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JCR: Journal of Clinical Rheumatology:
doi: 10.1097/RHU.0b013e3181d569e1
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Bone Marrow Hemophagocytosis Complicating Rheumatoid Arthritis

Collamer, Angelique MD; Arroyo, Ramon MD

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From the Rheumatology Service, Brooke Army Medical Center, Fort Sam Houston, TX.

The opinions or assertions contained herein are those of the authors and are not to be construed as official policy of the Department of the Army, Department of the Air Force, or the Department of Defense.

Correspondence: Angelique N. Collamer, MD, Rheumatology Service, Brooke Army Medical Center, 3851 Roger Brooke Dr, Fort Sam Houston, TX 78234. E-mail: angelique.collamer@amedd.army.mil.

A 52-year-old woman with a 26-year history of seropositive erosive rheumatoid arthritis presented with a 3-week history of fever, weakness, and diarrhea and was admitted to the intensive care unit with hypotension, pancytopenia, and elevated transaminases. One week later, despite aggressive supportive measures and antibiotics, the febrile episodes persisted and the patient developed poor oxygenation requiring mechanical ventilation, and had jaundice and a livedoid skin rash. Laboratories were notable for worsening pancytopenia, hypofibrinogenemia (92 mg/dL), elevated ferritin (4240 ng/mL), hypertriglyeridemia (493 mg/dL), and a paradoxically low erythrocyte sedimentation rate of 21 mm/h. Bone marrow biopsy revealed hemophagocytosis of erythrocytes, leukocytes, and thrombocytes; shown here is a histiocyte with lymphocyte and platelet inclusions (Fig. 1).

Figure 1
Figure 1
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The patient was diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH), and slowly recovered following treatment with dexamethasone, cyclosporine, and etoposide. HLH is a sepsis-like hyperinflammatory syndrome characterized by fever, pancytopenia, splenomegaly, hypertriglyceridema, hypofibrinogenemia, elevated ferritin, reduced erythrocyte sedimentation rate, and bone marrow hemophagocytosis. The syndrome can be familial or related to an underlying condition including malignancy and infection. Secondary HLH is known as macrophage activating syndrome when associated with autoimmune disease, and is most commonly described in patients with systemic onset juvenile arthritis and systemic lupus erythematosus. The condition has rarely been reported as a serious complication in adults with other rheumatic conditions, including rheumatoid arthritis1 and is likely underrecognized.2 Our patient was eventually discharged, however, she required readmission 1 month later with recurrence of the hemophagocytic syndrome, which ultimately proved fatal.

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REFERENCES

1. Dhote R, Simon J, Papo T, et al. Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum. 2003;49:633–639.

2. Janka G. Hemophagocytic syndromes. Blood Rev. 2007;21:245–253.

© 2010 Lippincott Williams & Wilkins, Inc.

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