scholarly journals Thrombotic thrombocytopenic purpura-like syndrome associated with systemic lupus erythematosus: combined treatment with plasmapheresis and fresh frozen plasma infusion

1992 ◽  
Vol 7 (1) ◽  
pp. 66 ◽  
Author(s):  
Gyu Taek Lim ◽  
Sung Soo Kim ◽  
Soo Hun Park ◽  
Won Oh Choo ◽  
Dong Heon Kang ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Combined Treatment ◽  
Fresh Frozen Plasma ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
Plasma Infusion ◽  
Fresh Frozen ◽  
Frozen Plasma

Systemic lupus erythematosus with C1q deficiency: treatment with fresh frozen plasma

Lupus ◽  
2017 ◽  
Vol 27 (1) ◽  
pp. 134-138 ◽  
Author(s):  
Z Ekinci ◽  
K Ozturk
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Fresh Frozen Plasma ◽  
Complement C3 ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
Fresh Frozen ◽  
C1q Deficiency ◽  
Frozen Plasma

Treatment and outcome of systemic lupus erythematosus (SLE) in C1q deficient patients are rarely reported. The aim of this report is to share our experience about the course of management of three cases diagnosed as SLE with C1q deficiency, in light of present literature. Initial and dominant complaints of three cases from two different families were cutaneous manifestations. One patient was also diagnosed with arthritis and thrombocytopenia. Antinuclear antibody was positive in all cases, whereas anti-dsDNA was negative with normal levels of complement C3, C4 and decreased CH50 activity. C1QA gene of two patients had homozygous nonsense mutation (c.622 > T/p.Gln208Ter). Previously, all of them had been treated with steroids, hydroxychloroquine and methotrexate or azathioprine. It was learned that they had responded only to high dosage prednisolone and their symptoms flared up during dosage reduction even under methotrexate or azathioprine. All symptoms of all three cases improved by daily fresh frozen plasma (FFP) infusions, and once cutaneous lesions subsided, the infusions were reduced to a frequency that would prevent the flare up of the symptoms. Literature search revealed seven reports on fresh frozen plasma treatment in SLE with C1q deficient patients. In this report, it is concluded that severe cutaneous lesions, as seen in these C1q deficient SLE patients, cannot be controlled with conventional immunosuppressive treatment. Instead, regular fresh frozen plasma infusions are proposed as a more reasonable method of treatment.

scholarly journals Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
João Tadeu Damian Souto Filho ◽  
Philipe Vianna de Barros ◽  
Aline Maria Yamaguti Rios Paes da Silva ◽  
Fernanda Alves Barbosa ◽  
Gustavo Fernandes Ribas
Keyword(s):  
Autoimmune Diseases ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Lupus Erythematosus ◽  
High Volume ◽  
Fresh Frozen Plasma ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Fresh Frozen ◽  
Frozen Plasma

Thrombotic thrombocytopenic purpura (TTP) is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD) is extremely rare and has only been described in nine patients. We describe the case of a 42-year-old female with MCTD who developed thrombocytopenia, microangiopathic hemolytic anemia, fever, and neurological symptoms. The patient had a good clinical evolution with infusion of high volume of fresh frozen plasma, steroid therapy, and support in an intensive care unit. Although the occurrence of TTP is rare in MCTD patients, it is important to recognize TTP as a cause of thrombocytopenia and hemolytic anemia in any patient with autoimmune diseases. Prompt institution of treatment remains the cornerstone of treatment of TTP even if plasma exchange is not available like what frequently happens in developing countries.

scholarly journals Spinal and cerebral hematoma in systemic lupus erythematosus and antiphospholipid syndrome: is drug interaction the culprit?

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Emine Duran ◽  
Emre Bilgin ◽  
Ertuğrul Çağrı Bölek ◽  
Oğuzhan Fırat ◽  
Elif Bulut ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Fresh Frozen Plasma ◽  
Systemic Lupus ◽  
Herbal Products ◽  
Spinal Subdural Hematoma ◽  
Anticoagulant Drug ◽  
Fresh Frozen ◽  
Cerebral Hematoma

Abstract Objectives Thrombotic events are common in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Warfarin is the most commonly used anticoagulant drug for thrombosis treatment, but it is can interact with many drugs, foods, or medicinal herbs. Herein, we presented a case with SLE and APS who was complicated by spinal and cerebral hematoma as a result of warfarin interaction. Case presentation Spinal subdural hematoma and frontal intraparenchymal hematoma were occurred in our patient, who was in remission for 2 years with rituximab, hydroxychloroquine and warfarin. We learned that she had been using some herbal products (shepherd’s purse and horsetail) and phenyramidol for a few days. Spinal and cerebral hematomas caused by the interaction of phenyramidol and warfarin were treated with fresh frozen plasma and vitamin K without the need for surgery. Conclusions The drug interactions with warfarin can cause fatal hemorrhagic or thrombotic events. Especially, the patients with SLE and/or APS using warfarin should be warned not to use different medications or herbal agents.

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.

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