Patients with TM are at risk of severe sepsis due to various reasons. The causes include the abnormalities in the immune system related to the disease including defective T and B lymphocyte function as well as the complement system. Patients with high iron levels are vulnerable to infections with siderophillic organisms such as Klebsiella spp, Yersinia enterocoloitica, E.coli, Vibrio vulnificus, Streptococcus pneumoniae, Listeria monocytogenes, Pseudomonas aeruginosa and Legionella pneumophila. Recurrent blood transfusions also predispose to blood borne infections. Iron chelation therapy also predispose to infections such as Yersinia enterocolitica. Splenectomised patients with TM are at risk of severe sepsis due to capsulated organisms especially if proper vaccination procedures are not undertaken. (10) Diabetes which occurs as a complication of iron overload further suppresses the immune system of these patients pre-disposing them to invasive infections. The spectrum of diseases related to sepsis in TM patients ranges from simple viral infections to abscesses at different places to disseminated sepsis including infective endocarditis. (8) (11–16)
Our patient had multiple risk factors which pre-disposed her to sepsis. She was splenectomised and had the vaccinations prior to her surgery without any booster doses and had poor compliance to antibiotic prophylaxis. She also had severe iron overload which was not managed adequately due to poor compliance of the patient as well as inadequate monitoring from her health care services. Brittle diabetes with poor glycemic control also contributed further to her immunosuppression. As a patient subjected to multiple and difficult cannulations for regular blood transfusions this is likely to introduce infections unless strict aseptic procedures are adhered to.
The initial non-specific symptoms with sudden onset respiratory distress and high inflammatory markers along with deranged liver enzymes led the clinical team to think of a respiratory pathology as the primary focus of infection. However, the persistence of high swinging fever spikes despite minimal lung signs while on multiple broad-spectrum antibiotics made it evident that the focus of infection was not the lung. Since the ultrasound scan of the abdomen and the 2D echocardiogram repeatedly became normal it was a diagnostic dilemma to the clinical team which led them to arrange a CECT chest abdomen pelvis which ultimately revealed the presence of the liver abscess with multiple septic emboli in lungs and thrombosis of the hepatic vein. This highlights the importance of early and repeated utilization of imaging investigations in patients with immunosuppression for identification of the focus of the infection which could ultimately lead to early treatment.
The presence of multiple septic foci warranted a differential diagnosis of severe disseminated sepsis, infective endocarditis or melioidosis. The 2D echocardiogram at this stage revealed the presence of a large oscillating mass in the right atrium which was unlikely to be a vegetation. The repeat blood cultures at this point became positive for Vancomycin resistant enterococci which was sensitive only to Linezolid and patient became negative for melioidosis. This signifies the importance of repeating relevant investigations in situations when the clinical picture does not tally with the available investigations.
The identity of the right atrial mass remained a conundrum for weeks since the patient was not suitable to undergo a Trans Oesphageal Echocardiogram (TOE) at that time. The possibility of a thrombus or a myxoma was high on the list of differential diagnoses considering the size of the mass. Considering that all her past echocardiograms including the one done on admission were normal, the possibility of a myxoma was considered unlikely since it was highly unlikely for a cardiac mass to grow rapidly to reach a size like this. An increased incidence of myxomas in patients with TM was not known and rapid growth of a myxoma had been reported only once which had been attributed to severe immunosuppression (17). Thalassaemia is well recognized to be a hypercoagulable state (8) Since TM patients are at high risk of thrombo-embolic disease the chance of the mass being a thrombus was considered to be more likely. (18)
Our patient underwent a TOE subsequently which revealed the presence of an oscillating mass attached to right atrium via a stalk – Fig. 1. The repeat CECT done revealed the presence of hepatic vein thrombosis extending through the inferior vena cava into the right atrium without significant interval change compared with the previous CECT. We concluded the right atrial mass to be a thrombus which would have become infected giving rise to multiple septic emboli which lead to severe consequences in this patient.
Through this case report we wish to highlight the significance of sepsis probably acting as a precipitant for venous thrombosis, presenting as an intra-cardiac thrombosis in a patient with thalassaemia major