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A 67-year-old woman with history of severe rheumatoid arthritis and use of multiple biologics including infliximab, tocilizumab and abatacept presented with fever of 39.1°C and severe pancytopenia (white blood cell count (WBC)=1.0×109/L, absolute neutrophil count (ANC)=0.55×109/L, haemoglobin=8.7 g/dL, platelets=46×109/L). As part of the pancytopenia evaluation, imaging (CT of the chest, abdomen and pelvis) showed diffuse lymphadenopathy. Further evaluation revealed an elevated ferritin (8564 ng/mL), hypofibrinogenaemia (fibrinogen=95 mg/dL), elevated triglycerides (399 mg/dL) and a soluble interleukin 2 receptor level of 41 167 units/mL, satisfying diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH). A subsequent bone marrow biopsy also revealed morphological evidence of haemophagocytosis (figure 1A), in addition to a population of very large and atypical mononuclear cells with markedly irregular, folded nuclear contours, prominent nucleoli and moderate amounts of cytoplasm (figure 1B,C). A similar large cell infiltrate was identified in …
Footnotes
Contributors XIR wrote the paper, prepared clinical images for publication, prepared manuscript for publication and approved the manuscript. LJM obtained and interpreted clinical images for publication, prepared clinical images for publication and approved the manuscript. JHS treated the patient, analysed the data providing clinical interpretations and approved the manuscript. SDP treated the patient, analysed the data providing clinical interpretations, wrote the paper and approved the manuscript.
Competing interests None declared.
Patient consent Consent obtained from guardian.
Provenance and peer review Not commissioned; externally peer reviewed.