Contributions
Abstract: PB2516
Type: Publication Only
Background
Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) without an obvious cause, and may include fever, mild renal failure, and neurologic deficits.
Refractory TTP defined by some authors as any one of the following:
1-TTP for which treatment with plasma exchange (PEX) and glucocorticoids fails to produce a satisfactory response.
2-new neurologic abnormalities during PEX that are not attributable to another cause such as bleeding or infection.
3- Exacerbation of symptoms or laboratory findings occurs during PEX or within the first 30 days of stopping PEX.
Aims
To show outcome of two difficult cases of refractory TTP to daily PEX plus rituximab twice weekly.
Methods
The first case is a 32 years old male patient, known case of ulcerative colitis, the second case is a 26 years old male healthy before both, diagnosed him with of TTP based on clinical and laboratory findings and started on PEX and prednisolone.They were not responding initially and their hospital stay complicated with renal failure, ARDS and seizures so both patients intubated and admitted in intensive care unit. We added rituximab to the treatment, and as desperate measures we made rituximab twice weekly. After several weeks improve gradually. Both of them were extubated, and later sent to the medical word for further rehabilitation.
Results
The pathophysiology is lack of protease which cleavage von willbrand large multimeric. The protease called ADAMT- 13 and it could be absent congenitally or acquired.
Severe deficiency of ADAMT-13 less than 5 % specific for idiopathic TTP.
The established management is plasma exchange (PE) along with steroids 80% of patients to survive, however, the result is still there are refractory and relapse cases
There is increase of usage of Rituximab as salvage therapy in cases of TTP.
Mischelle et al, reported four cases and systemic review of literature. The found patient who received Rituximab in relapse or refractory, their median age is 42, most were females 78 %, median doses 4, given once weekly and dose was 375mg /m2. Complete remission achieved in 95 % within 11- 35 days.
According to our knowledge rituximab twice weekly agents was never used before. Our two patients were refectory to PE although it was going on daily for more than 2-3 weeks along with steroids.
Conclusion
TTP is a serious hematological malignancy with considerable amount of refractory cases. Rituximab addition to PE and steroids is good strategy. We suggest it can be used twice weekly safely, although further studies needed to proof efficacy Young patients and healthy deserves all extraordinary measures, which include not only rituximab but continue PE daily with giving up on patient.
Session topic: 35. Thrombosis and vascular biology & translational Research
Abstract: PB2516
Type: Publication Only
Background
Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) without an obvious cause, and may include fever, mild renal failure, and neurologic deficits.
Refractory TTP defined by some authors as any one of the following:
1-TTP for which treatment with plasma exchange (PEX) and glucocorticoids fails to produce a satisfactory response.
2-new neurologic abnormalities during PEX that are not attributable to another cause such as bleeding or infection.
3- Exacerbation of symptoms or laboratory findings occurs during PEX or within the first 30 days of stopping PEX.
Aims
To show outcome of two difficult cases of refractory TTP to daily PEX plus rituximab twice weekly.
Methods
The first case is a 32 years old male patient, known case of ulcerative colitis, the second case is a 26 years old male healthy before both, diagnosed him with of TTP based on clinical and laboratory findings and started on PEX and prednisolone.They were not responding initially and their hospital stay complicated with renal failure, ARDS and seizures so both patients intubated and admitted in intensive care unit. We added rituximab to the treatment, and as desperate measures we made rituximab twice weekly. After several weeks improve gradually. Both of them were extubated, and later sent to the medical word for further rehabilitation.
Results
The pathophysiology is lack of protease which cleavage von willbrand large multimeric. The protease called ADAMT- 13 and it could be absent congenitally or acquired.
Severe deficiency of ADAMT-13 less than 5 % specific for idiopathic TTP.
The established management is plasma exchange (PE) along with steroids 80% of patients to survive, however, the result is still there are refractory and relapse cases
There is increase of usage of Rituximab as salvage therapy in cases of TTP.
Mischelle et al, reported four cases and systemic review of literature. The found patient who received Rituximab in relapse or refractory, their median age is 42, most were females 78 %, median doses 4, given once weekly and dose was 375mg /m2. Complete remission achieved in 95 % within 11- 35 days.
According to our knowledge rituximab twice weekly agents was never used before. Our two patients were refectory to PE although it was going on daily for more than 2-3 weeks along with steroids.
Conclusion
TTP is a serious hematological malignancy with considerable amount of refractory cases. Rituximab addition to PE and steroids is good strategy. We suggest it can be used twice weekly safely, although further studies needed to proof efficacy Young patients and healthy deserves all extraordinary measures, which include not only rituximab but continue PE daily with giving up on patient.
Session topic: 35. Thrombosis and vascular biology & translational Research