Characteristics of the 11 patients who died following recovery from their initial episode of TTP
| Patient No. . | Date . | Age, y . | Expected age of death, y . | Clinical course, attributed cause of death . | |
|---|---|---|---|---|---|
| First TTP . | Death . | ||||
| 2 | 1996 | 2007 | 46 | 70 | SLE diagnosed during a TTP relapse 4 months after initial TTP. Continuous treatment with steroids and plaquenil for the following 11 years. Five hospitalizations for alcohol/drug abuse, pancreatitis, hypertension, diabetes. ADAMTS13 activity was 100% in 2004. Died in hospital: sepsis. |
| 3 | 1996 | 1998 | 59 | 80 | Sudden death during outpatient PEX to consolidate response for first relapse of TTP; platelet count 181 000/µL on day of death. Diabetes. Autopsy: acute myocardial infarction superimposed on previous infarction; no systemic microvascular thrombi. |
| 6 | 1997 | 2007 | 51 | 81 | Four relapses from 1997 to 2002. Liver cirrhosis with portal hypertension (biopsy, nonalcoholic steatohepatitis), liver transplant recommended. Hypertension, diabetes, chronic obstructive pulmonary disease (COPD). ADAMTS13 activity 100% 2 months before death. Died in nursing home. Death certificate: Cirrhosis of liver. |
| 7 | 1998 | 2001 | 51 | 78 | Two days after initial diagnosis of TTP, presenting clinical features attributed to streptococcal sepsis; PEX stopped. No relapse. Insulin-dependent diabetes since 1993 with episodes of ketoacidosis. Alcohol abuse. Died at home. Death certificate: Acute coronary insufficiency. |
| 9 | 1999 | 2009 | 82 | 86 | One relapse in 1999. Hypertension, Alzheimer’s disease, COPD on continuous nasal O2, myocardial infarction in 2008. ADAMTS13 activity 100% at 1 year before death. Died in nursing home. Death certificate: Cerebrovascular event. |
| 11 | 1999 | 2008 | 76 | 85 | Diabetes, hypertension, and heart failure. Died several years after admission to a nursing home. ADAMTS13 activity 100% 2 years before death. Death certificate: Cardiopulmonary failure. |
| 15 | 2000 | 2004 | 51 | 74 | No previous relapse. Hypertension. Acute myocardial infarction; coronary artery bypass grafting surgery; sudden death in hospital on sixth postop day (hematocrit 32%, platelet count 134 000/µL on second postop day; no subsequent laboratory data). ADAMTS13 14% (no inhibitor) 3 weeks before death. TTP not suspected until the day of death. Autopsy: systemic microvascular thrombi consistent with TTP. |
| 19 | 2000 | 2009 | 42 | 77 | Hypertension. Metastatic ovarian carcinoma, 2008; unresponsive to chemotherapy; ADAMTS13 11% (no inhibitor) 2006. Two relapses of TTP after diagnosis of metastatic carcinoma. Hospice care. Death certificate: Ovarian cancer. |
| 20 | 2001 | 2008 | 46 | 82 | Hypertension, diabetes. Ten hospitalizations for bipolar disorder with suicide risk during previous 20 years. ADAMTS13 activity <10% (no inhibitor) 6 months before death. Sudden death at home. Death certificate: Cardiac arrest. |
| 29 | 2003 | 2003 | 46 | 79 | Diagnosed with SLE in 1992. Sudden death at home. Death certificate: Cerebrovascular accident. |
| 34 | 2005 | 2006 | 41 | 82 | Diagnosed with SLE, scleroderma, 1994; jejunostomy for gastric paresis, 2002. Hospitalized for intestinal fistulas and recurrent sepsis for 13 of the 15 months she survived after her initial diagnosis of TTP. Died in hospital: intestinal hemorrhage. |
| Patient No. . | Date . | Age, y . | Expected age of death, y . | Clinical course, attributed cause of death . | |
|---|---|---|---|---|---|
| First TTP . | Death . | ||||
| 2 | 1996 | 2007 | 46 | 70 | SLE diagnosed during a TTP relapse 4 months after initial TTP. Continuous treatment with steroids and plaquenil for the following 11 years. Five hospitalizations for alcohol/drug abuse, pancreatitis, hypertension, diabetes. ADAMTS13 activity was 100% in 2004. Died in hospital: sepsis. |
| 3 | 1996 | 1998 | 59 | 80 | Sudden death during outpatient PEX to consolidate response for first relapse of TTP; platelet count 181 000/µL on day of death. Diabetes. Autopsy: acute myocardial infarction superimposed on previous infarction; no systemic microvascular thrombi. |
| 6 | 1997 | 2007 | 51 | 81 | Four relapses from 1997 to 2002. Liver cirrhosis with portal hypertension (biopsy, nonalcoholic steatohepatitis), liver transplant recommended. Hypertension, diabetes, chronic obstructive pulmonary disease (COPD). ADAMTS13 activity 100% 2 months before death. Died in nursing home. Death certificate: Cirrhosis of liver. |
| 7 | 1998 | 2001 | 51 | 78 | Two days after initial diagnosis of TTP, presenting clinical features attributed to streptococcal sepsis; PEX stopped. No relapse. Insulin-dependent diabetes since 1993 with episodes of ketoacidosis. Alcohol abuse. Died at home. Death certificate: Acute coronary insufficiency. |
| 9 | 1999 | 2009 | 82 | 86 | One relapse in 1999. Hypertension, Alzheimer’s disease, COPD on continuous nasal O2, myocardial infarction in 2008. ADAMTS13 activity 100% at 1 year before death. Died in nursing home. Death certificate: Cerebrovascular event. |
| 11 | 1999 | 2008 | 76 | 85 | Diabetes, hypertension, and heart failure. Died several years after admission to a nursing home. ADAMTS13 activity 100% 2 years before death. Death certificate: Cardiopulmonary failure. |
| 15 | 2000 | 2004 | 51 | 74 | No previous relapse. Hypertension. Acute myocardial infarction; coronary artery bypass grafting surgery; sudden death in hospital on sixth postop day (hematocrit 32%, platelet count 134 000/µL on second postop day; no subsequent laboratory data). ADAMTS13 14% (no inhibitor) 3 weeks before death. TTP not suspected until the day of death. Autopsy: systemic microvascular thrombi consistent with TTP. |
| 19 | 2000 | 2009 | 42 | 77 | Hypertension. Metastatic ovarian carcinoma, 2008; unresponsive to chemotherapy; ADAMTS13 11% (no inhibitor) 2006. Two relapses of TTP after diagnosis of metastatic carcinoma. Hospice care. Death certificate: Ovarian cancer. |
| 20 | 2001 | 2008 | 46 | 82 | Hypertension, diabetes. Ten hospitalizations for bipolar disorder with suicide risk during previous 20 years. ADAMTS13 activity <10% (no inhibitor) 6 months before death. Sudden death at home. Death certificate: Cardiac arrest. |
| 29 | 2003 | 2003 | 46 | 79 | Diagnosed with SLE in 1992. Sudden death at home. Death certificate: Cerebrovascular accident. |
| 34 | 2005 | 2006 | 41 | 82 | Diagnosed with SLE, scleroderma, 1994; jejunostomy for gastric paresis, 2002. Hospitalized for intestinal fistulas and recurrent sepsis for 13 of the 15 months she survived after her initial diagnosis of TTP. Died in hospital: intestinal hemorrhage. |
Individual data for each of the 11 patients who have died following recovery from their initial episode of TTP. Patients’ numbers represent the sequence of their enrollment in the Registry, as designated in supplemental Table S1. Median difference between the actual age at death and the expected age at death, calculated from the time of their initial diagnosis of TTP, was 30 years (range, 4 to 41 years).