Lobato-Mendizábal E, Ruiz-Argüelles G J, Ruiz-Argüelles A, Figueroa S
Hospital Universitario de Puebla, México.
Rev Invest Clin. 1990 Jul-Sep;42(3):226-30.
Primary bone marrow lymphomata are infrequent; most of them are of B-cell origin, and those of a T-cell lineage produce mainly both hypercalcemia and osteolytic lesions apparently due to abnormal production of osteoclast-activating factor. We report a 15-year old patient with a primary bone marrow lymphoma: 85% of his infiltrating malignant lymphocytes displayed cytoplasmic mu-chains compatible with a pre-B phenotype. The cells failed to display the CALLA/CD 10 antigen. Serum calcium was 7.5 mEq/L (range 4-5 mEq/L); the bone biopsy of an osteolytic lesion disclosed a large-cell, diffuse non-Hodgkin's lymphoma. No malignant cells were found in the peripheral blood and there were no enlarged lymph nodes. The patient was treated with 6 courses of chemotherapy: hydroxyldaunorubicin, vincristine and prednisone (HOP). Complete remission was achieved and the patient was placed on continuation chemotherapy with daily six-mercaptopurine and weekly methotrexate, together with HOP pulses every three months. The hypercalcemia disappeared together with the fever and the bone pain: the patient has been followed 6 months. Data on this case are discussed together with those previously published in regard to the low prevalence of bone lesions in primary B-cell lymphomas of the bone marrow, and to the similarity of this B-cell malignancy to others that produce both hypercalcemia and bone lesions, i.e. multiple myeloma.
原发性骨髓淋巴瘤较为罕见;其中大多数起源于B细胞,而T细胞系的淋巴瘤主要导致高钙血症和溶骨性病变,这显然是由于破骨细胞激活因子的异常产生所致。我们报告了一名15岁的原发性骨髓淋巴瘤患者:其浸润的恶性淋巴细胞中有85%显示出与前B细胞表型相符的细胞质μ链。这些细胞未显示CALLA/CD 10抗原。血清钙为7.5 mEq/L(正常范围4 - 5 mEq/L);对一处溶骨性病变进行的骨活检显示为大细胞弥漫性非霍奇金淋巴瘤。外周血中未发现恶性细胞,也没有肿大的淋巴结。该患者接受了6个疗程的化疗:羟基柔红霉素、长春新碱和泼尼松(HOP方案)。实现了完全缓解,患者继续接受化疗,每日服用6 - 巯基嘌呤,每周服用甲氨蝶呤,每三个月进行一次HOP方案冲击治疗。高钙血症、发热和骨痛均消失:对该患者进行了6个月的随访。结合此前发表的关于原发性骨髓B细胞淋巴瘤中骨病变低发生率的数据,以及这种B细胞恶性肿瘤与其他导致高钙血症和骨病变的疾病(即多发性骨髓瘤)的相似性,对该病例的数据进行了讨论。