CT Features Of Lung Involvement In 28 Patients With Intravascular Lymphoma
Shimpei GotohIsao ItoMasanori KitaichiTakeshi KuboYuko NishimotoTakashi OguraKeisuke TomiiMoon Hee HwangNaoki NishimuraAsuka OkadaEiji KojimaYoshio TaguchiRyo TachikawaNoriko TsuchiyaSumito ChoKiyoshi KannoHiromasa TachibanaTadashi IshidaShoutarou OkachiMasanori NishikawaSeizo KadowakiMotonari FukuiMichiaki Mishima
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血管内大細胞型B細胞リンパ腫（intravascular large B-cell lymphoma：IVLBCL）は，全身臓器の細小血管内に腫瘍細胞が選択的に増殖する節外性B細胞リンパ腫の一型である．近年，MYD88変異やCD79B変異，PD-L1構造異常を高率に伴う分子遺伝学的特徴が明らかとなり，治療においては，R-CHOP（リツキシマブ，シクロホスファミド，ドキソルビシン，ビンクリスチン，プレドニゾロン）療法に中枢神経指向治療を組み合わせることによる良好な治療成績が示された．病態への理解の深化と治療成績のさらなる向上が今後の課題である．
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Abstract We report a case of a 59-year-old man who first presented with a nodal diffuse large B-cell lymphoma that later relapsed as an intravascular large B-cell lymphoma. In the initial biopsy specimen, a few intranodal small vessels that contained large lymphoma cells were noted. After 8 months of multiagent chemotherapy, clinical remission was attained. Two years after the initial diagnosis of nodal diffuse large B-cell lymphoma, the patient presented with a rapid onset of multiorgan failure, which at autopsy was shown to be due to intravascular large B-cell lymphoma. Molecular genetic studies showed that these 2 lymphomas had immunoglobulin heavy-chain gene rearrangements that were of identical size, suggesting that they were derived from the same clone. To our knowledge, this is the first report of a nodal large B-cell lymphoma that relapsed as an intravascular large B-cell lymphoma. Although this report is of only a single case, the presence of a relatively inconspicuous intravascular component in an otherwise typical nodal large B-cell lymphoma may be predictive and could affect clinical decisions regarding diagnosis, monitoring, and prognosis of such lymphomas.
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Intravascular large B-cell lymphoma (IVLBCL) is a rare (<1%), typically aggressive extranodal variant of mature non-Hodgkin B-cell lymphoma. IVLBCL is characterized by malignant lymphoid cells lodged within blood vessels, particularly capillary channels. Herein, we present a case of a 50-year-old man with a history of myeloradiculitis (∼1 year) and paraparesis requiring hospitalization. During the course of his hospital stay, computed tomography (CT), magnetic resonance imaging, CT-positron emission tomography, and biopsy failed to establish a diagnosis. The patient died 2 months later from bilateral pneumonia. Postmortem examination was undertaken to determine the cause of death. Histologic sections of the patient’s brain, heart, lung, and liver showed aggregates of highly atypical cells bearing enlarged, pleomorphic, and hyperchromatic nuclei. Strong intravascular positivity for CD45 and CD20 markers indicated the cells were of B-cell origin, supporting a diagnosis of IVLBCL.
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