Unexplained pulmonary embolism post SARS-CoV2 pneumonia in a patient with MYH9-related disease: a case report

Laura Tomaino; Vincenzo Giannicola Menditto; Cinzia Nitti; Gianluca Moroncini; Aldo Salvi

doi:10.23750/abm.v92iS1.11968

Unexplained pulmonary embolism post SARS-CoV2 pneumonia in a patient with MYH9-related disease: a case report

Authors

Laura Tomaino a:1:{s:5:"en_US";s:82:"Emergency Medicine Residency Program, Marche Polytechnic University, Ancona, Italy";}
Vincenzo Giannicola Menditto Department of Internal and Emergency Medicine, Azienda Ospedaliera Universitaria Ospedali Riuniti, Ancona, Italy
Cinzia Nitti Department of Internal and Emergency Medicine, Azienda Ospedaliera Universitaria Ospedali Riuniti, Ancona, Italy
Gianluca Moroncini Clinica Medica, Azienda Ospedaliera Universitaria Ospedali Riuniti, Marche Polytechnic University, Ancona, Italy
Aldo Salvi Department of Internal and Emergency Medicine, Azienda Ospedaliera Universitaria Ospedali Riuniti, Ancona, Italy

DOI: https://doi.org/10.23750/abm.v92iS1.11968

Keywords:

MYH9-related disease, macrothrombocytopenia, SARS-CoV-2 pneumonia, pulmonary embolism, ROTEM

Abstract

This report describes the case of a man affected by Myosin Heavy Chain 9 (MYH9)-related platelet disorder, with a recent history of SARS-CoV-2 pneumonia, who developed pulmonary embolism (PE). At the admittance the patient presented a marked thrombocytopenia. The rotational thromboelastometry (ROTEM) showed a reduction in maximum clot firmness. The CT scan showed a lobar PE while and no sign of superficial or deep venous thrombosis was found. Given the contraindication of anticoagulant therapy due to severe thrombocytopenia, after collegial evaluation of the case, an inferior vena cava filter was applied. The patient was discharged after 5 days of hospitalization, and fondaparinux 2.5 mg subcutaneously was prescribed for two months. Could MYH9 mutation contribute to thrombotic predisposition? Or rather the endothelial dysfunction induced by SARS-CoV-2 infection? The report presents a dissertation on the possible causes for the PE and describes the therapeutic strategy adopted.

References

Althaus K, Greinacher A. MYH9-related platelet disorders. Semin Thromb Hemost. 2009 Mar;35(2):189-203. doi: 10.1055/s-0029-1220327. Epub 2009 Apr 30. PMID: 19408192.

Martinez Licha CR, McCurdy CM, Maldonado SM, Lee LS. Current Management of Acute Pulmonary Embolism. Ann Thorac Cardiovasc Surg. 2020;26(2):65-71. doi:10.5761/atcs.ra.19-00158.

Pons S, Fodil S, Azoulay E, Zafrani L. The vascular endothelium: the cornerstone of organ dysfunction in severe SARS-CoV-2 infection. Crit Care. 2020 Jun 16;24(1):353. doi: 10.1186/s13054-020-03062-7. PMID: 32546188; PMCID: PMC7296907.

Schenk B, Görlinger K, Treml B, Tauber H, Fries D, Niederwanger C, Oswald E, Bachler M. A comparison of the new ROTEM® sigma with its predecessor, the ROTEMdelta. Anaesthesia. 2019 Mar;74(3):348-356. doi: 10.1111/anae.14542. Epub 2018 Dec 21. PMID: 30575011.

Levrat A, Gros A, Rugeri L, Inaba K, Floccard B, Negrier C, David JS. Evaluation of rotation thrombelastography for the diagnosis of hyperfibrinolysis in trauma patients. Br J Anaesth. 2008 Jun;100(6):792-7. doi: 10.1093/bja/aen083. Epub 2008 Apr 24. PMID: 18440953.

Cotton BA, Faz G, Hatch QM, Radwan ZA, Podbielski J, Wade C, Kozar RA, Holcomb JB. Rapid thrombelastography delivers real-time results that predict transfusion within 1 hour of admission. J Trauma. 2011 Aug;71(2):407-14; discussion 414-7. doi: 10.1097/TA.0b013e31821e1bf0. PMID: 21825945.

Almskog LM, Wikman A, Svensson J, Wanecek M, Bottai M, van der Linden J, Ågren A. Rotational thromboelastometry results are associated with care level in COVID-19. J Thromb Thrombolysis. 2021 Feb;51(2):437-445. doi: 10.1007/s11239-020-02312-3. Epub 2020 Oct 17. PMID: 33068277; PMCID: PMC7568025.

Heller P, Pecci A, Glembotsky A, et al. Unexplained recurrent venous thrombosis in a patient with MYH9-related disease. Platelets, June 2006; 17(4): 274-5.

Konstantinides SV, Meyer G, Becattini C, et al, 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS): The Task Force for the diagnosis and management of acute pulmonary embolism of the European Society of Cardiology (ESC), European Heart Journal, Volume 41, Issue 4, 21 January 2020, Pages 543–603, https://doi.org/10.1093/eurheartj/ehz405.

Gagnier JJ, Kienle G, Altman DG, et al. The CARE Guidelines: Consensus-based Clinical Case Reporting Guideline Development. Glob Adv Health Med. 2013;2(5):38-43. doi:10.7453/gahmj.2013.008.