Liesveld J L, Rowe J M, Lichtman M A
Blood. 1987 Mar;69(3):820-6.
One hundred-nine cases of autoimmune hemolysis were reviewed to determine the frequency of reticulocytopenia, the state of the erythroid marrow in reticulocytopenic cases, and the course of reticulocyte production indices with time and glucocorticoid treatment. The mean hematocrit at presentation was 24 mL/dL, but 30% of cases had an initial hematocrit less than 20 mL/dL. Median reticulocyte percentage at diagnosis was 9%, and median reticulocyte production index was 2.8 times basal. Twenty percent of cases had an initial reticulocyte count less than 4%, and 37% had an initial reticulocyte production index less than 2.0 times basal. These reticulocytopenic patients were nearly evenly distributed between warm and cold antibody-mediated cases and between primary and secondary cases. Fifty-four percent of reticulocytopenic cases had a bone marrow examination during hospitalization. Three-fourths of these marrows showed erythroid hyperplasia, and erythroid hypoplasia was seen in only one case. Eighty-eight cases had serial reticulocyte measurements, and in only 15% of patients did the reticulocyte production index remain less than 2.0 times basal. Thus, in most cases, the initially low reticulocyte production index may represent a lag in marrow responsiveness to hemolytic stress. In cases with persistent reticulocytopenia, ineffective erythropoiesis is suggested by the frequency of marrow erythroid hyperplasia. In the cases that were initially reticulocytopenic and demonstrated an increase in reticulocyte production index, the magnitude of this increase was significantly greater in glucocorticoid-treated patients than in those not so treated, indicating that a glucocorticoid sensitive component exists in the marrow erythropoietic response to hemolysis. Awareness of the frequency of an initial reticulocytopenia in cases of autoimmune hemolysis may be important in initial diagnosis and treatment.
回顾了109例自身免疫性溶血病例，以确定网织红细胞减少的发生率、网织红细胞减少病例的红系骨髓状态，以及网织红细胞生成指数随时间和糖皮质激素治疗的变化过程。就诊时的平均血细胞比容为24 mL/dL，但30%的病例初始血细胞比容低于20 mL/dL。诊断时网织红细胞百分比中位数为9%，网织红细胞生成指数中位数为基础值的2.8倍。20%的病例初始网织红细胞计数低于4%，37%的病例初始网织红细胞生成指数低于基础值的2.0倍。这些网织红细胞减少的患者在温抗体介导和冷抗体介导的病例之间，以及原发性和继发性病例之间分布几乎均匀。54%的网织红细胞减少病例在住院期间进行了骨髓检查。其中四分之三的骨髓显示红系增生，仅1例可见红系发育不全。88例进行了系列网织红细胞测量，只有15%的患者网织红细胞生成指数持续低于基础值的2.0倍。因此，在大多数情况下，最初较低的网织红细胞生成指数可能代表骨髓对溶血应激反应的延迟。在持续存在网织红细胞减少的病例中，骨髓红系增生的频率提示存在无效造血。在最初网织红细胞减少但网织红细胞生成指数增加的病例中，糖皮质激素治疗患者的增加幅度明显大于未接受治疗的患者，表明骨髓对溶血的造血反应中存在糖皮质激素敏感成分。认识到自身免疫性溶血病例中网织红细胞减少的发生率，对初始诊断和治疗可能很重要。
