Intravascular lymphomatosisの1例—化学療法の治療経験

Intravascular Lymphomatosis : A Case Report

I．はじめに

　Intravascular lymphomatosis（血管内リンパ腫症）は全身の小血管が腫瘍細胞によって閉塞し，致死的経過を辿る極めて稀な疾患である^7）．今回，われわれは脳表静脈に腫瘍細胞を認め，化学療法を施行するも効果なく，発症3カ月で死亡した症例を経験したので文献的考察を含め報告する．

We report a case of a 64-year-old man with intravascular lymphomatosis. He had dementia and general-ized seizure. Computed tomogram scan and magnetic resonance imaging showed multiple infarctions in theright frontal lobe, right cerebellar hemisphere and left occipital lobe.

A month after admission he had a daily high fever and frequent convulsions, The level of serum LDHwas progressively getting higher and the level of β2-microglobulin in both serum and CSF was high, Oneand a half months after admission we performed an open biopsy for making a definite diagnosis by patho-logical examination. Histological diagnosis was intravascular lymphomatosis (angiotrophic lymphoma) oflarge B cell type.

He was treated with regimens of combined chemotherapy with M-CHOP (methotrexate, cyclophospha-mide, adriamycin, vincristine, prednisolone). After chemotherapy his consciousness was gradually improv-ing better and the level of serum LDH and β2-microglobulin was reduced to a low level. Three months af-ter admission he died of respiratory failure.

After a review of the literature and experience with this case, it is obrious that early diagnosis of thisdisease is difficult. However, we think that multiple cerebral infarctions on neuroradiological examinationsand high levels of serum LDH (especially LDH₂ and LDH₃₎ and β2-microglobulin in CSF and serumshouid lead to a presumptive diagnosis of this disese, and biopsy should be used to make a difinite diagno-sis.