t(14;19)を含む複雑な核型を有する超高リスク難治性多発性骨髄腫

[Ultra high-risk refractory multiple myeloma with a complex karyotype including t(14;19)].

• Go Aoki
• Aiko Sawazaki
• Kazuo Notsumata
• Yasuyuki Ushiogi
• Kazuhiro Okafuji
• Daishu Toya

抄録

78歳の男性が慢性腎不全の急速な進行により入院し、t(14;19)を含む複雑な核型を持つ多発性骨髄腫（MM）IgG-λ型ISS-III R-ISS-IIと診断された。ボルテゾミブを中心とした治療を受けても腎不全が進行した。週3回の透析に依存するようになった。daratumumab（DARA）をベースとした治療法を導入したところ、腎機能は改善し、透析回数は週2回に減少し、遊離軽鎖（FLC）比も改善した。しかし，骨髄腫は最終的に難治性の経過をたどったため，ポマリドマイド（POM）-デキサメタゾン（Pd）療法が施行された。Pd療法は顕著な効果を示し，3回の治療でFLC比が正常化した。しかし，骨髄腫は多発性髄外腫瘤を伴って再発し，del(17p)陽性となり，最終的には発症470日目に死亡した．t(14;19)を有するMMは稀であり、予後は悪く、非常に侵攻的な経過をたどる；しかしながら、DARAやPOMを早期に導入することで、長期的な治療効果が得られる可能性がある。

A 78-year-old man was hospitalized because of rapid progression of chronic renal failure and diagnosed with multiple myeloma (MM) IgG-λ type ISS-III R-ISS-II with complex karyotype including t(14;19). Even after receiving bortezomib-based regimens, his renal failure progressed. He became dependent on dialysis, which was required three times a week. After introducing the daratumumab (DARA)-based regimen, his renal function improved, the frequency of dialysis decreased to twice a week, and the free light chain (FLC) ratio also improved. However, his myeloma eventually followed a refractory course; therefore, pomalidomide (POM)-dexamethasone (Pd) regimen was administered. Pd regimen had a marked effect and normalized the FLC ratio after three courses of the treatment. However, his myeloma reprogressed with multiple extramedullary masses and he became del(17p) positive; eventually, he died on the 470th day of disease. MM with t(14;19) is rare and has a poor prognosis with a highly aggressive course; however, early introduction of DARA or POM may provide long-term response.