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5-2019 Differentiating TTP from Hypertensive Hemolytic Anemia Chelsea Dixon-Dionne Henry Ford Health System

Robert Curtis Henry Ford Health System

Adele Amine Henry Ford Health System

Joseph Abbo Henry Ford Health System

Rajika Munasinghe Henry Ford Health System

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Recommended Citation Dixon-Dionne, Chelsea; Curtis, Robert; Amine, Adele; Abbo, Joseph; and Munasinghe, Rajika, "Differentiating TTP from Hypertensive Hemolytic Anemia" (2019). Case Reports. 99. https://scholarlycommons.henryford.com/merf2019caserpt/99

This Poster is brought to you for free and open access by the Medical Education Research Forum 2019 at Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Case Reports by an authorized administrator of Henry Ford Health System Scholarly Commons. For more information, please contact [email protected]. Differentiating Hypertensive Hemolytic Anemia from Thrombotic Thrombocytopenic Purpura

Chelsea Dixon-Dionne, D.O., Robert Curtis, D.O., Adele Amine, D.O., Joseph Abbo, M.D., Eva Saha, M.D., Rajika Munasinghe, M.D.

Introduction Discussion

It is established that Focal Segmental Glomerulosclerosis (FSGS) can lead to This patient’s presentation was concerning for TTP due to severe difficult to control hypertension. It has also been shown that malignant thrombocytopenia and MAHA. Given the prolonged turnaround time for the hypertension can cause microangiopathic hemolytic anemia (MAHA). ADAMTS13 assay, he was empirically treated for TTP with PEX. However, in Distinguishing MAHA due to malignant hypertension from other causes of MAHA hindsight we believe this patient developed FSGS with subsequent renal such as Thrombotic Thrombocytopenic Purpura (TTP) or Hemolytic Uremic hypertension that went undiagnosed and untreated for an unknown period of Syndrome (HUS) can be difficult. We present an interesting case of a young patient time, leading to his presentation of MAHA secondary to hypertensive crisis. who we believe developed undiagnosed FSGS that led to hypertensive crisis, acute renal failure, and MAHA. It is difficult to discern TTP from other causes of MAHA. One helpful tool is called the PLASMIC score which estimates the likelihood of severe ADAMTS13 deficiency in adults with possible TTP.

Case Description The average turnaround time for ADAMTS13 is prolonged. Our patient’s assay did not result for >2 weeks. A study at Brigham and Women’s Hospital in Our patient is a 19 year old male with no past medical history who presented to the 2016 with rapid in-house assay reduced number of plasma exchanges for emergency department with nausea, vomiting, subjective fever, and fatigue for roughly suspected TTP by more than half without an increase in mortality. A second two weeks. He stated that he had been sleeping most of the day, had been unable to study in 2017 demonstrated that a rapid assay for ADAMTS13 was Pictured above: our patient’s peripheral blood smear. Two examples of schistocytes keep anything down, and was unable to attend work. He described feeling feverish at associated with reduced mortality for patient’s diagnosed with TTP. some point, but did not take his temperature. are highlighted with blue arrows. Up to 12/hpf were noted in pathology report. Social history was positive for social marijuana use (last use was >1 week prior), social Official pathology report: Microangiopathic hemolytic anemia is suspected in view of marked schistocytosis, thrombocytopenia and anemia with polychromasia. Among An article published in 2015 analyzed 19 cases of hypertension induced alcohol use. He works part time in the warehouse of a grocery store. MAHA via literature review. About 1/3 of the patients underwent PLEX while Family history was remarkable for early death in his mother, in her mid-30’s. Cause of other causes for presence of schistocytes, hypertensive crisis or mechanical death was unknown, possibly related to an overdose. The patient has been under the hemolysis due to artificial heart valves is to be ruled out. awaiting ADAMTS13 results. At the time of presentation, all had significant care of his aunt ever since. hypertension with MAPs ranging from 123-190 and prominent renal He had never had surgery, no allergies, and took no daily medications prior to arrival. dysfunction, with milder thrombocytopenia.

Vitals: BP 229/142, HR 92, RR 18, 100% RA, T 98.9oF FSGS HTN MAHA As the mortality rate of Thrombotic Thrombocytopenic Purpura approaches The patient was markedly hypertensive on presentation. Physical exam was significant 90% when not treated with plasma exchange, it is understandable that for scleral icterus and the patient was slow to respond to questions, with an otherwise providers err on the side of caution when choosing whom to treat. Until there intact neurological exam. Initial blood work revealed renal failure, anemia, and is a readily available rapid in-house assay for ADAMTS13, the decision to thrombocytopenia. Examination of the peripheral smear demonstrated marked initiate empiric treatment for TTP will rely on clinical judgment with perhaps schistocytosis. some assistance from the PLASMIC scoring system. LDH 1,093 Microangiopathic Hemolytic Anemia (MAHA) - descriptive term for non-immune ALP 75 AST 48 hemolysis, red blood cell fragmentation that takes place inside the vasculature. Alb 4.7 ALT 25 LA 1.7 T bili 2.1 Thrombotic Microangiopathy (TMA) - microvascular thrombosis caused by D bili 0.4 abnormalities in the vessel walls of capillaries and arterioles. Diagnosis made by UA: >500 protein INR – 1.11 tissue biopsy. References Coombs – negative MCV – 80.5 Haptoglobin <30.0

The patient was started on nicardipine infusion for hypertensive emergency and was PLASMIC Score Bendapudi, P. K., Hurwitz, S., Fry, A., Marques, M. B., Waldo, S. W., Li, A., . . . Makar, R. S. (2017). Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: A cohort study. The Lancet Haematology,4(4). doi:10.1016/ empirically treated for TTP with emergent plasma exchange (PEX). He underwent 3 s2352-3026(17)30026-1 sessions of PEX, until platelets were above 100K. He was started on oral therapy with PLASMIC score Risk of Severe A high PLASMIC score (6-7) has a Connell, N. T., Cheves, T., & Sweeney, J. D. (2015). Effect of ADAMTS13 activity turnaround time on plasma utilization for suspected thrombotic hydralazine, metoprolol, and nicardipine and was able to be weaned off of the sensitivity ~91%. A low (0-4) score has thrombocytopenic purpura. Transfusion,56(2), 354-359. doi:10.1111/trf.13359 (points) ADAMTS13 deficiency George, J. N. (2015). Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: When, how, and why? Transfusion, nicardipine drip after 4 days. His creatinine remained 6.5+ throughout his admission. He a specificity of 99%. The PLASMIC 0-4 Low score was applied to 108 patients with 55(1), 11-13. doi:10.1111/trf.12885 underwent renal biopsy that demonstrated changes consistent with focal segmental George, J. N. (2017). The importance of clinical judgment for the diagnosis of thrombotic thrombocytopenic purpura. Transfusion,57(11), 2558-2561. doi: suspected TMA. The system had a glomerulosclerosis. ADAMTS13 resulted two weeks after his presentation and was within 10.1111/trf.14357 5 Intermediate positive predictive value of 72%, and a Kim, C. H., Simmons, S. C., Iii, L. A., Staley, E. M., Zheng, X. L., & Pham, H. P. (2017). ADAMTS13 test and/or PLASMIC clinical score in management of normal limits. acquired thrombotic thrombocytopenic purpura: A cost-effective analysis. Transfusion,57(11), 2609-2618. doi:10.1111/trf.14230 6-7 high negative predictive value of 99%. Our patient’s PLASMIC score was 5. Shibagaki, Y., & Fujita, T. (2005). Thrombotic Microangiopathy in Malignant Hypertension and Hemolytic Uremic Syndrome (HUS)/Thrombotic The patient was discharged home on: hydralazine, metoprolol tartrate, nifedipine and Thrombocytopenic Purpura (TTP): Can We Differentiate One from the Other? Hypertension Research,28(1), 89-95. doi:10.1291/hypres.28.89 1 point for each: Vesely, S. K. (2003). ADAMTS13 activity in thrombotic thrombocytopenic purpura--hemolytic uremic syndrome: Relation to presenting features and clinical calcium acetate. He has been following with nephrology as an outpatient, though has  No history of solid organ or hematopoietic stem  Platelet count <30,000/microL outcomes in a prospective cohort of 142 patients. Blood,102(1), 60-68. doi:10.1182/blood-2003-01-0193 cell transplant Yoshii, Y., Fujimura, Y., Bennett, C. L., Isonishi, A., Kurumatani, N., & Matsumoto, M. (2017). Implementation of a rapid assay of ADAMTS13 activity was missed his last several appointments.  One or more of the following:  MCV <90 fl associated with improved 30-day survival rate in patients with acquired primary thrombotic thrombocytopenic purpura who received platelet reticulocyte count >2.5%, haptoglobin  INR <1.5 transfusions. Transfusion,57(8), 2045-2053. doi:10.1111/trf.14152 undetectable, or indirect bilirubin >2.0 mg/dL  Creatinine <2.0mg/dL  No active cancer in the preceding year