Enteropathy associated T-cell lymphoma is one of the most problematic diseases encountered by gastroenterologists. Despite being the most common intestinal T-cell lymphoma, it is rare, with an estimated incidence of 0.1 per 100 000 in Western populations.1–3
It can be easily missed, as only half of EATL cases have a previous diagnosis of coeliac disease. Even among those who have a diagnosis of coeliac disease, the histology is variable and can overlap with refractory coeliac disease or ulcerative jejunitis.4 5
Finally, even with a diagnosis, the treatment options are limited; EATL responds poorly to chemotherapy or stem-cell transplantation, and the most recent literature estimates the 2-year survival at less than 20%.6–8
In our patient's case, despite rapid recognition of the disease, appropriate investigation and numerous attempts to obtain diagnostic tissue samples, we failed to confirm the diagnosis prior to her death. What was unusual about our patient's case was the presence of cardiac involvement that escaped diagnosis and that contributed to her death.
The literature relating to EATL suggests that 20% of patients will present with Ann Arbour stage IV disease, commonly involving the lungs, liver, spleen or bone marrow.6 9
However, large autopsy series of patients with non-Hodgkin's lymphoma have identified cardiac involvement in approximately 20% of cases.10 11
Even among solid organ tumours, myocardial metastases are present in up to one quarter of cases at postmortem, most commonly arising from melanoma, breast or lung. However, these cardiac metastases are usually asymptomatic and are rarely a terminal event, and as a result, they are rarely sought or diagnosed.12
Curiously, the published literature relating to EATL has not frequently documented this complication, which may suggest that EATL has a lower rate of cardiac involvement than other forms of NHL. Indeed, cardiac disease arising from EATL has only been documented in one patient among a single case series.6
Our patient's case became more unusual when ECG changes suggested an acute myocardial infarction and echocardiography demonstrated findings consistent with ischaemic heart disease. Confounding the diagnosis of lymphomatous cardiac involvement was the concurrent presence of atheromatous disease in the coronary vasculature on angiography, a finding that the postmortem confirmed in addition to demonstrating mural fibrosis consistent with previous myocardial infarction. Cardiac involvement by tumour frequently results in ECG changes, but it is rare to see ST segment alterations mimicking a myocardial infarction.12 13
Furthermore, echocardiography, which is reported to be the radiological modality of choice for the investigation of cardiac metastases, especially in the left-ventricular wall, failed to demonstrate the lesions in this instance.14
We should perhaps have been suspicious of the diagnosis of coronary artery disease in a patient who was asymptomatic from a cardiac point of view, whose ECG changes failed to progress with time and who was under investigation for an EATL. However, the concurrent diagnosis of coronary artery disease, made with electrocardiographic, biochemical, angiographic and echocardiographic evidence masked this rare complication of a rare disease.
- EATL is a rare disease that can easily be overlooked.
- Cardiac involvement is thought to occur in up to 20% of lymphoma cases but is rarely symptomatic and infrequently associated with terminal events.
- Data must be interpreted in the appropriate clinical context. Attributing abnormal investigation results to a previously known disease must be done with caution in the setting of a new unrelated presentation.