Neurological changes in patients with Thrombotic Thrombocytopenic Purpura and ADAMTS13 deficiency: A case report
DOI:
https://doi.org/10.33448/rsd-v13i8.46728Keywords:
Thrombotic Thrombocytopenic Purpura; Neurology; Plasmapheresis; Hematology.Abstract
The objective of this article is to report a case of Thrombotic Thrombocytopenic Purpura (TTP) with significant neurological manifestations, highlighting the importance of early diagnosis and appropriate therapeutic management to improve patients' prognosis. Previously healthy adult patient, who presented symptoms of TTP in October 2022, 49 years old, was admitted with severe headache, disorientation and ecchymosis, associated with thrombocytopenia, microangiopathic hemolytic anemia and renal and cardiac impairment. The diagnosis of TTP was based on severe thrombocytopenia, hemolytic anemia, presence of schistocytes, elevated LDH and bilirubin, and reduced levels of ADAMTS13. Therapy included corticosteroids, caplacizumab and plasmapheresis, with partial clinical response. However, the patient developed new neurological manifestations, including dyslalia and cerebral ischemia, evidenced by computed tomography. The case analysis suggests that severe ADAMTS13 deficiency was the main factor related to the severity of neurological complications, even though there was a dissociation between the site of ischemia and the clinical presentation. This case study contributes to the literature by detailing a rare and serious episode of TTP with neurological complications, highlighting the need for rapid and precise interventions to prevent disease progression. Despite the limitations inherent to the single-case study, the findings reinforce the importance of immediate treatment, including plasmapheresis and immunosuppression, to reduce mortality and improve the recovery of patients with TTP.
References
Altaf, F., Shrivastava, S., Allena, R., Kumar, J., & Khaja, M. (2024). Uncommon Manifestation: Thrombotic Thrombocytopenic Purpura Presenting Solely as Petechial Rash. Cureus, 16(4). https://doi.org/10.7759/CUREUS.57994
Anstadt, M. P., Carwile, J. M., Guill, C. K., Conklin, L. D., Soltero, E. R., Lucci, A., & Kroll, M. H. (2002). Relapse of thrombotic thrombocytopenic purpura associated with decreased VWF cleaving activity. The American journal of the medical sciences, 323(5), 281–284. https://doi.org/10.1097/00000441-200205000-00011
Arcudi, S., Ferrari, B., Pontiggia, S., Tufano, A., Artoni, A., Mancini, I., & Peyvandi, F. (2019). Prevention of relapse in patients with acquired thrombotic thrombocytopenic purpura undergoing elective surgery: a case series. Journal of thrombosis and haemostasis : JTH, 17(3), 492–498. https://doi.org/10.1111/JTH.14381
Azevedo, R., Bazurto, G., Krum, L., Munhoz, L., Gomes, C., Vanelli, T., Filik, H., Lamaison, D., Brun, C., & Fogliatto, L. (2022). Púrpura trombocitopênica trombótica - Um relato de caso. Hematology, Transfusion and Cell Therapy, 44, S292–S293. https://doi.org/10.1016/J.HTCT.2022.09.496
Borogovac, A., Tarasco, E., Kremer Hovinga, J. A., Friedman, K. D., Asch, A. S., Vesely, S. K., Prodan, C. I., Terrell, D. R., & George, J. N. (2022). Prevalence of neuropsychiatric symptoms and stroke in patients with hereditary thrombotic thrombocytopenic purpura. Blood, 140(7), 785–789. https://doi.org/10.1182/BLOOD.2022016044
Filatov, A., Kassar, E., & Cole, O. (2020). Thrombotic Thrombocytopenic Purpura Masquerading as Acute Ischemic Stroke. Cureus, 12(4). https://doi.org/10.7759/CUREUS.7661
Kremer Hovinga, J. A., Heeb, S. R., Skowronska, M., & Schaller, M. (2018). Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Journal of thrombosis and haemostasis : JTH, 16(4), 618–629. https://doi.org/10.1111/JTH.13956
Lakra, R., Lopez, J., & Graham, C. (2023). Thrombotic Thrombocytopenic Purpura Without Evidence of Microangiopathic Hemolytic Anemia: A Case Report. Cureus, 15(3). https://doi.org/10.7759/CUREUS.35927
Long, B., Bridwell, R. E., Manchanda, S., & Gottlieb, M. (2021). Evaluation and Management of Thrombotic Thrombocytopenic Purpura in the Emergency Department. The Journal of emergency medicine, 61(6), 674–682. https://doi.org/10.1016/J.JEMERMED.2021.07.045
Merchán-Hamann, E., & Tauil, P. L. (2021). Proposta de classificação dos diferentes tipos de estudos epidemiológicos descritivos. Epidemiologia e Serviços de Saúde, 30(1), e2018126. https://doi.org/10.1590/S1679-49742021000100026
Medronho, R., & Bloch, K. V. (2008). Epidemiologia (2nd ed.). Atheneu.
Neuman, L., Joseph, A., Bouzid, R., Lescroart, M., Mariotte, E., Ederhy, S., Tuffet, S., Baudel, J. L., Benhamou, Y., Galicier, L., Grangé, S., Provôt, F., Neel, A., Pène, F., Delmas, Y., Presne, C., Poullin, P., Wynckel, A., Perez, P., … Patricia, Z. (2023). Predictors of acute ischemic cerebral lesions in immune-mediated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Journal of neurology, 270(10), 5023–5033. https://doi.org/10.1007/S00415-023-11830-Y
Page, E. E., Kremer Hovinga, J. A., Terrell, D. R., Vesely, S. K., & George, J. N. (2017). Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood advances, 1(10), 590–600. https://doi.org/10.1182/BLOODADVANCES.2017005124
Pereira, A. S., Shitsuka, D. M., Parreira, F. J., & Shitsuka, R. (2018). Metodologia da pesquisa científica.
Pinheiro Maux Lessa, M., Soares Ferreira Junior, A., Graton, M., Simon, E., Ledbetter, L., & Onwuemene, O. A. (2024). Managing surgical relapse risk in acquired thrombotic thrombocytopenic purpura: a systematic review. Research and practice in thrombosis and haemostasis, 8(5). https://doi.org/10.1016/J.RPTH.2024.102478
Scully, M., Rayment, R., Clark, A., Westwood, J. P., Cranfield, T., Gooding, R., Bagot, C. N., Taylor, A., Sankar, V., Gale, D., Dutt, T., McIntyre, J., & Lester, W. (2023). A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies. British journal of haematology, 203(4), 546–563. https://doi.org/10.1111/BJH.19026
Tonaco, L. C., Rios, D. R. A., Vieira, L. M., Carvalho, M. G., & Dusse, L. M. S. (2010). Púrpura trombocitopênica trombótica: o papel do fator von Willebrand e da ADAMTS13. Revista Brasileira de Hematologia e Hemoterapia, 32(2), 155–161. https://doi.org/10.1590/S1516-84842010005000043
Wada, H., Matsumoto, T., & Yamashita, Y. (2014). Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Seminars in thrombosis and hemostasis, 40(8), 866–873. https://doi.org/10.1055/S-0034-1395154
Williams, L. A., & Marques, M. B. (2016). Pathology Consultation on the Diagnosis and Treatment of Thrombotic Microangiopathies (TMAs). American journal of clinical pathology, 145(2), 158–165. https://doi.org/10.1093/AJCP/AQV086
Zhang, Z., & He, M. (2023). Thrombotic thrombocytopenic purpura presenting as stroke mimics with normal diffusion-weighted MRI. BMC neurology, 23(1). https://doi.org/10.1186/S12883-023-03489-9
Zheng, X. L., Vesely, S. K., Cataland, S. R., Coppo, P., Geldziler, B., Iorio, A., Matsumoto, M., Mustafa, R. A., Pai, M., Rock, G., Russell, L., Tarawneh, R., Valdes, J., & Peyvandi, F. (2020). ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. Journal of thrombosis and haemostasis : JTH, 18(10), 2496–2502. https://doi.org/10.1111/JTH.15010
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Phamela de Freitas Geraldo Antunes; Daniel Antunes Pereira; Fernanda Alvarez de Oliveira ; Shara Aline Bueno Dantas; Bárbara Fernandes Costa de Araújo; Andreza de Lima Moura; Lígia Costa de Moraes; Amanda Menescal Sias Lins ; Priscila Marino Pereira; Pablo Rafael Chaves Dias; Guilherme Anderson de Freitas Lemos
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
