Contributions
Abstract: PB2239
Type: Publication Only
Background
Thrombotic microangiopathies are a group of rare diseases characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia and involvement of organs of varying intensity, mainly renal and CNS damage. TTP and HUS are the most important forms of TMA and without adequate treatment administered early are associated with high morbidity and mortality.
Aims
To review our experience in the management of the primary TMA and to raise a series of questions that perhaps could improve the approach of these pathologies
Methods
We made a retrospective, descriptive analysis of ten cases diagnosed of primary thrombotic microangiopathy (TTP n=5; typical HUS n=3; atypical HUS n=2) over the last eight years, 70% of which were women with an average age between 40-60 years. Only three cases had previous records of autoinmune diseases (MTCD, RA and HIV), all of which would eventually develop TTP. We requested ADAMTS13 levels on all cases, they were low (< 5-10%) only in those pacients diagnosed with TTP, and on the other hand confirming the positivity for Shiga toxin in those patients who eventually developed typical HUS.
Results
Regardless of the diagnosis, 10-12 plasma exchanges were performed to improve the biological parameters of hemolysis, requiring the placement of a central catheter, most commonly at the right jugular vein (70%) due to the lower risk of thrombotic and infectious complications. Although renal involvement is frequent in HUS, only two of the patients required dialysis without recovery of baseline renal function. Is to be noted the part played by the aproval in 2011 of eculizumab and how patients eight years ago suffered a torpid course, requiring a greater number of plasmapheresis and the side effects this carries. However, we still do not know its repercussions out of indication. In our study, it was used in a patient with a diagnosis of HUS associated with an infection with good evolution, although perhaps this result is due only to the natural evolution of the disease. Another controversial point is the use of antibiotics, which are known to worsen the clinical course of these processes, but because of a concomitant infection or new positive determination of the Shiga toxin, as occurred with two of our patients diagnosed of HUS had to be used. Finally, 30% of the patients have relapsed after the first episode with a primary diagnosis of a TTP.
Conclusion
Thrombotic microangiopathies are a group of processes of enormous complexity, in addition to the low frequency with which they are usually present in our usual clinical practice requiring a large deployment of means to reach an early diagnosis and begin treatment as soon as possible given that the unfortunate prognosis of these patients.
With this study we have raised a series of questions to improve the management of this type of diseases:
Session topic: 30. Transfusion medicine
Keyword(s): TTP/HUS, ADAMTS13
Abstract: PB2239
Type: Publication Only
Background
Thrombotic microangiopathies are a group of rare diseases characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia and involvement of organs of varying intensity, mainly renal and CNS damage. TTP and HUS are the most important forms of TMA and without adequate treatment administered early are associated with high morbidity and mortality.
Aims
To review our experience in the management of the primary TMA and to raise a series of questions that perhaps could improve the approach of these pathologies
Methods
We made a retrospective, descriptive analysis of ten cases diagnosed of primary thrombotic microangiopathy (TTP n=5; typical HUS n=3; atypical HUS n=2) over the last eight years, 70% of which were women with an average age between 40-60 years. Only three cases had previous records of autoinmune diseases (MTCD, RA and HIV), all of which would eventually develop TTP. We requested ADAMTS13 levels on all cases, they were low (< 5-10%) only in those pacients diagnosed with TTP, and on the other hand confirming the positivity for Shiga toxin in those patients who eventually developed typical HUS.
Results
Regardless of the diagnosis, 10-12 plasma exchanges were performed to improve the biological parameters of hemolysis, requiring the placement of a central catheter, most commonly at the right jugular vein (70%) due to the lower risk of thrombotic and infectious complications. Although renal involvement is frequent in HUS, only two of the patients required dialysis without recovery of baseline renal function. Is to be noted the part played by the aproval in 2011 of eculizumab and how patients eight years ago suffered a torpid course, requiring a greater number of plasmapheresis and the side effects this carries. However, we still do not know its repercussions out of indication. In our study, it was used in a patient with a diagnosis of HUS associated with an infection with good evolution, although perhaps this result is due only to the natural evolution of the disease. Another controversial point is the use of antibiotics, which are known to worsen the clinical course of these processes, but because of a concomitant infection or new positive determination of the Shiga toxin, as occurred with two of our patients diagnosed of HUS had to be used. Finally, 30% of the patients have relapsed after the first episode with a primary diagnosis of a TTP.
Conclusion
Thrombotic microangiopathies are a group of processes of enormous complexity, in addition to the low frequency with which they are usually present in our usual clinical practice requiring a large deployment of means to reach an early diagnosis and begin treatment as soon as possible given that the unfortunate prognosis of these patients.
With this study we have raised a series of questions to improve the management of this type of diseases:
Session topic: 30. Transfusion medicine
Keyword(s): TTP/HUS, ADAMTS13