Unusual bleeds, unusual clots^[*]

Summary

Nine unusual bleeding and clotting disorders (or mimickers of such) are described in the format of case presentations, with focus on clinical history, images and diagnostic tests, followed by a discussion of the disease itself and a summarizing clinical teaching point. The disease entities discussed are acquired factor VIII inhibitor, acquired von Willebrand factor inhibitor, haemophilic pseudotumour, Gardner-Diamond syndrome, coumarin-induced skin necrosis, purple toe syndrome, brachiocephalic vein thrombosis with breast enlargement, and leg swelling due to nephrogenic fibrosing dermopathy and lymphoedema. The publication is meant to demonstrate the fascination of clinical coagulation.

Zusammenfassung

Neun ungewöhnliche Blutungs- und Thromboseerkrankungen (oder Erkrankungen, die damit verwechselt werden können) werden dargestellt bzg. Symptomatik, Klinik, Diagnostik und Diskussion einschl. klinischer ,,teaching point“. Vorgestellt werden: erworbener Faktor-VIII-Inhibitor, erworbener von-Willebrand-Faktor-Inhibitor, hämophiler Pseudotumor, Gardner-Diamond-Syndrom, Coumarin- induzierte Hautnekrose, Purple-toe-Syndrom, Brustschwellung durch Vena-brachiocephalica-Thrombose und Beinschwellung aufgrund nephrogener fibrosierender Dermatopathie und Lymphödem. Die Darstellungen sollen die Faszination der klinischen Gerinnung vermitteln.

Keywords

Coagulation - bleeding - thrombosis - vena cava syndrome - nephrogenic fibrosing dermopathy - warfarin skin necrosis - lymphoedema - factor VIII inhibitor - pseudotumour - haemophilia - von Willebrand factor inhibitor - Gardner Diamond syndrome - purple toe syndrome - brachiocephalic vein thrombosis - superior vena cava syndrome

Schlüsselwörter

Gerinnung - Blutung - Thrombose - nephrogene fibrosierende Dermatopathie - Coumarin-induzierte Hautnekrose - Lymphödem - Faktor-VIII-Inhibitor - Pseudotumor - Hämophilie - von-Willebrand-Erkrankung - Gardner-Diamond- Syndrom - Purple-toe-Syndrom - Vena-brachiocephalica- Thrombose - Vena-cava-Syndrom

^* This article is dedicated to my father, Dr. Helmut Moll, paediatrician, Papenburg, Germany, to his 80^th birthday. His clinical images of paediatric medicine are an inspiration for me and a guideline for excellence.

• References

• 1 Hay CR, Brown S, Collins PW. et al. The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. Br J Haematol 2006; 133: 591-605.
• 2 Franchini M. Acquired hemophilia A. Hematology 2006; 11: 119-25.
• 3 Moll S, Fuller E, Cassara J. et al. Acquired von Willebrand’s disease not responding to Rituximab. J Thromb Haemost 2003; 1 (Suppl. 01) CD050.
• 4 Rogers JL, Moll S. Pseudotumour in haemophilia A. Br J Haematol 2007; 136: 519.
• 5 Magallon M, Monteagudo J, Altisent C. et al. Hemophilic pseudotumor: multicenter experience over a 25-year period. Am J Hematol 1994; 45: 103-108.
• 6 Moll S. Psychogenic purpura. Am J Hematol 1997; 55: 146-147.
• 7 Ratnoff OD. Psychogenic purpura (autoerythrocyte sensitization): an unsolved dilemma. Am J Med 1989; 87: 16N-21N.
• 8 Behrendt C, Goos M, Thiel H. et al. Painful bruising syndrome. Hautarzt 2001; 52: 634-637.
• 9 Moll S. Warfarin-induced skin necrosis. Br J Haematol 2004; 126: 628.
• 10 Warkentin TE, Sikov WM, Lillicrap DP. Multicentric warfarin-induced skin necrosis complicating heparin-induced thrombocytopenia. Am J Hematol 1999; 62: 44-48.
• 11 Isenberg JS, Tu Q, Rainey W. Mammary gangrene associated with warfarin ingestion. Ann Plast Surg 1996; 37: 553-555.
• 12 Stirling Y. Warfarin-induced changes in procoagulant and anticoagulant proteins. Blood Coagul Fibrinolysis 1995; 6: 361-373.
• 13 Sallah S, Thomas DP, Roberts HR. Warfarin and heparin-induced skin necrosis and the purple toe syndrome: infrequent complications of anticoagulant treatment. Thromb Haemost 1997; 78: 785-790.
• 14 Moll S, Huffman J. Cholesterol emboli associated with warfarin treatment. Am J Hematol 2004; 77: 194-195.
• 15 Wright RS, Quinones-Baldrich WJ, Anders AJ. et al. Pleural effusion associated with ipsilateral breast and arm edema as a complication of subclavian vein catheterization and arteriovenous fistula formation for hemodialysis. Chest 1994; 106: 950-952.
• 16 Schiffer JT, Jefferson BK. Cases from the Osler Medical Service at Johns Hopkins University. Am J Med 2003; 115: 404-406.
• 17 Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents--St.. Louis, Missouri, 2002–2006. MMWR Morb Mortal Wkly Rep 2007; 56: 137-141.
• 18 Lim YL, Lee HY, Low SC. et al. Possible role of gadolinium in nephrogenic systemic fibrosis: report of two cases and review of the literature. Clin Exp Dermatol 2007; 32: 353-358.
• 19 Scheinfeld N. Nephrogenic fibrosing dermopathy: a comprehensive review for the dermatologist. Am J Clin Dermatol 2006; 7: 237-47.
• 20 http://www.icnfdr.org (last accessed May 2, 2007).
• 21 Tiwari A, Cheng KS, Button M, Myint F, Hamilton G. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg 2003; 138: 152-161.
• 22 Carver C, Brice G, Mansour S. et al. Three children with Milroy disease and de novo mutations in VEGFR3. Clin Genet 2007; 71: 187-189.