JOURNAL ARTICLE

Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab

Xinglong Zheng, Arnel M Pallera, Lawrence T Goodnough, J Evan Sadler, Morey A Blinder
Annals of Internal Medicine 2003 January 21, 138 (2): 105-8
12529092

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) in adults is usually caused by autoantibody inhibitors of ADAMTS13. Treatment with plasma exchange is often effective but does not address the underlying autoimmune process.

OBJECTIVE: To report the efficacy of intensive immunosuppressive therapy in refractory TTP.

DESIGN: Case report.

SETTING: University medical center.

PATIENT: 42-year-old woman with chronic relapsing TTP.

INTERVENTION: Immunosuppression therapy with rituximab and cyclophosphamide.

MEASUREMENTS: ADAMTS13 activity and inhibitors and hematologic variables for TTP.

RESULTS: For 19 months, the patient had relapsing thrombotic microangiopathy despite plasma exchange; splenectomy; and therapy with vincristine, prednisone, and cyclosporine. ADAMTS13 activity was low, and tests detected an IgG inhibitor that recognized the metalloprotease domain of recombinant ADAMTS13. After treatment with rituximab and cyclophosphamide, the disease remitted, ADAMTS13 levels normalized, and the inhibitor was undetectable. The patient has required no treatment for 13 months.

CONCLUSION: Intensive immunosuppressive therapy can lead to sustained clinical remission in patients with refractory autoimmune TTP.

Full Text Links

Find Full Text Links for this Article

Discussion

You are not logged in. Sign Up or Log In to join the discussion.

Trending Papers

Remove bar
Read by QxMD icon Read
12529092
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"