Pulmonary embolism in a patient with eltrombopag-treated aplastic anaemia and paroxysmal nocturnal haemoglobinuria clone during COVID-19 pneumonia

Abstract

Thrombosis in patients with thrombocytopenia has several risk factors, both disease-related and treatment-associated. Recently, COVID-19 infection was recognized as an additional risk factor, further complicating the delicate balance between thrombosis and bleeding in these patients. Here we describe the case of a patient with aplastic anaemia on eltrombopag who developed pulmonary embolism during COVID-19 pneumonia, despite receiving oral anticoagulation with edoxaban. Notably, he was also carrying a large paroxysmal nocturnal haemoglobinuria clone, although without evidence of haemolysis. The presented case recapitulates some of the open questions in thrombotic risk management of cytopenic patients, such as the management of thrombopoietin receptor agonists and the choice of anticoagulation in PNH, while also accounting for the additional thrombotic risk linked to COVID-19.

Deciphering treatment patterns in non-severe/moderate aplastic anemia: an international observational study

“…These findings, although in a limited number of patients, warrant close monitoring of renal and liver function, already routinely suggested for SAA. Additionally, assessment of thrombotic risk pre-eltrombopag is advisable, particularly in patients with PNH clones, since thromboses have been described even in thrombocytopenic patients in this setting [ 10 ]. The use of complement inhibitor, already described in combination with AA therapy [ 11 ], may be useful to limit the risk under such circumstances, and may be started before adding eltrombopag in those with large PNH clones associated with hemolysis.…”

Complement inhibition in paroxysmal nocturnal hemoglobinuria: From biology to therapy

“…13 The role of direct oral anticoagulants (DOACs) is also debated in PNH since thrombosis under DOACs have been reported in this setting. 22 The main risk of complement inhibition are infections, particularly related to meningococcal and other encapsulated bacteria. The former should be prevented by mandatory administration of anti-ACYW135 and B N. meningitidis vaccines, before starting C5 inhibitors.…”

“…Full dose anticoagulants (generally low molecular weight heparin and vitamin K antagonists) are to be used in patients presenting with thrombosis; it is still debated whether to withhold them once complement inhibitor has been started or to continue them long term 13 . The role of direct oral anticoagulants (DOACs) is also debated in PNH since thrombosis under DOACs have been reported in this setting 22 …”

Correction: Pulmonary embolism in a patient with eltrombopag-treated aplastic anaemia and paroxysmal nocturnal haemoglobinuria clone during COVID-19 pneumonia

“…Author details 1 Haematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20100 Milan, Italy. 2 Department of Oncology and Haemato-oncology, University of Milan, Milan, Italy.…”

“…Following publication of the original article [ 1 ], it was reported that the Given and Family names of the authors were transposed. The correct authorship panel is given in this Correction article and the original article [ 1 ] has been updated.…”