Gamna-Gandy nodules of the spleen and asplenism in SLE: a novel association?

Petri AJ, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-220566 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. No thrombocytosis or antiphospholipid antibodies—that are suggested as clues to autosplenectomy—were found in our patient. Postmortem blood cultures became positive for Streptococcus pneumonia. Pathology revealed perivascular infiltrates in the liver and GamnaGandy bodies (GGB) in a remarkably small spleen (figure 1). The patient 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. However, these conditions known to be associated with GGB were absent in our patient. 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. 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 Gamna-Gandy nodules of the spleen and asplenism in SLE: a novel association? Annefleur J Petri, Maarten Limper, Anya Milne, Lenneke E M Haas Images in...