS.…”

“…[1][2] Recently, it has been gradually found that outcome of CLIPPERS is not necessarily benign, because some of the cases turned out to be lymphoma. [3][4][5][6] However, whether CLIPPER is an independent, new disease or a pre-lymphoma state remains unclear. [7] Now, we describe one more patient clinically diagnosed as CLIPPERS developed peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), which was confirmed by extra-brain biopsy.…”