Diagnostic dilemma in a patient presenting with thrombotic microangiopathy in the setting of pregnancy

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Published Mar 30, 2022
DOI: https://doi.org/10.22551/2022.34.0901.10199
Mohamed Zakee Mohamed Jiffry Mohammad Aimal Ahmed-khan Felipe Carmona Pires Nkechi Okam Mahnoor Hanif

Abstract

We report a case of thrombotic microangiopathy in a postpartum female for which considerable diagnostic uncertainty existed initially regarding the etiology. This case highlights the limitations surrounding PLASMIC scoring criteria for the diagnosis of thrombotic thrombocytopenic purpura (TTP). A 32-year-old woman presented to maternofetal medicine in her third trimester of pregnancy at 32 weeks for a routine follow up and was subsequently found to have elevated blood pressures with proteinuria, and was diagnosed with pre-eclampsia. Worsening anemia and thrombocytopenia prompted a blood smear which showed schistocytes, concerning for a thrombotic microangiopathy. Creatinine was also elevated with normal liver enzymes being noted. A PLASMIC score of 4 placed her in the low-risk category for severe ADAMTS13 deficiency whilst she fulfilled criteria for partial HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome per Tennessee classification. Despite delivery, her symptoms persisted with subsequent ADAMTS13 assay confirming acquired TTP, subsequently requiring repeated plasmapheresis and rituximab to achieve disease control. Thrombotic microangiopathy remains a diagnostic challenge especially in the peripartum population, and scoring systems such as PLASMIC score and Tennessee classification may be of limited utility.

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Keywords

thrombotic microangiopathy, thrombotic thrombocytopenic purpura, hemolysis, elevated liver enzymes, low platelets, atypical hemolytic uremic syndrome, PLASMIC score, pregnancy, ADAMTS13

References
1. Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: a systematic review and meta-analysis. Transfusion. 2020; 60(9):2047-2057. doi:10.1111/trf.15954
2. Kaiser C, Distler W. Thrombotic thrombocytopenic purpura and HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome: differential diagnostic problems. Am J Obstet Gynecol. 1996; 175(2):506-507. doi:10.1016/s0002-9378(96)70179-5
3. Uslu M, Güzelmeriç K, Aşut I. Familial thrombotic thrombocytopenic purpura imitating HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) in two sisters during pregnancy. Am J Obstet Gynecol. 1994; 170(2):699-700. doi:10.1016/S0002-9378(94)70252-7
4. Rehberg JF, Briery CM, Hudson WT, Bofill JA, Martin JN Jr. Thrombotic thrombocytopenic purpura masquerading as hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in late pregnancy. Obstet Gynecol. 2006; 108(3 Pt 2):817-820. doi:10.1097/01.AOG.0000215994.25958.31
5. Aydin S, Ersan F, Ark C, Arioğlu Aydin Ç. Partial HELLP syndrome: maternal, perinatal, subsequent pregnancy and long-term maternal outcomes. J Obstet Gynaecol Res. 2014; 40(4):932-940. doi:10.1111/jog.12295
6. Ramadan MK, Malas A, El-Tal R, Itani SE, Rabah H, Badr DA. Appraisal of short- and long-term outcomes of partial versus complete HELLP syndromes: A retrospective cohort study. J Fetal Med. 2021; 8:185-192. doi:10.1007/s40556-021-00316-4
7. Martin JN Jr, Rose CH, Briery CM. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol. 2006; 195(4):914-934. doi:10.1016/j.ajog.2005.08.044
8. Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 2004; 103(5 Pt 1):981-991. doi:10.1097/01.AOG.0000126245.35811.2a
9. Bendapudi PK, Hurwitz S, Fry A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017; 4(4):e157-e164. doi:10.1016/S2352-3026(17)30026-1
10. Little DJ, Mathias LM, Page EE, Kremer Hovinga JA, Vesely SK, George JN. Long-term kidney outcomes in patients with acquired thrombotic thrombocytopenic purpura. Kidney Int Rep. 2017; 2(6):1088-1095. doi:10.1016/j.ekir.2017.06.007
11. Kim HY, Sohn YS, Lim JH, et al. Neonatal outcome after preterm delivery in HELLP syndrome. Yonsei Med J. 2006; 47(3):393-398. doi:10.3349/ymj.2006.47.3.393
How to Cite
Mohamed Jiffry, M. Z., Ahmed-khan, M. A., Carmona Pires, F., Okam, N., & Hanif, M. (2022). Diagnostic dilemma in a patient presenting with thrombotic microangiopathy in the setting of pregnancy. Archive of Clinical Cases, 9(1), Arch Clin Cases 2022; 9(1):24-28. https://doi.org/10.22551/2022.34.0901.10199
Issue
Vol. 9 No. 1 (2022)
Section
Case Reports

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How to Cite

Mohamed Jiffry, M. Z., Ahmed-khan, M. A., Carmona Pires, F., Okam, N., & Hanif, M. (2022). Diagnostic dilemma in a patient presenting with thrombotic microangiopathy in the setting of pregnancy. Archive of Clinical Cases, 9(1), Arch Clin Cases 2022; 9(1):24-28. https://doi.org/10.22551/2022.34.0901.10199