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Management of Gardner–Diamond syndrome with therapeutic plasma exchange

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Gardner–Diamond syndrome (GDS), also known as autoerythrocyte sensitization syndrome or psychogenic purpura, is an unusual disorder of uncertain etiology and pathophysiology. GDS is characterized by episodes of extensive painful target-shaped… Click to show full abstract

Gardner–Diamond syndrome (GDS), also known as autoerythrocyte sensitization syndrome or psychogenic purpura, is an unusual disorder of uncertain etiology and pathophysiology. GDS is characterized by episodes of extensive painful target-shaped bruises. Approximately 100 cases have been reported and have shown a female preponderance. One case report of therapeutic plasma exchange (TPE) with sham control demonstrated symptomatic improvement. In this case, a 30-year-old female with past medical significant for anxiety disorder/panic attacks, migraine headaches, and heavy menses, who presented with diffuse, painful, targetoid ecchymosis on her upper and lower extremities (Figures 1 and 2). Coagulation studies, platelet aggregation, hematology, and chemistry parameters were all normal with the exception of mild anemia (hemoglobin of 10.8 mg/dL). Sm-RNP, SS-A, and SS-B were negative. ANA was 1:320. Following a clinical diagnosis of GDS, she underwent 1 plasma volume TPE with 5% albumin replacement via central line for six procedures over 10 days. Procedures were well tolerated except for anxiety. No new bruising developed during treatment and pre-existing lesions began fading (Figures 3 and 4). Following a bruise-free interval of several weeks post-TPE, the patient reported new onset of less severe painful bruising. Although the pathophysiology is unclear, TPE may be a possible adjunct in the management of GDS.

Keywords: diamond syndrome; gardner diamond; plasma exchange; therapeutic plasma

Journal Title: Journal of Clinical Apheresis
Year Published: 2017

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