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Various clinical manifestations in patients with thrombotic microangiopathy
Jae C Chang1, Satheesh K Kathula
1Department of Medicine, Wright State University School of Medicine, and Good Samaritan Hospital, Dayton, Ohio 45406, USA. jae.chang@wright.edu
Background:
Thrombotic microangiopathy (TM) is characterized by thrombocytopenia and microangiopathic hemolytic anemia in association with diffuse microthrombi in the arteriolar capillaries of various organs. Its clinical manifestation is protean, and a few well-defined clinical syndromes have been recognized. A clear understanding of the consequence of TM is needed to appreciate the unusual clinical syndromes due to atypical presentation of thrombotic thrombocytopenic purpura (TTP).
Methods:
The medical records of patients with known diagnoses of TTP, hemolytic uremic syndrome (HUS), and the syndrome in which hemolysis, elevated liver enzymes, and low platelet count are found in association with pregnancy were examined retrospectively from 1981 to 1994 and prospectively from 1995 to 2000. Various thrombotic microangiopathic presentations were identified in these patients. Their response to exchange plasmapheresis was evaluated, and their clinical outcome was determined.
Results:
A total of 74 patients were diagnosed with TM. Among these patients, several well-defined thrombotic microangiopathic presentations were identified. These presentations included TTP in 57 patients, acute respiratory distress syndrome (ARDS) in 13 patients, HUS in 9 patients, the syndrome in which hemolysis, elevated liver enzymes, and low platelet count are found in association with pregnancy in 9 patients, peripheral digit ischemic syndrome (PDIS) in 6 patients, pancreatitis in 3 patients, hepatitis in 3 patients, and nonocclusive mesenteric ischemia (NOMI) in 2 patients. Exchange plasmapheresis was an effective treatment, with a response rate of 79%. A poor prognosis was evident when ARDS was present, with an overall survival rate of 46%.
Conclusion:
Traditionally, TTP and HUS are considered the main entities of TM. It is evident that other manifestations of TM, if unrecognized in a timely fashion, can lead to fatality. The understanding of the pathophysiologic consequences of TM and the recognition of its atypical presentations are essential to achieve favorable outcomes in patients with this life-threatening disease.
Insights
Thrombotic microangiopathy (TM) presents with diverse syndromes beyond TTP and HUS. Early recognition of atypical TM manifestations is crucial for effective treatment and improved patient outcomes.
Area of Science:
- Hematology
- Internal Medicine
- Critical Care Medicine
Background:
- Thrombotic microangiopathy (TM) encompasses conditions like TTP and HUS, characterized by microthrombi and organ damage.
- TM presents with varied clinical manifestations, necessitating a broader understanding beyond typical TTP and HUS diagnoses.
Purpose of the Study:
- To identify and characterize diverse clinical presentations of thrombotic microangiopathy (TM).
- To evaluate the efficacy of exchange plasmapheresis and determine prognostic factors in TM patients.
Main Methods:
- Retrospective and prospective review of medical records for patients diagnosed with TM, TTP, HUS, and pregnancy-associated TM.
- Analysis of treatment response to exchange plasmapheresis and patient outcomes.
Main Results:
- 74 patients diagnosed with TM, including TTP (57), ARDS (13), HUS (9), pregnancy-associated TM (9), PDIS (6), pancreatitis (3), hepatitis (3), and NOMI (2).
- Exchange plasmapheresis showed a 79% response rate.
- Acute respiratory distress syndrome (ARDS) was associated with a poor prognosis (46% survival).
Conclusions:
- TM encompasses a spectrum of conditions beyond TTP and HUS, including ARDS, PDIS, and NOMI.
- Timely recognition and management of atypical TM presentations are vital for improving patient survival.
- Understanding TM pathophysiology is key to managing this life-threatening disease effectively.