(P-TA-5) Importance of infectious disease testing prior to planned large volume exchanges: A case report of thrombotic thrombocytopenic purpura secondary to unknown infection with human immunodeficiency virus.
Indiana University School of Medicine Indianapolis, Indiana, United States
Background/Case Studies: Thrombotic thrombocytopenic purpura (TTP), a deficiency of ADAMS13,is a microangiopathic hemolytic anemia with thrombocytopenia that can be congenital or acquired. AcquiredTTPcan be preceded by various scenarios including viral infection, particularly human immunodeficiency virus (HIV).
Study
Design/Methods: Hospital electronic medical records and a state-based health informationexchange wereutilized to review hospital records. Laboratory values and patientimmunohematologic testing was performed at a hospital-based laboratory. Donor lookback testing was performed at respective donor centers.
Results/Findings: Heron, we report a case of a male in the third decade of life presenting with mucosal bleeding for one week with associated orthostatic tachycardia and presyncope. Review of systems elucidatedintermittent chest pain unrelated to exertion, extremity bruising, left upper abdominal pain, jaundice, brown urine, and subjective fever and chills. The patient reports no chronic healthconditions nor any medications. The patient had no history of prior transfusions or intravenous drug use.The patient had one sexual partner in which barrier protection was not routinely used. Laboratory values werenotable for a platelet count of13k/cumm and a PLASMIC score of 7/7. The patient wasinitiatedon steroids and rituximab with near daily plasmapheresis procedures. The patient’s plateletcount moderately improved to slightly below normal reference range but did not further respond to treatment. Infectious disease testing was performed to explore confounding therapy response. The patient tested positive for HIV-1 on confirmatory testing. Antivirals wereinitiatedand the patient’s platelet count increased to normal values. During the patient’s hospital stay, he received 11 plasma exchanges prior to diagnosis(approximately161 unitsof plasma) and twounits of packed red blood cells at an outside hospital prior to admission.No HIV testing was performed on the patient prior, and lookback was performed on the units the patient received to rule out HIV associated transfusion. Donor units weresubsequentlynegative on testing. Conclusions: This case elucidates the importance of considering an underlying cause including infectious disease in a suspected case of TTP. The initiation of plasmapheresis should not be delayed given the risk of disease; however additional samples drawn at the start of treatment can be vital to diagnosing an underlying infection or autoimmune disorder. Given the presentation, these samples will not only rule out HIV and other infection associated microangiopathic anemia, but also to determine what other therapy modalities may be warranted. In the case of this patient, infectious disease testing can also mitigate the impact of time and resources utilizedby a lookback.
