Bone marrow aspirate LE cells at acute lupus presentation

A 17-year-old male presented with altered mental status and septic shock (temperature, 101.5°F [38.6°C], tachycardia [188 beats per minute], and tachypnea [52 breaths per minute]), with Staphylococcus aureus bacteremia. This presentation followed several days of fever, chills, and gastrointestinal distress, as well as months of anorexia with a 43-pound weight loss, alopecia, and pustular rash. Social history was significant for smoking cigars and e-cigarettes and for unprotected sex with men, and urine was positive for cannabinoids. His blood cell counts showed anemia (hemoglobin, 9.3 g/dL), thrombocytopenia (46 000 platelets per microliter), mild neutropenia (1440 neutrophils per microliter), lymphopenia (260 lymphocytes per microliter), and monocytopenia (100 monocytes per microliter). Bone marrow aspiration and biopsy revealed normocellular marrow with preserved granulopoiesis and megakaryopoiesis and markedly decreased erythropoiesis. Additionally, scattered cells (panels A-F; Wright-Giemsa stain, original magnification ×1000) were evident on the aspirate smear. These cells, known as lupus erythematosus (LE) cells, are neutrophils with phagocytosed nuclei bound by immunoglobulin; advanced forms (arrow) are known as “hematoxylin bodies.” Hemophagocytic histiocytes were not seen. Additional studies were positive for antinuclear antibody (1:1280) with a homogeneous pattern, for anti–double-stranded DNA (>1:300), for lupus anticoagulant, and for anti-cardiolipin. The patient was diagnosed with new-onset systemic lupus erythematosus. Despite aggressive support, the patient died after a complicated hospital course with multiple infections.

The early identification of LE cells was a critical clue to his underlying diagnosis.