Case Presentation: We present a case of a 68-year-old female with a history of CKD IV and chronic iron deficiency anemia who was admitted for symptoms of generalized weakness and found to be in hypovolemic shock from a suspected GI bleed. The hospital course was complicated by a left MCA stroke. During the initial workup, the patient was found to have newly diagnosed thrombocytopenia.On exam, she had a temperature of 97.9F, B/P of 90/67, heart rate of 156, respiratory rate of 16, and O2 saturation of 97% on room air. She was noted to have an irregularly irregular heart rate. On initial laboratory workup, she was found to have platelets of 26, Cr: 5.0 (baseline: 2.9) Hgb of 9.8 (at baseline), LDH of 1202, Haptoglobin of 95, Indirect Bilirubin of 0.8, INR of 1.4, and blood smear showing few schistocytes and some nucleated RBCs. She tested negative for HIV, Hepatitis B/C, and urine tox screen.During the hospital course, the patient’s platelets continued to drop down to 15k.On day 5 of hospital admission, the patient’s neurological status had not improved, and platelet levels could not be maintained in the normal range. She was initiated on plasmapheresis for suspected TTP. Soon after initiation, her platelet levels started to uptrend.On day 8, her ADAMTS13 resulted as < 5. The patient received 5 rounds of plasmapheresis, and platelets started to normalize to levels of 180k, with an initial uptrend in reticulocyte count which eventually normalized, as well as normalized LDH, while maintaining a normal haptoglobin level. Unfortunately, her neurological status did not see much improvement. She continued with steroid and folic acid therapy and was eventually discharged to a skilled nursing facility.

Discussion: Thrombotic thrombocytopenic purpura (TTP) is a hematological emergency that if left untreated, can reach a mortality of up to 90%.1 It has been described as a pentad of fever, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, renal failure, and neurological symptoms. However, < 10% of patients with TTP will have the complete pentad [1]. The diagnosis is typically confirmed by a deficiency in ADAMTS13 enzyme activity defined as < 10% [1]. However, treatment for TTP should begin promptly if there is a high suspicion, even before a diagnosis is confirmed. Recognition of TTP has remained difficult as it shares many features with other pathologies. In our patient, the diagnosis of TTP was not as evident for multiple reasons. Her increase in creatinine was thought to be due to pre-renal azotemia in the context of CKD with hypotension. Additionally, she had a long-standing history of iron deficiency anemia with a hemoglobin that was at baseline. Although her LDH and reticulocyte count was high, her indirect bilirubin was not impressively high. She had a normal haptoglobin level, with a blood smear showing only a few schistocytes. These findings were not striking for a MAHA in acute TTP. Her PLASMIC score was calculated to be 5, placing her at a 6% risk of severe ADAMTS13 deficiency [2]. However, since our patient was diagnosed with a left MCA stroke, plasmapheresis was initiated for suspected TTP, and surprisingly the patient responded favorably to it.

Conclusions: Our case highlights the importance of assessing symptoms, lab work, and scoring calculators in synergy to assess the probability of TTP so that treatment can be initiated promptly until a diagnosis can be confirmed by a decreased ADAMTS13 enzyme level.