Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hematol. May 6, 2015; 4(2): 10-15
Published online May 6, 2015. doi: 10.5315/wjh.v4.i2.10
Intravascular proliferating anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma
Kohei Shiroshita, Jun-ichiro Kida, Kensuke Matsumoto, Makiko Uemura, Genji Yamaoka, Yumi Miyai, Reiji Haba, Osamu Imataki
Kohei Shiroshita, Post-Graduate Clinical Training Center, Kagawa University Hospital, Kagawa 761-0793, Japan
Jun-ichiro Kida, Kensuke Matsumoto, Makiko Uemura, Osamu Imataki, Division of Hematology and Stem Cell Transplantation, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa 761-0793, Japan
Genji Yamaoka, Department of Laboratory Medicine, Kagawa University Hospital, Kagawa 761-0793, Japan
Yumi Miyai, Reiji Haba, Department of Diagnostic Pathology, Faculty of Medicine, Kagawa University, Kagawa 761-0793, Japan
Author contributions: Shiroshita K and Imataki O contributed equally to this work; Shiroshita K and Imataki O wrote the manuscript; Kida J and Matsumoto K designed the research; Uemura M essentially contributed to the treatment of the case; Yamaoka G performed analysis; Miyai Y and Haba R contributed to the pathological analysis; and Imataki O organized the report.
Ethics approval: The study was reviewed and approved by the Kagawa University, Medcal, Institutional Review Board.
Informed consent: The patients or patient’s representative provided informed written consent prior to study enrollment.
Conflict-of-interest: None.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Osamu Imataki, Division of Hematology and Stem Cell Transplantation, Department of Internal Medicine, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa 761-0793, Japan. oima@med.kagawa-u.ac.jp
Telephone: +81-878-912145 Fax: +81-878-912147
Received: July 17, 2014
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
Abstract

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.

Keywords: Malignant lymphoma, Cytotoxic molecule, Intravascular lymphoma, Anaplastic lymphoma kinase, Anaplastic large-cell lymphoma

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.