Case Presentation:
A 43-year-old female Jehovah’s Witness was admitted for 30 minutes of blurred vision, dysarthria, and left upper extremity weakness. She had two similar episodes in the past 2-3 weeks, each transient with full recovery. Associated symptoms included bi-frontal and occipital headache. Three weeks prior to admission, the patient had profuse watery diarrhea with fever to 101.6 degrees F that self-resolved.
On presentation, her physical exam was notable for multiple bruises in different stages of healing. Neuro exam was notably normal, and brain CT negative. Laboratory data were significant for Platelets 15,000/L, Hemoglobin 6.7 g/dL, Lactate dehydrogenase 2019 U/L, total bilirubin 5.9 mg/dL, d-dimer 21,456 ng/mL, and Creatinine 1.5 mg/dL (baseline 1.0mg/dL). Stool cultures were negative. Blood film demonstrated 4-7 schistocytes per high-powered field. ADAMSTS 13 activity level resulted as <5%, confirming the diagnosis of thrombotic thrombocytopenic purpura (TTP).
While the presentation of TTP was classic in this case, treatment decisions were complicated by the need to consider the patient’s strong religious preference not to get blood products. Prednisone 1mg/kg/day was started. After extensive discussion, patient declined traditional plasmapheresis but was amenable to apheresis using cryoprecipitate-poor plasma. In addition, folic acid 1mg/day and epoetin 10,000U were initiated on hospital day 2 due to inability to use pRBC transfusion. Laboratory values improved steadily and the patient was discharged on hospital day 9.
Discussion:
This case elegantly demonstrates the classic, but rarely seen, pentad associated with TTP: neurologic symptoms, thrombocytopenia, renal pathology, microangiopathic hemolytic anemia and fever. Cryo-precipitate poor plasma is an alternative to fresh frozen plasma (FFP) in the traditional treatment of TTP. It is made from the supernatant of a unit of thawed FFP, and therefore is deficient in factors VIII, XIII, fibronectin, fibrinogen, and vWF. Theoretically, using such a media could be useful as it is deficient in vWF, the very factor that is dysfunctional in TTP. However, multiple studies, including prospective randomized studies, have shown no clinical benefit as compared to traditional plasmapheresis with fresh frozen plasma. The power of these studies is significantly impaired by small sample sizes. However, if a patient is absolutely refusing fresh frozen plasma, as in this case, crypo-precipitate poor plasma is a valuable treatment option.
Conclusions:
A high degree of clinical suspicion is required for TTP in patients presenting with neurological findings and thrombocytopenia. The management of TTP requires specialized care in certain populations and needs to account for individual patient preferences. Alternatives to traditional plasmapheresis exist, particularly in the use of Cryoprecipitate-poor formula, though the clinical benefit is not well described compared to traditional therapy.