Abstract
Background: Thrombotic microangiopathy (TMA) is a syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, fever and renal dysfunction. Therapeutic Plasma Exchange (TPE) is a standart treatment approach for the patients. Our aim is to determine the clinical characteristics and outcome of patients with TMA treated with TPE in our center.
Patients and Methods: We retrospectively evaluated 46 patients who have been diagnosed as TMA at Ankara University Department of Hematology between 2007 and 2015. Patients were treated with TPE (Frensenius Kabi AG, Homburg, Germany) until the normalization of laboratory parameters. The plasma exchanged was 1.5 times the predicted plasma volume with fresh frozen plasma for the first procedure, and usually 1.0 time the predicted volume thereafter until remission.
Results: 22M/24F was included in the study with a median age of 55 (range, 18-8310 of 46 patients (22%) were consultated from intensive care units, 7/46 (15%) from emergency unit and 6/46 (13%) from nephrology unit. The most common presenting symptom was purpura in 52%, followed by neurological disturbance 48%, renal function abnormality in 43% and fever in 28% of patients. At diagnosis the median hemoglobin (g/dl), leucocyte count (10^9/L) and thrombocyte count (10^9/L) were as follows: 9.4, 11.4 and 58.6. Median time period of procedure was 99 minutes (range, 64-313). 5/46 (11%) patients had femoral catheters and central venous catheters were the access for the rest of patients. None of the patients had severe adverse events during procedures. 21 patients achieved complete response (46%) after 2-40 sessions and 2 of them were died during follow-up. Responders were diagnosed mostly with infectious related TMA. 21 patients (46%) who had progressive disease died within 30 days after diagnosis. 2 non-responder patients had diagnosis of TTP and treated with succesfully with Rituximab (Table 1).
Conclusions: TPE is safe treatement modality in patients with TMA however there is still high mortality rate.
Disclosures
No relevant conflicts of interest to declare.
Prospective registry of adult patients receiving therapeutic plasma exchange with a presumptive diagnosis of thrombotic microangiopathy (TMA): The Turkish hematology research and education group (ThREG)-TMA02 study
