Studies on the hyperammonemia and amino acid disturbances in multiple myeloma

报道了两名似乎产生氨的多发性骨髓瘤患者（Acta Haematol. 84：130-134，1990）。两例患者均表现出高氨血症和氨基酸紊乱，如低Fischer比值。1例Bence Jones蛋白（λ）型骨髓瘤患者出现昏迷，经化疗后高氨血症和意识障碍改善。另一名患者患有伊加κ型骨髓瘤，嗜睡，尽管接受了强化化疗，但仍死于恶性胸膜炎。两个病例的尸检显示广泛的多发性骨髓瘤和几乎正常的肝脏。从第二位患者建立细胞系（KHM-4）（Internal Med.31：339-343，1992）。KHM-4细胞的表面标志物研究表明，细胞对细胞质免疫球蛋白（伊加κ）、HLA-DR和T10呈阳性。通过向无氨基酸的RPMI 1640培养基中添加L-谷氨酰胺和L-精氨酸来检测KHM-4细胞的氨分泌。在L-谷氨酰胺的存在下，KHM-4细胞比T细胞系CEM分泌更大量的氨。然而，在其他细胞系中未观察到L-精氨酸产生氨。这些观察结果提供了高氨血症和血清氨基酸紊乱。

Two patients with multiple myeloma who appeared to be producing ammonia are reported(Acta Haematol. 84 : 130-134, 1990). Both patients showed hyperammonemia and amino acid disturbances, such as a low Fischer ratio. One patient had Bence Jones protein(lambda)type myeloma and became comatose, but the hyperammonemia and disturbance of consciousness were improved by chemotheray for the myeloma. The other patient had IgA kappa type myeloma and somnolence and died of malignant pleurisy despite intensive chemotherapy. Autopsy of both cases, showed widespread multiple myeloma and an almost normal liver. A cell line(KHM-4)was established from a second patient (Internal Med. 31 : 339-343, 1992). Surface marker studies of KHM-4 cells showed that the cells were positive for cytoplasmic immunoglobulins(IgA kappa), HLA-DR, and T10. Secretion of ammonia by the KHM-4 cells was detected by the addition of L-glutamine and L-arginine into the culture medium of amino acid-free RPMI 1640. In the presence of L-glutamine, KHM-4 cells secreted a greater amount of ammonia than the T cell line, CEM. However, production of ammonia by L-arginine was not observed in other cell lines. These observation provide hyperammonemia and serum amino acid disturbances.