Systemic lupus erythematosus presenting to haematology with pancytopenia and features of macrophage activation syndrome

Description 

This bone marrow biopsy (figure 1) shows haemophagocytosis consistent with macrophage activation syndrome (MAS) secondary to previously undiagnosed systemic lupus erythematosus (SLE).

Figure 1

Bone marrow biopsy with abundant cell-bound haemosiderin and focal haemophagocytosis with cellular debris identified in macrophage cytoplasm (arrows). This is consistent with, but not specific for, macrophage activation syndrome.

A 44-year-old woman had been unwell for 4 weeks with fever, weight loss and an aphthous ulcer. There were no other clinical features of SLE.

There was pancytopenia (platelet count 65x10⁹/L; neutrophil count 0.5x10⁹/ L; haemoglobin 107 g/L). The reticulocyte count was 20x10⁹/ L. Parvovirus and Epstein-Barr virus IgM were not detected.

A very high ferritin level of 3717 µg/L in the context of cytopenias was suggestive of MAS, a life-threatening hyperinflammatory state.1 Clinical features of MAS include fever, lymphadenopathy and hepatosplenomegaly. Laboratory markers include pancytopenia, altered liver function...

http://ift.tt/2HyeylD