BMJ Case Reports 2017; doi:10.1136/bcr-2017-220401
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Double hit lymphoma presenting as haemophagocytic lymphohistiocytosis

  1. Soham D Puvvada1
  1. 1Division of Hematology and Oncology, Department of Medicine, University of Arizona Cancer Center, Tucson, Arizona, USA
  2. 2Department of Pathology, University of Arizona Cancer Center, Tucson, USA
  3. 3Department of Medicine, Division of Hematology, University of Miami Miller School of Medicine Palm Beach Regional Campus, Miami, Florida, USA
  1. Correspondence to Mr Xavier Issac Rivera, xirivera{at}
  1. ↵XIR and LJMG contributed equally.

  • Accepted 1 May 2017
  • Published 15 May 2017


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 …

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