Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)

Thrombotic thrombocytopenic purpura (TTP) is a nonimmune, microangiopathic hemolytic anemia, associated with thrombocytopenia, fever, neurologic, or renal dysfunction. Plasma exchange (PEX) with or without steroids constitutes first-line therapy in TTP. However, a subset of the patients may be refra...

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Main Authors: B Saroj Kumar Prusty, Kiran Kumar Ramineni, G Krishna Mohan Reddy, Sashi Kiran Annavarajula, Majed Abdul Basit Momin
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-01-01
Series:APIK Journal of Internal Medicine
Subjects:
Online Access:http://www.ajim.in/article.asp?issn=2666-1802;year=2023;volume=11;issue=2;spage=128;epage=131;aulast=Prusty
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author B Saroj Kumar Prusty
Kiran Kumar Ramineni
G Krishna Mohan Reddy
Sashi Kiran Annavarajula
Majed Abdul Basit Momin
author_facet B Saroj Kumar Prusty
Kiran Kumar Ramineni
G Krishna Mohan Reddy
Sashi Kiran Annavarajula
Majed Abdul Basit Momin
author_sort B Saroj Kumar Prusty
collection DOAJ
description Thrombotic thrombocytopenic purpura (TTP) is a nonimmune, microangiopathic hemolytic anemia, associated with thrombocytopenia, fever, neurologic, or renal dysfunction. Plasma exchange (PEX) with or without steroids constitutes first-line therapy in TTP. However, a subset of the patients may be refractory to PEX. Rituximab appears to be an alternative effective therapy for refractory or relapsing TTP. Here, we report a case of TTP in a 43-year-old female presented with fever, generalized weakness, headache, vomiting, and ecchymotic patches over forearms and upper chest for 7 days along with one episode of seizure. The laboratory evaluation revealed severe thrombocytopenia, anemia, and indirect hyperbilirubinemia with peripheral blood smear showing schistocytes (fragmented red blood cells). Initial therapy with multiple PEXs along with parenteral corticosteroids resulted in only minimal improvement of platelet count. Subsequently, rituximab was administered which helped in normalization of platelet count and overall clinical improvement. This case highlights the importance of timely utilization of second-line drugs such as rituximab in refractory TTP.
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spelling doaj.art-c12146e8892e4813a79890f5bff7ed142023-05-18T04:59:01ZengWolters Kluwer Medknow PublicationsAPIK Journal of Internal Medicine2666-18022666-18102023-01-0111212813110.4103/ajim.ajim_56_21Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)B Saroj Kumar PrustyKiran Kumar RamineniG Krishna Mohan ReddySashi Kiran AnnavarajulaMajed Abdul Basit MominThrombotic thrombocytopenic purpura (TTP) is a nonimmune, microangiopathic hemolytic anemia, associated with thrombocytopenia, fever, neurologic, or renal dysfunction. Plasma exchange (PEX) with or without steroids constitutes first-line therapy in TTP. However, a subset of the patients may be refractory to PEX. Rituximab appears to be an alternative effective therapy for refractory or relapsing TTP. Here, we report a case of TTP in a 43-year-old female presented with fever, generalized weakness, headache, vomiting, and ecchymotic patches over forearms and upper chest for 7 days along with one episode of seizure. The laboratory evaluation revealed severe thrombocytopenia, anemia, and indirect hyperbilirubinemia with peripheral blood smear showing schistocytes (fragmented red blood cells). Initial therapy with multiple PEXs along with parenteral corticosteroids resulted in only minimal improvement of platelet count. Subsequently, rituximab was administered which helped in normalization of platelet count and overall clinical improvement. This case highlights the importance of timely utilization of second-line drugs such as rituximab in refractory TTP.http://www.ajim.in/article.asp?issn=2666-1802;year=2023;volume=11;issue=2;spage=128;epage=131;aulast=Prustyadamts13 enzymecryo-poor plasma exchangemicroangiopathic hemolytic anemiarefractory thrombotic thrombocytopenic purpurarituximab
spellingShingle B Saroj Kumar Prusty
Kiran Kumar Ramineni
G Krishna Mohan Reddy
Sashi Kiran Annavarajula
Majed Abdul Basit Momin
Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)
APIK Journal of Internal Medicine
adamts13 enzyme
cryo-poor plasma exchange
microangiopathic hemolytic anemia
refractory thrombotic thrombocytopenic purpura
rituximab
title Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)
title_full Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)
title_fullStr Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)
title_full_unstemmed Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)
title_short Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)
title_sort refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody rituximab
topic adamts13 enzyme
cryo-poor plasma exchange
microangiopathic hemolytic anemia
refractory thrombotic thrombocytopenic purpura
rituximab
url http://www.ajim.in/article.asp?issn=2666-1802;year=2023;volume=11;issue=2;spage=128;epage=131;aulast=Prusty
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AT kirankumarramineni refractorythromboticthrombocytopenicpurpuratreatedsuccessfullywithmonoclonalantibodyrituximab
AT gkrishnamohanreddy refractorythromboticthrombocytopenicpurpuratreatedsuccessfullywithmonoclonalantibodyrituximab
AT sashikiranannavarajula refractorythromboticthrombocytopenicpurpuratreatedsuccessfullywithmonoclonalantibodyrituximab
AT majedabdulbasitmomin refractorythromboticthrombocytopenicpurpuratreatedsuccessfullywithmonoclonalantibodyrituximab