Description

We present a case of a 53-year-old woman who presented to the emergency room with acute abdominal pain, fever and haemodynamic and respiratory instability and was admitted to the intensive care unit with fulminant septic shock with multiorgan failure. CT imaging of the abdomen showed no gross abnormalities, initial laboratory results are presented in table 1.

Despite adequate resuscitation, broad-spectrum antibiotics and supportive care, following the International Guidelines of the Surviving Sepsis Campaign, she died within a couple of hours. The patient had a history of systemic lupus erythematosus (SLE) with arthralgia, positive antinuclear antibodies and persistently high anti-DNA antibodies, but without active disease and no immunosuppressive medication since years.

Leucocyte typing of the initial peripheral blood smear showed Howell-Jolly bodies and diplococci, indicating massive bacterial load and functional asplenism. Functional asplenism or hyposplenism is present in up to 5% of patients with SLE with or without disease activity before.1 No thrombocytosis or antiphospholipid antibodies—that are suggested as clues to autosplenectomy—were found in our patient.2

Postmortem blood cultures became positive for Streptococcus pneumonia. Pathology revealed perivascular infiltrates in the liver and Gamna-Gandy bodies (GGB) in a remarkably small spleen (figure 1).

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Figure 1

Microscopy of the spleen (HE ×25), revealing Gamna-Gandy nodules (centre).

The%20patient did not take medication or drugs that could explain this image of the liver. GGB or haemosiderotic nodules are foci of haemosiderin deposition resulting from intrasplenic haemorrhage (figure 2 and figure 3). Portal hypertension is considered the primary cause of GGBs and their appearance is associated with sickle-cell anaemia, hereditary haemochromatosis, haemolytic anaemia, acquired haemosiderosis, paroxysmal nocturnal haemoglobinuria, portal or splenic vein thrombosis, leukaemia, lymphoma and repeated blood transfusion.3 However, these conditions known to be associated with GGB were absent in our patient.

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Figure 2

High-power view (HE ×200) of the Gamna-Gandy bodies. These rust-coloured foci contained fibrous tissue with haemosiderin deposits, and probably form due to scarring at sites of small perivascular haemorrhages.

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Figure 3

Prussian blue stain showing iron deposition (blue) in the Gamna-Gandy bodies.

Although the pathophysiological processes leading to hyposplenism in SLE remain largely unknown, it has been proposed that this is a result of silent infarction due to hyposplenism.4 As a consequence of inflammatory microvessel damage, splenic haemosiderin depositions may occur and remain visible, also after the resolution of vasculitis. Furthermore, despite the absence of antiphospholipid antibodies in this patient, splenic microangiopathy and microthrombosis associated with SLE may have contributed to ‘leaky’ splenic microvasculature, resulting in GGBs and partial autosplenectomy as described above.

In conclusion, this case once again illustrates the association of SLE with asplenism and the fulminant course of pneumococcal sepsis in those patients,5 despite disease inactivity for years. Moreover, GGB were found, which could not be related to known associated diseases. As far as we know, this is the first case describing GGB in a patient with SLE and functional asplenism.