Dominika Grzegowska, Anna Masiak, Zenobia Czuszyńska, Marcin Ziętkiewicz, Wojciech Homenda, Michalina Pejska, Zbigniew Zdrojewski


Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) are two separate diseases, whose clinical symptoms may be similar. The coexistence of these two diseases is very rare. We present the case report of a 29-year-old female patient with simultaneous diagnosis of TTP and SLE. The purpose of this article is to draw attention to the diagnostic difficulties which may result from the co-existence of both diseases.


thrombotic thrombocytopenic purpura; systemic lupus erythematosus; metalloproteinase ADAMTS13; thrombotic microangiopathy; overlap syndrome; plasma exchange (PEX)

Full Text:

PDF (Język Polski)


Moschcowitz E.: Hyaline thrombosis of the terminal arterioles and capillaries: a hither to undescribed disease. Proc NY Pathol Soc. 1924, 24, 21–24.

Furlan M., Robles R., Lammle B.: Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996, 87 (10), 4223–4234.

Kremer Hovinga J.A., Studt J.D., Alberio L., Lammle B.: Von Willebrand factor cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience. Semin Hematol. 2004, 41 (1), 75–82.

Ruggenenti P., Remuzzi G.: The pathophysiology and management of thrombotic thrombocytopenic purpura. Eur J Haematol. 1996, 56 (4), 191–207.

Scully M., Hunt B.J., Benjamin S., Liesner R., Rose P., Peyvandi F. et al.: Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012, 158 (3), 323–335.

Petri M., Orbai A.M., Alarcon G.S., Gordon C., Merrill J.T., Fortin P.R. et al.: Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012, 64 (8), 2677–2686.

Hamasaki K., Mimura T., Kanda H., Kubo K., Setoguchu K., Satoh T. et al.: Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report and literature review. Clin Rheumatol. 2003, 22 (4-5), 355–358.

George P., Das J., Pawar B., Kakkar N.: Thrombotic thrombocytopenic purpura and systemic Lupus erythematosus: Successful management of a rare presentation. Indian J Crit Care Med. 2008, 12 (3), 128–131.

Kwok S.K., Ju J.H., Cho C.S., Kim H.Y., Park S.H.: Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus. 2009, 18 (1), 16–21.

Ali Taha A.H., Elbadry M.I., Tawfik M.A.: A case report of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: overlapping features. Am J Med Case Rep. 2014, 10 (2), 206–213.

Coppo P., Veyradier A.: Thrombotic microangiopathies: towards a pathophysiology-based classification. Cardiovasc Hematol Disord Drug Targets. 2009, 9 (1), 36–50.

Mok C.C., Lau C.S.: Pathogenesis of systemic lupus erythematosus. J Clin Pathol. 2003, 56 (7), 481–490.

Bellone M.: Autoimmune Disease: Pathogenesis. In: Encyclopedia of life sciences. John Wiley & Sons, New York 2010.

Hahn B.H.: Antibodies to DNA. N Engl J Med. 1998, 338 (19), 1359–1368.

Cheung W.Y.: Thrombotic thrombocytopenic purpura and systemic lupus erythematosus – distinct entities or overlapping syndromes? Transfus Apher Sci. 2006, 34 (4), 263–266.

Mannucci P.M., Vanoli M., Forza I., Canciani M.T., Scorza R.: Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis). Haematologica. 2003, 88 (8), 914–918.

Shah A.A., Higgins J.P., Chakravarty E.F.: Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties. Nat Clin Pract Rheumatol. 2007, 3 (6), 357–362.

Al-Homood I.A.: Thrombosis in systemic lupus erythematosus: a review article. ISRN Rheumatol. 2012, Article ID 428269.

Hrycaj P., Cieślak D.: Zajęcie układu krwiotwórczego w przebiegu tocznia rumieniowatego układowego. Reumatologia. 2013, 51 (1), 31–35.

George J.N., Vesely S.K., James J.A.: Overlapping features of thrombotic thrombocytopenic purpura and systemic lupus erythematosus. South Med J. 2007, 100 (5), 512–514.

Scully M., McDonald V., Cavenagh J., Hunt B.J., Longair I., Cohen H. et al.: A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011, 118 (7), 1746–1753.

Schiviz A., Wuersch K., Piskernik C., Dietrich B., Hoellriegl W., Rottensteiner H. et al.: A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13. Blood. 2012, 119 (25), 6128–6135.

Zdrojewski Z.: Nowoczesne metody diagnostyczne i terapeutyczne nefropatii toczniowej. Ann Acad Med Stetin. 2011, 57, 3, 39–48.


Copyright (c) 2015 Dominika Grzegowska, Anna Masiak, Zenobia Czuszyńska, Marcin Ziętkiewicz, Wojciech Homenda, Michalina Pejska, Zbigniew Zdrojewski

License URL: