Intravascular T‐cell lymphoma: A rare, poorly characterized entity with cytotoxic phenotype

Abstract

Intravascular T-cell lymphomas are rare, poorly characterized lesions. We discuss the clinical, radiologic and especially the laboratory characteristics of a lesion which presented in a 62-year-old woman with a history of progressive CNS abnormalities. Throughout the course of the disease, radiologic findings consisted mainly of multifocal mixed areas of ischemia and vasogenic edema involving cortical and subcortical regions. A brain biopsy identified an abnormal T-cell population confined to lumens of vessels. These T-cells were abnormal cytotoxic cells, positive for CD3, CD8, and negative for CD2, CD4, CD5, CD7 and CD30. While flow cytometry and immunohistochemistry failed to identify a similar population in the blood or bone marrow, molecular studies showed a clonal T-cell population in both the brain and the bone marrow. No other organs were involved. In spite of aggressive treatment, the patient's medical condition continued to progress and she passed away. In conclusion, this abnormal population of cytotoxic T-cells with intravascular localization probably represents a specific type of T-cell lymphoma with specific clinical, radiologic, molecular and immunophenotypic characteristics.

Intravascular NK/T-cell lymphoma, Epstein–Barr virus positive with multiorgan involvement: a clinical dilemma

“…Hematologic findings, including leukopenia, thrombocytopenia, pancytopenia occurred in 4 patients as well as in our case [ 2 , 3 , 7 ]. Bone marrow involvement is uncommon, being detected in 3/16 evaluated, including the present report [ 3 , 17 ]. In the case by Wu et al the diagnosis was made on bone marrow biopsy in a 47-year-old female with protean systemic and neurological symptoms [ 3 ].…”

“…The disease often presented with skin lesions (22/24) and in 11 cases the skin was the unique site of involvement. Sharma et al reported a case presenting with almost unique brain involvement [ 17 ]. In 5 patients the disease affected both skin and brain [ 2 , 5 , 7 , 12 , 16 ].…”

“…As Wu et al pointed out the lymphomatous sinusoidal infiltrate in bone marrow might be subtle (as in our case) and detected only by immunohistochemical staining [ 3 ]. In the case of Sharma et al bone marrow involvement was identified only by molecular studies showing a clonal T-cell population [ 17 ].…”

Cutaneous intravascular CD30+ T‐cell pseudolymphoma occurring in a regressing keratoacanthoma

“…EBV was found in 9 cases and 6 cases were assessed for beta‐F1, a marker of the alpha/beta‐TCR, with all cases being negative. Like IVLBCL, the prognosis is poor and death is related to CNS involvement …”

The Role of Co-Stimulatory Molecules in Chagas Disease

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