Diversity of initial presentations
| Patient No. . | Age, y . | Race . | Sex . | Date . | Summary . | Patient’s presentation, clinical course, and outcome . |
|---|---|---|---|---|---|---|
| 1 | 20 | W | F | 2016 | Asymptomatic, normal physical examination, thrombocytopenia discovered by a routine CBC | Excellent health. Initial prenatal visit for first pregnancy, gestational age 7 w. CBC: platelets, 16 × 109/L; hematocrit, 24%. Laboratory error was assumed, so the tests were repeated twice during the next 3 d; the patients was then admitted. No symptoms, normal examination, no petechiae or purpura. Cr, 0.9 mg/dL; LDH, 813 U/L; ADAMTS13, 7%; inh, 3.4 BU. |
| 2 | 37 | AA | F | 2005 | Minor symptoms, sudden death | Excellent health except for intermittent menorrhagia. Headache and abdominal pain for 2-3 w. Vomiting for 2 d with blood streaks. At the hospital emergency department: platelets, 12 × 109/L; hematocrit, 27%; Cr, 1.1 mg/dL; LDH, 722 U/L. Diagnosis: ITP, GI, and/or menstrual bleeding. Treated with corticosteroids. Hospital day 4: DAT, negative; haptoglobin, <15 mg/dL. TTP suspected, PEX begun, and then sudden death. ADAMTS13, <5%, <5%; inh, >2 BU. |
| 3 | 41 | AA | M | 1998 | Characteristic clinical features of TTP, normal ADAMTS13 activity | Nausea, vomiting, syncope, no fever. Platelets, 7 × 109/L; hematocrit, 28%; Cr, 1.2 mg/dL; LDH, 1730 U/L. Sepsis suspected. Three days later, transient numbness occurred in the left face and left arm, left face weakness, aphasia. Diagnosis: TTP; resolved with PEX. ADAMTS13, 53%, 60%. Four relapses. Fourth relapse, ADAMTS13, <5%, <5%; inh, 1.4 BU. |
| 4 | 55 | W | F | 2008 | Endocarditis | Chest pain, myocardial infarction. Subsequent hemiplegia, aphasia. Platelets, 33 × 109/L; hematocrit, 33%; Cr, 1.6 mg/dL; LDH, 1141 U/L. Echocardiogram: aortic valve vegetation. Blood cultures: Enterococcus faecium. Diagnosis: endocarditis and TTP. PEX begun. ADAMTS13, <5%, <5%; inh, 0 (obtained at admission; results reported 4 mo later). 2009: ADAMTS13, 92%, 100%. 2016: relapse, ADAMTS13, <5%; inh, 1.2 BU. |
| Patient No. . | Age, y . | Race . | Sex . | Date . | Summary . | Patient’s presentation, clinical course, and outcome . |
|---|---|---|---|---|---|---|
| 1 | 20 | W | F | 2016 | Asymptomatic, normal physical examination, thrombocytopenia discovered by a routine CBC | Excellent health. Initial prenatal visit for first pregnancy, gestational age 7 w. CBC: platelets, 16 × 109/L; hematocrit, 24%. Laboratory error was assumed, so the tests were repeated twice during the next 3 d; the patients was then admitted. No symptoms, normal examination, no petechiae or purpura. Cr, 0.9 mg/dL; LDH, 813 U/L; ADAMTS13, 7%; inh, 3.4 BU. |
| 2 | 37 | AA | F | 2005 | Minor symptoms, sudden death | Excellent health except for intermittent menorrhagia. Headache and abdominal pain for 2-3 w. Vomiting for 2 d with blood streaks. At the hospital emergency department: platelets, 12 × 109/L; hematocrit, 27%; Cr, 1.1 mg/dL; LDH, 722 U/L. Diagnosis: ITP, GI, and/or menstrual bleeding. Treated with corticosteroids. Hospital day 4: DAT, negative; haptoglobin, <15 mg/dL. TTP suspected, PEX begun, and then sudden death. ADAMTS13, <5%, <5%; inh, >2 BU. |
| 3 | 41 | AA | M | 1998 | Characteristic clinical features of TTP, normal ADAMTS13 activity | Nausea, vomiting, syncope, no fever. Platelets, 7 × 109/L; hematocrit, 28%; Cr, 1.2 mg/dL; LDH, 1730 U/L. Sepsis suspected. Three days later, transient numbness occurred in the left face and left arm, left face weakness, aphasia. Diagnosis: TTP; resolved with PEX. ADAMTS13, 53%, 60%. Four relapses. Fourth relapse, ADAMTS13, <5%, <5%; inh, 1.4 BU. |
| 4 | 55 | W | F | 2008 | Endocarditis | Chest pain, myocardial infarction. Subsequent hemiplegia, aphasia. Platelets, 33 × 109/L; hematocrit, 33%; Cr, 1.6 mg/dL; LDH, 1141 U/L. Echocardiogram: aortic valve vegetation. Blood cultures: Enterococcus faecium. Diagnosis: endocarditis and TTP. PEX begun. ADAMTS13, <5%, <5%; inh, 0 (obtained at admission; results reported 4 mo later). 2009: ADAMTS13, 92%, 100%. 2016: relapse, ADAMTS13, <5%; inh, 1.2 BU. |
When 1 result for ADAMTS13 activity is reported, it is with FRET; when 2 results are reported, the first is with FRET, and the second is with IB. Date indicates the year of the patient's initial presentation with TTP. Summary represents key point for including the patient's story in this review.
AA, African American; Cr, serum creatinine; DAT, direct antiglobulin test; F, female; GI, gastrointestinal; inh, functional inhibitor; ITP, immune thrombocytopenic purpura; LDH, lactate dehydrogenase; M, male; W, white.