Sequential Immune Thrombocytopenia (ITP) and Thrombotic Thrombocytopenic Purpura (TTP) in an Elderly Male Patient with Primary Sjogren’s Syndrome: When in Doubt, Use the PLASMIC Score

Miller, Devon; Krenzer, Joseph A.; Kenkre, Vaishalee P.; Rose, William Nicholas

doi:10.1155/2021/6869342

Cited by 9 publications

(9 citation statements)

References 24 publications

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“…In this regard, it is conceivable that the cause of TTP in our case was related to primary SS. To the best of our knowledge, 19 cases of TTP with SS have been reported in the past 55 years, and only 7 of them were diagnosed with SS prior to TTP, as in our case [ 13 , 14 ]. In an analysis of 261 cases of acquired TTP, 56 patients were reported to have developed autoimmune diseases, including systemic lupus erythematosus (SLE) and SS [ 15 ].…”

Section: Discussionmentioning

confidence: 81%

Sudden Cardiac Death in a Patient with Thrombotic Thrombocytopenic Purpura: A Case Report

et al. 2022

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A 49-year-old female was admitted to our hospital with malaise and gross hematuria. As ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13) activity was absent and the ADAMTS13 inhibitor was detected, she was diagnosed with acquired thrombotic thrombocytopenic purpura (TTP). In addition to plasma exchange and corticosteroid therapy, she received rituximab therapy for inhibitor boosting but died suddenly of a cardiac arrest on day 9. The postmortem revealed microvascular platelet thrombi in multiple organs. In this case, the deterioration of the patient’s clinical status was considered to have been caused by inhibitor boosting-induced systemic microvascular occlusion. In particular, her sudden death may have been due to cardiovascular microthrombosis. Since inhibitor boosting can cause TTP patients to deteriorate rapidly, it is crucial to manage TTP patients who undergo inhibitor boosting appropriately. The monitoring of cardiac complications in TTP patients may also be essential, especially in the acute phase.

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Section: Discussionmentioning

confidence: 81%

Sudden Cardiac Death in a Patient with Thrombotic Thrombocytopenic Purpura: A Case Report

et al. 2022

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“…The PLASMIC score was high. The PLASMIC score is a seven-component clinical prediction tool that was developed to reliably assess the pretest probability of severe ADAMTS13 deficiency [ C statistic 0.96, 95% confidence interval (CI) 0.92–0.98] [ 9 , 10 ]. TTP was suspected.…”

Section: Resultsmentioning

confidence: 99%

“…Therefore, differential diagnosis of ITP and TTP is essential in clinical practice. Concurrence is rare; however, previous case reports have shown that ITP and TTP can occur concurrently in acquired immune deficiency syndrome (AIDS), pregnancy, and Sjögren’s syndrome [ 7 – 9 ]. We report herein a case of concurrent ITP and TTP in a patient without knowledge of any underlying diseases.…”

Section: Introductionmentioning

confidence: 99%

Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature

Lin

Huang

et al. 2023

J Med Case Reports

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Background Immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura are both causes of thrombocytopenia. Recognizing thrombotic thrombocytopenic purpura is crucial for subsequent treatment and prognosis. In clinical practice, corticosteroids and rituximab can be used to treat both immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura; plasma exchange therapy is the first-line treatment in thrombotic thrombocytopenic purpura, while corticosteroids are strongly recommended as first-line treatment in immune thrombocytopenic purpura. The differential diagnosis of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is essential in clinical practice. However, case reports have suggested that immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura can occur concurrently. Case presentation We report the case of a 32-year-old Asian female without previous disease who presented with pancytopenia, concurrent with immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura. The morphology of the megakaryocytes in the bone marrow indicated immune-mediated thrombocytopenia. The patient received glucocorticoid treatment, and her platelet count increased; however, schistocytes remained high during the course of the therapy. Further investigations revealed ADAMTS13 activity deficiency and positive ADAMTS13 antibodies. The high titer of antinuclear antibody and positive anti-U1-ribonucleoprotein/Smith antibody indicated a potential autoimmune disease. However, the patient did not fulfill the current criteria for systemic lupus erythematosus or mixed connective tissue disease. The patient responded well to plasma exchange therapy, and her platelet count remained normal on further follow-up. Conclusions Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is rare, but clinicians should be aware of this entity to ensure prompt medical intervention. Most of the reported cases involve young women. Human immunodeficiency virus infection, pregnancy, and autoimmune disease are the most common underlying conditions.

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“…23 ITP is an acquired hematologic disorder caused by immunemediated destruction of platelets, and TTP caused by an acquired autoantibody that leads to decreased activity of the von Willebrand factor-cleaving protease ADAMTS13 that results in hemolytic anemia and severe thrombocytopenia. 24 Symptoms appear related to the severity and number of platelets, in mild case (platelet counts less then 20 x 109/L) petechiae and purpura may appear, in more severe cases (platelet counts less then 10 x 109/L) mucous bleeding can occur in the oral cavity, gastrointestinal and genitourinary tracts . Intracerebral hemorrhage, although rare, is the most common cause of death.…”

Section: Platelet Disordersmentioning

confidence: 99%

Diathesis Hemorrhagic, Coagulation and Fibrinolytic System

Putri¹,

Diansyah

Ashariati

et al. 2022

BHSJ

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Bleeding is one of the most common complaints when coming to the hospital which can be mild to life-threatening. The balance of the impaired hemostasis system allows for abnormal bleeding such as hemorrhagic diathesis. Balance between blood clotting and bleeding is always maintained in the body under normal physiology. The coagulation system stops existing bleeding with vasoconstricts of blood vessels and the formation of early platelet plugs, this blockage is strengthened by the presence of a cascade of coagulations to form stable and sturdy blockages. Once bleeding has stopped, the fibrinolytic pathway is initiated to dissolve the blood clot to restore normal blood flow. balance the coagulants, fibrinolytic and inhibitor systems, creating a perfect physiological balance. Hemostatic imbalance is a global problem that can lead to thrombosis or hemorrhage.

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Sequential Immune Thrombocytopenia (ITP) and Thrombotic Thrombocytopenic Purpura (TTP) in an Elderly Male Patient with Primary Sjogren’s Syndrome: When in Doubt, Use the PLASMIC Score

Cited by 9 publications

References 24 publications

Sudden Cardiac Death in a Patient with Thrombotic Thrombocytopenic Purpura: A Case Report

Sudden Cardiac Death in a Patient with Thrombotic Thrombocytopenic Purpura: A Case Report

Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature

Diathesis Hemorrhagic, Coagulation and Fibrinolytic System

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