Johannes J.A. Auwerda, MD; J.H. Paul Wilson, MD; Pieter Sonneveld, MD, PhD
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Auwerda J., Wilson J., Sonneveld P.; Foamy Macrophage Syndrome due to Hydroxyethyl Starch Replacement: A Severe Side Effect in Plasmapheresis. Ann Intern Med. 2002;137:1013-1014. doi: 10.7326/0003-4819-137-12-200212170-00037
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Published: Ann Intern Med. 2002;137(12):1013-1014.
TO THE EDITOR:
A 38-year-old woman had received stanozolol, cyclosporine, and frequent plasmapheresis since 1997 because of IgG λ monoclonal gammopathy with cryoglobulinemia-associated ulcerating leukocytoclastic vasculitis. Initially, gelatin-based plasma expanders and pasteurized human albumin were used. Beginning in February 1999, plasma volumes were exchanged at a 1:1 ratio with undiluted 6% hydroxyethyl starch (HES) (Fresenius–Kabi, Hamburg, Germany) because of the reported advantages of HES.
In August 2000, the patient developed severe weight loss, sensory polyneuropathy of the legs, and deteriorating eyesight. On examination, she was malnourished (body mass index, 17.6 kg/m2) with hepatosplenomegaly and ascites. She had normocytic anemia (hemoglobin level, 7.1 mmol/L; mean corpuscular volume, 82 fL), thrombocytopenia (platelet count, 121 × 109 cells/L), low serum creatinine concentration (40 µmol/L [0.45 mg/dL]) and albumin level (26 g/L), and slightly elevated serum IgG level (15.9 g/L) and aminotransferase levels (aspartate aminotransferase level, 62 U/L; alanine aminotransferase level, 52 U/L). Magnetic resonance imaging of the brain revealed vasculitis of the choroid plexus with hydrocephalus and pituitary stalk edema. Histologic examination of bone marrow, skin, duodenal mucosa, liver, peritoneum, and dura mater and cytologic examination of the ascitic fluid revealed massive infiltration with typical foamy macrophages (Figure). Hematoxylin–eosin, periodic acid Schiff, and oil red O staining showed empty vacuoles. Mycobacterium avium infection was ruled out. Enzyme analysis excluded Wolman, Niemann–Pick, and Gaucher diseases (all lysosomal storage diseases). Immunoelectron microscopy with polyclonal rabbit anti-HES serum, performed by Dr. S. Ständer (Westfälische Wilhelm-Universität, Munster, Germany), confirmed massive tissue storage of HES, especially in the vacuoles of the macrophages (Figure). Although plasmapheresis with conventional expanders was continued, the patient improved only slightly. After 8 months, a bone marrow biopsy still revealed massive foamy cell infiltration.
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