Thrombotic thrombocytopenic purpura associated with myelodysplastic syndrome
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
Thrombotic thrombocytopenic purpura (TTP), a life-threatening disseminated thrombotic microangiopathy (TMA) syndrome, is characterized by bicytopenias with thrombocytopenia and hemolytic anemia (HA) with schistocytes, neurological disturbances, acute renal failure, and pyrexia. It has an incidence of 2–7 per million person per year with a peak incidence between the ages of 30 and 40 [1, 2]. Myelodysplastic syndrome (MDS) is also characterized by multilineage cytopenias due to ineffective hematopoiesis, and predominantly affects the elderly with a peak incidence between the ages of 60 and 75. Compared with other disorders, such as aplastic anemia, phenomena secondary to leukemias, autoimmune disorders, such as systemic lupus erythematosus or Evans syndrome, viral infection, or adverse drug effects, both MDS and TTP are less common as causatives for multilineage cytopenias in young patients. This report describes a rare case of MDS associated with TTP in a young female presented with mul ...
- Rock GA. Management of thrombotic thrombocytopenic purpura. Br J Haematol. 2000;109:496–507. doi:10.1046/j.1365-2141.2000.01941.x. CrossRef
- Torok TJ, Holman RC, Chorba TL. Increasing mortality from thrombotic thrombocytopenic purpura in the United States-analysis of national mortality data, 1968–1991. Am J Hematol. 1995;l50:84–90. CrossRef
- Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood. 2002;100:2292–302. doi:10.1182/blood-2002-04-1199. CrossRef
- Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lämmle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89:3097–103.
- Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585–94. doi:10.1056/NEJM199811263392203. CrossRef
- Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413:488–94. doi:10.1038/35097008. CrossRef
- Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991;325:393–7.
- Fontana S, Kremer Hovinga JA, Lämmle B, Mansouri Taleghani B. Treatment of thrombotic thrombocytopenic purpura. Vox Sang. 2006;90:245–54. doi:10.1111/j.1423-0410.2006.00747.x. CrossRef
- Shumak KH, Rock GA, Nair RC. Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura. Canadian Apheresis Group. Ann Intern Med. 1995;122:569–72.
- Doudenko-Pirozzolo I, Booth R. A 20-year-old man with a history of hemoptysis and purpura. Arch Pathol Lab Med. 2001;125:835–7.
- Leone G, Sica S, De Stefano V, et al. Acute onset of juvenile myelodysplastic syndrome mimicking thrombotic thrombocytopenic purpura and rapidly evolving in overt myeloid leukemia. Am J Hematol. 1992;41:64–5. doi:10.1002/ajh.2830410114. CrossRef
- Marisavljevic D, Kraguljac N, Rolovic Z. Immunologic abnormalities in myelodysplastic syndrome: clinical features and characteristics of the lymphoid population. Med Oncol. 2006;23:385–92. doi:10.1385/MO:23:3:385. CrossRef
- Thrombotic thrombocytopenic purpura associated with myelodysplastic syndrome
International Journal of Hematology
Volume 88, Issue 4 , pp 457-459
- Cover Date
- Print ISSN
- Online ISSN
- Springer Japan
- Additional Links
- Industry Sectors
- Author Affiliations
- 1. Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, 602-8566, Japan
- 2. Division of Hematology, Kyoto Second Red Cross Hospital, 355-5, Haruobi-cho, Kamigyo-ku, Kyoto, 602-8026, Japan