Clinical features and causes of death in patients with ADAMTS13 activity < 10%
| Patient . | ADAMTS13 activity, inhibitor . | Day . | Clinical course . |
|---|---|---|---|
| Death attributed to TTP–thrombotic | |||
| 1 | < 5% (F, IB), >> 2 BU | 4 | 30-year-old BM, no response to 3 PEX and corticosteroids, TTP documented by autopsy |
| 2 | < 5% (F, IB), >> 2 BU | 17 | 51-year-old WF, response to 7 PEX, died 10 days after PEX stopped from pulmonary emboli31 |
| 3 | 23% (F), 7% (IB), no inhibitor | 8 | 68-year-old WF, cardiac arrest with coma 4 days after cardiac surgery. Platelet count response to 5 PEX, then DNR; died 3 days after PEX stopped. |
| 4 | < 5% (F), 18% (IB), inhibitor not tested | 1 | 50-year-old WF, died before PEX begun,31 TTP documented by autopsy |
| 5 | < 5% (F, IB), > 2 BU | 1 | 37-year-old BF, died after 1st PEX,21 TTP documented by autopsy |
| 6 | 7% (F), < 5% (IB), >> 2 BU | 72 | 70-year-old WF, 4 responses to 45 PEX, corticosteroids, rituximab; none sustained, then DNR. Died 10 days after PEX stopped. |
| Death attributed to TTP–PEX complications | |||
| 7 | < 5% (F, IB), >> 2 BU | 16 | 41-year-old BF, urine cocaine +, comatose, no response to 12 PEX and corticosteroids, death from sepsis attributed to CVC31,32 |
| 8 | < 5% (F, IB), > 2 BU | 16 | 25-year-old WF, response to 14 PEX, exacerbation required new CVC, death from hemorrhage caused by CVC insertion31,32 |
| 9 | < 5% (F, IB), >> 2 BU | 26 | 68-year-old WF, response to PEX then apparent exacerbation, death from sepsis attributed to CVC33 |
| Death not attributed to TTP | |||
| 10 | 9% (F), 20% (IB), 1.3 BU | 6 | 39-year-old WF, hepatitis A, hepatic and renal failure, confusion (bilirubin 24 mg/dL). No response to 3 PEX. Autopsy: hepatic necrosis, no evidence of TTP. |
| 11 | 28% (F), 8% (IB), 1.2 BU | 21 | 65-year-old WF, hypotension, confusion, Candida pneumonia after liver transplantation. Fibrinogen 53 mg/dL. No response to 7 PEX. Death attributed to sepsis. |
| 12 | 9% (F), 25% (IB), > 2 BU | 34 | 43-year-old WF, relapsed AML after HSCT. Acute GVHD (bilirubin 64 mg/dL), Aspergillus pneumonia. No response to 18 PEX. Death attributed to sepsis and GVHD. |
| 13 | 6% (F), 13% (IB), 0.9 BU | 11 | 45-year-old WM, HIV infection, pneumonia. No response to 5 PEX. Autopsy: disseminated Kaposi sarcoma, no evidence of TTP.29 |
| Patient . | ADAMTS13 activity, inhibitor . | Day . | Clinical course . |
|---|---|---|---|
| Death attributed to TTP–thrombotic | |||
| 1 | < 5% (F, IB), >> 2 BU | 4 | 30-year-old BM, no response to 3 PEX and corticosteroids, TTP documented by autopsy |
| 2 | < 5% (F, IB), >> 2 BU | 17 | 51-year-old WF, response to 7 PEX, died 10 days after PEX stopped from pulmonary emboli31 |
| 3 | 23% (F), 7% (IB), no inhibitor | 8 | 68-year-old WF, cardiac arrest with coma 4 days after cardiac surgery. Platelet count response to 5 PEX, then DNR; died 3 days after PEX stopped. |
| 4 | < 5% (F), 18% (IB), inhibitor not tested | 1 | 50-year-old WF, died before PEX begun,31 TTP documented by autopsy |
| 5 | < 5% (F, IB), > 2 BU | 1 | 37-year-old BF, died after 1st PEX,21 TTP documented by autopsy |
| 6 | 7% (F), < 5% (IB), >> 2 BU | 72 | 70-year-old WF, 4 responses to 45 PEX, corticosteroids, rituximab; none sustained, then DNR. Died 10 days after PEX stopped. |
| Death attributed to TTP–PEX complications | |||
| 7 | < 5% (F, IB), >> 2 BU | 16 | 41-year-old BF, urine cocaine +, comatose, no response to 12 PEX and corticosteroids, death from sepsis attributed to CVC31,32 |
| 8 | < 5% (F, IB), > 2 BU | 16 | 25-year-old WF, response to 14 PEX, exacerbation required new CVC, death from hemorrhage caused by CVC insertion31,32 |
| 9 | < 5% (F, IB), >> 2 BU | 26 | 68-year-old WF, response to PEX then apparent exacerbation, death from sepsis attributed to CVC33 |
| Death not attributed to TTP | |||
| 10 | 9% (F), 20% (IB), 1.3 BU | 6 | 39-year-old WF, hepatitis A, hepatic and renal failure, confusion (bilirubin 24 mg/dL). No response to 3 PEX. Autopsy: hepatic necrosis, no evidence of TTP. |
| 11 | 28% (F), 8% (IB), 1.2 BU | 21 | 65-year-old WF, hypotension, confusion, Candida pneumonia after liver transplantation. Fibrinogen 53 mg/dL. No response to 7 PEX. Death attributed to sepsis. |
| 12 | 9% (F), 25% (IB), > 2 BU | 34 | 43-year-old WF, relapsed AML after HSCT. Acute GVHD (bilirubin 64 mg/dL), Aspergillus pneumonia. No response to 18 PEX. Death attributed to sepsis and GVHD. |
| 13 | 6% (F), 13% (IB), 0.9 BU | 11 | 45-year-old WM, HIV infection, pneumonia. No response to 5 PEX. Autopsy: disseminated Kaposi sarcoma, no evidence of TTP.29 |
Clinical features and causes of death of the 13 patients with ADAMTS13 < 10% who died. ADAMTS13 activity was determined by the FRETS-VWF73 (F) and immunoblotting (IB) assays. Day indicates day of death; the day of the first plasma exchange (or day of diagnosis in patient 4) was designated as day 1. Citations are provided when patients have been previously described.
ARDS indicates acute respiratory distress syndrome; AML, acute myeloid leukemia; CVC, central venous catheter; DNR, do not resuscitate; GVHD, graft-versus-host disease; HSCT, hematopoietic stem cell transplantation; F, FRETS-VWF73 assay; IB, immunoblotting assay; BU, Bethesda units/mL; PEX, plasma exchange; TTP, thrombotic thrombocytopenic purpura; BF, black female; BM, black male; WF, white female; and WM, white male.