Presenting features of 22 consecutive patients with severe deficiency (< 5%) of ADAMTS13 activity.*
| * Data are presented on 22 consecutive patients from the Oklahoma TTP-HUS Registry, November 13, 1995-December 31, 2003, who had a severe deficiency of ADAMTS13 activity at the time of their initial presentation. This experience represents an extension of our previously published data.3 The number of major presenting symptoms exceeds 22 because some patients had multiple major symptoms. The duration of symptoms describes the patient’s history of the initial onset of symptoms until diagnosis of TTP-HUS. Severe neurologic abnormalities included coma, stroke, seizure, or fluctuating focal signs; mild neurologic abnormalities included headache, blurred vision, ataxia, or mental status changes with transient confusion. Acute renal failure and renal insufficiency have been previously defined.3 Lactate dehydrogenase (LDH) values were adjusted to an upper limit of normal value of 200 U/L. Age, duration of symptoms, and laboratory data are median values. Laboratory data are the most abnormal values at the time of diagnosis of TTP-HUS ± 7 days, to account for transient effects of transfusion and potential worsening after diagnosis. | |
| Age | 39 years (range, 19–71) |
| Gender | 18 (82%) female |
| Race | 10 (45%) African-American |
| Obesity | 12 (55%) BMI ≥ 30 kg/m2 |
| Presenting symptoms: | |
| neurologic abnormalities | 13 (59%) |
| nausea, vomiting, diarrhea | 6 (27%) |
| abdominal pain | 6 (27%) |
| weakness | 4 (18%) |
| chest pain | 3 (14%) |
| hematuria | 2 (9%) |
| menorrhagia | 1 (5%) |
| purpura | 1 (5%) |
| flank pain | 1 (5%) |
| Duration of symptoms | 6 days (1–21) |
| Neurologic abnormalities: | |
| severe | 10 (45%) |
| mild | 3 (14%) |
| none | 9 (41%) |
| Renal function: | |
| acute renal failure | 1 (5%) |
| renal insufficiency | 8 (36%) |
| normal | 13 (59%) |
| Platelet count | 9000/μL (range, 4000–27,000/μL) |
| Hematocrit | 21% (range, 13%–30%) |
| LDH | 1431 U/L (range, 436–3909 U/L) |
| * Data are presented on 22 consecutive patients from the Oklahoma TTP-HUS Registry, November 13, 1995-December 31, 2003, who had a severe deficiency of ADAMTS13 activity at the time of their initial presentation. This experience represents an extension of our previously published data.3 The number of major presenting symptoms exceeds 22 because some patients had multiple major symptoms. The duration of symptoms describes the patient’s history of the initial onset of symptoms until diagnosis of TTP-HUS. Severe neurologic abnormalities included coma, stroke, seizure, or fluctuating focal signs; mild neurologic abnormalities included headache, blurred vision, ataxia, or mental status changes with transient confusion. Acute renal failure and renal insufficiency have been previously defined.3 Lactate dehydrogenase (LDH) values were adjusted to an upper limit of normal value of 200 U/L. Age, duration of symptoms, and laboratory data are median values. Laboratory data are the most abnormal values at the time of diagnosis of TTP-HUS ± 7 days, to account for transient effects of transfusion and potential worsening after diagnosis. | |
| Age | 39 years (range, 19–71) |
| Gender | 18 (82%) female |
| Race | 10 (45%) African-American |
| Obesity | 12 (55%) BMI ≥ 30 kg/m2 |
| Presenting symptoms: | |
| neurologic abnormalities | 13 (59%) |
| nausea, vomiting, diarrhea | 6 (27%) |
| abdominal pain | 6 (27%) |
| weakness | 4 (18%) |
| chest pain | 3 (14%) |
| hematuria | 2 (9%) |
| menorrhagia | 1 (5%) |
| purpura | 1 (5%) |
| flank pain | 1 (5%) |
| Duration of symptoms | 6 days (1–21) |
| Neurologic abnormalities: | |
| severe | 10 (45%) |
| mild | 3 (14%) |
| none | 9 (41%) |
| Renal function: | |
| acute renal failure | 1 (5%) |
| renal insufficiency | 8 (36%) |
| normal | 13 (59%) |
| Platelet count | 9000/μL (range, 4000–27,000/μL) |
| Hematocrit | 21% (range, 13%–30%) |
| LDH | 1431 U/L (range, 436–3909 U/L) |