Catastrophic anti-phospholipid syndrome with Libman-Sacks endocarditis following eltrombopag therapy for immune thrombocytopenic purpura: A case report

Lupus ◽  
2021 ◽  
pp. 096120332110651
Author(s):  
Sara Dichtwald ◽  
Avraham Meyer ◽  
Nisim Ifrach
Keyword(s):  
Case Report ◽  
Adverse Events ◽  
Lupus Erythematosus ◽  
Immune Thrombocytopenic Purpura ◽  
Intravenous Immunoglobulins ◽  
Young Female ◽  
The European Union ◽  
Positive Serology ◽  
Thrombocytopenic Purpura ◽  
Refractory Itp

Background Immune thrombocytopenic purpura (ITP) is an autoimmune disease, with accelerated destruction of platelets, estimated to affect 1.6–3.9 in 100,000 adults every year in the European Union. Glucocorticoids and intravenous immunoglobulins are common drug therapies. In refractory cases, drugs that enhance thrombopoiesis may be used. Eltrombopag is a thrombopoietin receptor agonist, known to increase platelet count in patients with refractory ITP. Thrombotic adverse events have been described in association with Eltrombopag administration. Case report A young female patient of Ethiopian ancestry with systemic lupus erythematosus, triple Antiphospholipid (APLA) positive serology and refractory ITP who received Eltrombopag and 2 weeks later developed catastrophic APLA syndrome with severe Libman-Sacks endocarditis of the mitral and aortic valves, multiple intracerebral infracts and arterial thrombosis of the left upper limb. Conclusion Eltrombopag is a salvage drug, used in refractory ITP. Thrombotic adverse events, some of which may be life-threatening, are a possible complication, especially in high-risk patients.

Combination immunosuppressant therapy for patients with chronic refractory immune thrombocytopenic purpura

Blood ◽  
2010 ◽  
Vol 115 (1) ◽  
pp. 29-31 ◽  
Author(s):  
Donald M. Arnold ◽  
Ishac Nazi ◽  
Aurelio Santos ◽  
Howard Chan ◽  
Nancy M. Heddle ◽  
...  
Keyword(s):  
Platelet Count ◽  
Mycophenolate Mofetil ◽  
Adverse Events ◽  
Observational Study ◽  
Immune Thrombocytopenic Purpura ◽  
Treatment Options ◽  
Initial Response ◽  
Immunosuppressant Therapy ◽  
Thrombocytopenic Purpura ◽  
Refractory Itp

Abstract Treatment options for patients with chronic refractory immune thrombocytopenic purpura (ITP) are limited. Because combination immunosuppressant therapy appeared to be effective in ITP and other disorders, we used this approach in patients with particularly severe and refractory ITP. In this retrospective, observational study, we determined the response (platelet count above 30 × 109/L and doubling of baseline) among 19 refractory ITP patients. Treatment consisted of azathioprine, mycophenolate mofetil, and cyclosporine. The patients had failed a median of 6 prior treatments, including splenectomy (in all except 1). Of 19 patients, 14 (73.7%) achieved a response lasting a median of 24 months, after which time 8 (57.1%) relapsed. Of the 8 relapsing patients, 6 responded to additional treatments. Of the 14 patients who achieved an initial response, 2 (14.3%) remained in remission after eventually stopping all medications. Severe adverse events did not occur. Combination immunosuppressant therapy can produce a rise in the platelet count that is sometimes sustained in refractory ITP patients.

Single Dose of Anti-CD20 Monoclonal Antibody (Rituximab) Treatment in Adults with Refractory Immune Thrombocytopenic Purpura (ITP).

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1077-1077
Author(s):  
Eri Tanaka ◽  
Shuji Hayashi ◽  
Katsumichi Fujimaki ◽  
Hiroyuki Fujita
Keyword(s):  
Platelet Count ◽  
Single Dose ◽  
Gastrointestinal Bleeding ◽  
Immune Thrombocytopenic Purpura ◽  
Intravenous Immunoglobulins ◽  
Thrombocytopenic Purpura ◽  
Platelet Counts ◽  
History Of ◽  
Refractory Itp

Abstract Refractory Immune Thrombocytopenic Purpura (ITP) is a difficult disease to treat effectively and the mortality approaches nearly 10% over 10 years. Moreover, the side effect profile of chronic steroid administration is undesirable due to the multi-systemic actions of these drugs. Recently, it has been reported in the literature that Rituximab is effective treatment for chronic ITP and it has been used at a dose of 375mg/m2 weekly for up to four weeks, as with lymphoma therapy. Rituximab is an expensive treatment, but according to previous data, patients treated with this drug have responded with increased and sustained platelet counts following only one infusion. Based on this, we treated five refractory ITP patients with single-dose Rituximab and all responded well. Patient 1 is with a 15 year history of ITP and Patient 2 is with a 34 year long history of ITP. Following treatment with Rituximab, although there was an interval of up to 7 months, both eventually responded well. Patient 3 is a 93 year old male who presented to our hospital with an acute presentation of ITP which involved severe gastrointestinal bleeding and this proved to be refractory to both steroids and intravenous immunoglobulins, but responded very quickly to Rituximab within 24 days. Patient 4 is a 75 year old female with diabetes mellitus and three-vessel coronary artery disease who presented with a bleeding diathesis. She was treated with steroids, intravenous immunoglobulins, danazol and azathioprine but with no response. Single-dose Rituximab was effective within 20 days with an improvement in platelet counts. Patient 5 is a 51 year old male with a six year history of ITP who presented to our hospital with massive intraabdominal and gastrointestinal bleeding and after Rituximab therapy his platelet count responded appropriately after 45 days. These patient responses were not associated with prior response to therapy, age, previous splenectomy, duration of ITP or platelet count. All patients tolerated treatment well except one patient who developed SLE-nephrotic syndrome 15 months later although it cannot be proven that such therapy induced this collagen-vascular diseases because Rituximab can be used to actually treat such conditions. Three patients remained in remission for more than one year after just one dose of the Rituximab therapy. Even from our small number of refractory ITP patients treated with Rituximab, it is our experience that single-dose treatment is also effective in some cases of refractory ITP and its effect may continue to provide long-term remission whereby it is now possible to decrease and even stop long-term steroid treatment in such patients.

scholarly journals An Early Unexpected Immune Thrombotic Thrombocytopenic Purpura Relapse Associated with SARS-CoV-2 Infection: A Case Report and Literature Review

2021 ◽  
pp. 1-5
Author(s):  
Maya Kornowski Cohen ◽  
Liron Sheena ◽  
Yair Shafir ◽  
Vered Yahalom ◽  
Anat Gafter-Gvili ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Response To Therapy ◽  
Intravascular Hemolysis ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
History Of ◽  
Triggering Factor

SARS-CoV-2 has been reported as a possible triggering factor for the development of several autoimmune diseases and inflammatory dysregulation. Here, we present a case report of a woman with a history of systemic lupus erythematosus and antiphospholipid syndrome, presenting with concurrent COVID-19 infection and immune thrombotic thrombocytopenic purpura (TTP). The patient was treated with plasma exchange, steroids, and caplacizumab with initial good response to therapy. The course of both TTP and COVID-19 disease was mild. However, after ADAMTS-13 activity was normalized, the patient experienced an early unexpected TTP relapse manifested by intravascular hemolysis with stable platelet counts requiring further treatment. Only 3 cases of COVID-19 associated TTP were reported in the literature thus far. We summarize the literature and suggest that COVID-19 could act as a trigger for TTP, with good outcomes if recognized and treated early.

Case report: a pregnant woman with immune thrombocytopenic purpura and unusual red cell antibodies

2020 ◽  
Vol 11 (4) ◽  
pp. 153-155
Author(s):  
Carmela R. Nanton ◽  
Sharon M. Martin ◽  
Lloyd O. Cook ◽  
Patricia J. Larison
Keyword(s):  
Case Report ◽  
Pregnant Woman ◽  
Immune Thrombocytopenic Purpura ◽  
Red Cell ◽  
Thrombocytopenic Purpura

scholarly journals Immune Thrombocytopenic Purpura Secondary to Cytomegalovirus Infection: A Case Report

2015 ◽  
Vol 2 ◽  
Author(s):  
Bessy S. Flores-Chang ◽  
Carlos E. Arias-Morales ◽  
Francis G. Wadskier ◽  
Sorab Gupta ◽  
Nicoleta Stoicea
Keyword(s):  
Case Report ◽  
Immune Thrombocytopenic Purpura ◽  
Cytomegalovirus Infection ◽  
Thrombocytopenic Purpura

scholarly journals Autoimmune hemolytic anemia associated with vitamin B12 deficiency and viral illness in DiGeorge syndrome. Case report and literature review.

2021 ◽  
Author(s):  
Zohaib Yousaf ◽  
Fateen Ata ◽  
Phool Iqbal ◽  
Bassam Muthanna ◽  
Adeel Khan ◽  
...  
Keyword(s):  
Case Report ◽  
Digeorge Syndrome ◽  
Vitamin B12 Deficiency ◽  
Intravenous Immunoglobulins ◽  
Chromosome 22 ◽  
Thrombocytopenic Purpura ◽  
Immunodeficiency Disorder ◽  
Viral Illness ◽  
B12 Deficiency ◽  
Intravenous Steroids

DiGeorge syndrome, an immunodeficiency disorder due to long arm microdeletion of chromosome 22. Isolated hemolysis is rarely seen in DiGeorge syndrome and is usually reported in conjunction with idiopathic thrombocytopenic purpura. We report a case of DiGeorge syndrome with AIHA, which was successfully managed by intravenous steroids and intravenous immunoglobulins.

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