肥厚性硬膜炎様の神経症状で発症したマントル細胞リンパ腫  [in Japanese] Mantle cell lymphoma manifesting neurological symptoms similar to those of hypertrophic cranial pachymeningitis  [in Japanese]

Access this Article

Author(s)

Abstract

症例は58歳男性。2007年8月より頭痛,飛蚊症を自覚し,左視神経乳頭浮腫・出血を指摘された。頭部MRIにて硬膜肥厚像を認め,肥厚性硬膜炎として抗生剤,ステロイド加療されたが症状が持続した。転移性腫瘍を疑い施行された胸腹部CTにて表在リンパ節,腹腔内リンパ節腫脹を認め当院へ紹介転院となり,リンパ節生検にてマントル細胞リンパ腫(MCL)と診断された。また,開頭生検により硬膜肥厚も腫瘍細胞のびまん性浸潤によることを確認した。R-Hyper-CVAD/R-MTX-AraC療法にて硬膜肥厚は消失し,眼底所見,症状の改善を認めた。地固め療法として自家末梢血幹細胞移植併用大量化学療法を施行し,現在完全寛解持続中である。リンパ腫の二次性硬膜病変は極めて稀であり,一方,肥厚性硬膜炎は脳や脊髄硬膜の肥厚を生じる慢性進行性炎症性疾患であり,様々な病因で発症するため,その原因精査が重要となる。血液悪性腫瘍に合併する硬膜肥厚を鑑別する上で貴重な症例と考えられ報告する。

The patient was a 58-year-old man, who presented with headache and myodesopsia. He demonstrated papilledema and hemorrhage in the fundus of the left eye and MRI findings showed localized hypertrophic dura mater. He was diagnosed as having hypertrophic cranial pachymeningitis and treated with antibiotics and prednisolone. However, the patient complained of persistent headache. Therefore, CT scans of the chest and abdomen were obtained. These images demonstrated superficial and intraabdominal lymphadenopathy and a histological diagnosis of mantle cell lymphoma was made on biopsy of an inguinal mass. Specimen obtained at craniotomy also showed the same lymphoma cells diffusely infiltrating the dura mater. Complete remission of the lymphoma including disappearance of hypertrophic dura mater was obtained after 4 courses of rituximab plus hyper-CVAD alternating with high-dose methotrexate and cytarabine, and the neurological manifestation improved thereafter. Subsequently, he underwent autologous peripheral blood stem cell transplantation. Hypertrophic pachymeningitis is a chronic progressive inflammatory disorder with hypertrophic dura mater of brain and spinal cord, caused by diverse illnesses. Mantle cell lymphoma infiltrating the dura mater is extremely rare, and has not been reported previously. In some cases of hematological malignancy with hypertrophic pachymeningitis, dural biopsy is required to differentiate the etiology.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 52(11), 1788-1793, 2011

    The Japanese Society of Hematology

Codes

Page Top