PMC3262387_01
Lactate dehydrogenase (normal 108-202 U/L) shown as a function of time (hospital days). Lactate dehydrogenase gradually dropped after initiating treatment with eculizumab. Eculizumab administration is denoted by vertical tick marks (hospital days 6, 13, 20, 27, 34, 48, and day 63). Four days after the first dose, lactate dehydrogenase dropped to 837 U/L. After the second dose of eculizumab on day 13, lactate dehydrogenase dropped to 556 U/L. A week after the third dose, lactate dehydrogenase was down to 349 U/L. Three days after the sixth dose, lactate dehydrogenase was normal. It did rise slightly before the seventh dose was due.
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Four days after receiving her first dose of eculizumab, the patient's lactate dehydrogenase dropped to 837 U/L and her complement levels rose: C3 rose to 81 (83-184) mg/dL and C4 rose to 16 (17-59) mg/dL, as shown in Figures 1 and 2. A week after her third dose of eculizumab, her lactate dehydrogenase was down to 349 U/L (Figure 1). Three days after her sixth dose, her lactate dehydrogenase, haptoglobin, C3, and C4 were all normal (see Figures 1-5 for a summary of treatment responses).
C3 (normal 83-184 mg/dL) shown as a function of time (hospital days). Eculizumab administration is denoted by vertical tick marks (hospital days 6, 13, 20, 27, 34, 48, and day 63). After the second dose of eculizumab on day 13, C3 rose to 81. Thirteen days after the sixth dose, C3 was normal and remained normal.
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Four days after receiving her first dose of eculizumab, the patient's lactate dehydrogenase dropped to 837 U/L and her complement levels rose: C3 rose to 81 (83-184) mg/dL and C4 rose to 16 (17-59) mg/dL, as shown in Figures 1 and 2. Three days after her sixth dose, her lactate dehydrogenase, haptoglobin, C3, and C4 were all normal (see Figures 1-5 for a summary of treatment responses).
Creatinine (normal 0.5-1.3 mg/dL) shown as a function of time (hospital days). Eculizumab administration is denoted with vertical tick marks (hospital days 6, 13, 20, 27, 34, 48, and day 63). The start and end dates of continuous venovenous hemodialysis are marked on the time axis by 2 carat (^) symbols (hospital days 4 and 27). Three days after the second dose of eculizumab, dialysis frequency was reduced to three times per week. Continuous venovenous hemodialysis was completely discontinued after the fourth dose of eculizumab. Despite a delayed and reduced dose of eculizumab at the seventh administration, creatinine continued to decrease to as low as 2.24 mg/dL.
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Three days after her sixth dose, her lactate dehydrogenase, haptoglobin, C3, and C4 were all normal (see Figures 1-5 for a summary of treatment responses). Additionally, her creatinine has continued to decrease off dialysis, with recent levels as low as 2.24 mg/dL (see Figure 3 for a summary of creatinine over time).
Haptoglobin shown as a function of time (hospital days). Eculizumab administration is denoted by vertical tick marks (hospital days 6, 13, 20, 27, 34, 48, and days 63 and 69). Initial haptoglobin was <6 (normal range 62-197) mg/dL. Five days after the third dose, haptoglobin was normal and remained normal until the time the seventh dose was due. Haptoglobin then dropped precipitously, indicating an ongoing need for the drug. Haptoglobin did increase again after the seventh dose on day 63, and later normalized to 71 mg/dL three days after receiving the eighth dose on day 69.
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Three days after her sixth dose, her lactate dehydrogenase, haptoglobin, C3, and C4 were all normal (see Figures 1-5 for a summary of treatment responses). As shown in Figure 4, the haptoglobin increased again after the seventh dose, and normalized to 71 mg/dL three days after the eighth dose.
C4 (normal 17-59 mg/dL) shown as a function of time (hospital days). Eculizumab administration is denoted by vertical tick marks (hospital days 6, 13, 20, 27, 34, 48, and day 63). The day after the second dose of eculizumab on day 13, C4 rose to 16. Three days after the sixth dose, C4 was normal and remained normal.
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Three days after her sixth dose, her lactate dehydrogenase, haptoglobin, C3, and C4 were all normal (see Figures 1-5 for a summary of treatment responses).
Magnetic resonance imaging scan findings.
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There was also a suspected tiny subacute infarct within the right centrum semiovale (Figure 6).