Case Report

Thrombotic Thrombocytopenic Purpura Without Schistocytes on the Peripheral Blood Smear

Authors: Sumanth R. Daram, MD, Marie Philipneri, MD, Nidhi Puri, BS, Bahar Bastani, MD

Abstract

A hallmark of the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) is evidence of microangiopathic hemolytic anemia. The presence of schistocytes on the peripheral blood smear, elevated plasma lactic dehydrogenase, and decreased haptoglobin concentration are used as evidence of microangiopathic hemolytic anemia to make a diagnosis of TTP. This report describes a case of recurrence of TTP in the absence of schistocytes in the peripheral blood smear during the recurrent episode. Although careful attention should be paid to microscopic examination of a blood smear in any patient presenting with acute renal failure and thrombocytopenia, this case emphasizes the need to consider TTP-hemolytic uremic syndrome in the differential diagnosis, even in the absence of peripheral schistocytosis.


Key Points


* Renal involvement is a hallmark of thrombotic thrombocytopenic purpura (TTP).


* Absence of schistocytes on the peripheral blood smear does not rule out TTP.


* Prompt institution of plasma exchange therapy is critical in management of TTP.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Rock GA. Management of thrombotic thrombocytopenic purpura. Br J Haematol 2000;109:496–507.
 
2. Asada Y, Sumiyoshi A, Hayashi T, et al. Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen. Thromb Res1985;38:469–479.
 
3. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998;339:1578–1584.
 
4. Tsai H-M, Lian EC-Y. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998;339:1585–1594.
 
5. Zheng X, Chung D, Takayama TK, et al. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem2001;276:41059–41063.
 
6. Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001;413:488–494.
 
7. 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;50:84–90.
 
8. Elliott MA, Nichols WL. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Mayo Clinic Proc 2001;76:1154–1162.
 
9. Rock G, Kelton JG, Shumak KH, et al. Laboratory abnormalities in thrombotic thrombocytopenic purpura: Canadian Apheresis Group. Br J Haematol 1998;103:1031–1036.
 
10. Moake JL. Thrombotic microangiopathies. N Engl J Med 2002;347:589–600.
 
11. Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 2001;60:831–846.
 
12. Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries, an undescribed disease. Arch Intern Med 1925;36:89–93.
 
13. Brilliant SE, Lester PA, Ohno AK, et al. Hemolytic-uremic syndrome without evidence of microangiopathic hemolytic anemia on peripheral blood smear. South Med J 1996;89:342–345.
 
14. Scully RE, Mark EJ, McNeely WF, et al. Case records of the Massachusetts General Hospital: weekly clinicopathological exercises: case 41–1990. N Engl J Med 1990;323:1050–1061.
 
15. Morel-Maroger L, Kanfer A, Solez K, et al. Prognostic importance of vascular lesions in acute renal failure with microangiopathic hemolytic anemia (hemolytic-uremic syndrome): clinicopathologic study in 20 adults. Kidney Int 1979;15:548–558.
 
16. Fava S, Galizia AC. Thrombotic thrombocytopenic purpura-like syndrome in the absence of schistocytes. Br J Haematol 1995;89:643–644.