Published online May 6, 2015. doi: 10.5315/wjh.v4.i2.10
Peer-review started: July 18, 2014
First decision: August 28, 2014
Revised: March 2, 2015
Accepted: March 16, 2015
Article in press: March 18, 2015
Published online: May 6, 2015
An 82-year-old Japanese man visited our emergency unit complaining of dyspnea. Laboratory data showed 15% atypical lymphocytes in peripheral blood which expressed the T-cell phenotype. Chest/abdominal computed tomography depicted hepatosplenomegaly and swelling of systemic lymph nodes. The patient died of advanced respiratory failure 5 d after the first occurrence of his dyspnea. At autopsy, the pathological features revealed a diffuse infiltration of large atypical lymphocytes to systemic organs including the spleen and lung. In immunohistochemical staining, these cells expressed CD30, TIA-1, anaplastic lymphoma kinase (ALK), CD5 and CD3. An advanced surface molecule analysis revealed a lack of CD54 (intercellular cell adhesion molecule-1) and CD56 (neural cell adhesion molecule). We observed the proliferation and infiltration of these lymphoma cells specifically at the intravascular lesions similar to intravascular lymphoma (IVL). T-cell IVL is not established as an independent clinical entity in the World Health Organization classification, and our patient’s ALK-positive T-IVL in lung appears to be the first reported case.
Core tip: Intravascular lymphoma (IVL), known as a mature B-cell neoplasm that proliferates intravascularly, is established as an independent clinical entity in the World Health Organization classification. Our patient’s case suggests that IVL is one of the characteristic proliferation patterns commonly seen in lymphomas including B- and T-cell neoplasms, anaplastic large-cell lymphoma, and more. We speculate that the function of the original lymphocytes (T or B cells) designates the tumor biology of IVL. Intravascular proliferation is determined by a lack of adhesion molecules. We suspect that the biological aggressiveness would be modified by other characteristic phenotypes such as anaplastic lymphoma kinase, CD30, and CD56.