Outcome of Thrombotic Thrombocytopenic Purpura Patients: Single Center Experience

Çeneli̇, Özcan; Yilmaz, Seda; Karaselek, Mehmet Ali; Çamlı, Kazım

doi:10.4274/tjh.galenos.2019.2019.0048

Cited by 3 publications

(1 citation statement)

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“…TTP is a thrombotic microangiopathy (TMA) caused by reduced activity of the vWF-cleaving protease ADAMTS13. It is a medical emergency that is almost always fatal if appropriate treatment is not initiated promptly [5].…”

Section: Discussionmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: Revisiting a Miss and an Inevitable Consequence

Gogia¹,

Gbujie²,

Benge³

et al. 2020

Cureus

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Thrombotic thrombocytopenic purpura (TTP) is typically characterized by the symptomatic pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, and renal failure. Atypical TTP is the diagnosis used to describe the subset of patients with TTP who present with symptoms that deviate from the classic pentad. We report a case an 86-year-old woman who presented to the emergency department complaining of chest pain for one day. She was reportedly on antibiotics for sinus infection. Physical examination revealed multiple bilateral superficial hematomas, predominantly on her extremities. On admission, her lab values were as follows: platelet count of 6,000/cubic millimeter, hemoglobin of 10.4 grams/deciliter, leukocyte count of 5100 cells/cubic millimeter, total bilirubin of 2.3 milligrams/deciliter, and troponin-I of 5.190 nanograms/milliliter. Peripheral blood smear was normal and did not reveal any schistocytes. The patient was admitted to the intensive care unit with a diagnosis of a non-ST-elevation myocardial infarction and a presumed diagnosis of immune thrombocytopenic purpura from antibiotic use. She was treated with intravenous solumedrol and a high-intensity statin. On the third day of her admission, the patient's mental functioning deteriorated and was intubated to protect her airway. A second peripheral smear revealed schistocytes, and subsequent laboratory studies supported the diagnosis of TTP. Plasma exchange therapy was planned. However, the patient succumbed to cardiac arrest before it could be initiated. The diagnosis was later confirmed with an ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) assay. This case serves as an example of one of the many ways in which TTP can present, and emphasizes the importance of considering TTP as a differential diagnosis.

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Section: Discussionmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: Revisiting a Miss and an Inevitable Consequence

Gogia¹,

Gbujie²,

Benge³

et al. 2020

Cureus

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Evaluation Of Clinical, Laboratory and Treatment Results Of Our Patients With Thrombotic Thrombocytopenic Purpura.

MEŞELİ,

ÜSKÜDAR TEKE,

ANDIC

et al. 2024

Osmangazi̇ Journal of Medicine

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Trombotik trombositopenik purpura (TTP) klinik olarak ADAMTS-13 proteazının eksik aktivitesinden kaynaklanır. TTP’nin klasik pentadı mikroanjiyopatik hemolitik anemi (MAHA), trombositopeni, nörolojik bulgular, ateş ve böbrek yetmezliğidir. Tedavide plazma değişimi (PD), kortikosteroidler, rituksimab kullanılmakta ve son yıllarda kaplasizumab tedavi kombinasyonunda yer almaktadır. Çalışmamızın amacı yılda milyonda 3,7 ile 11 arasında yeni vakanın görüldüğü TTP hastalığına ait gerçek yaşam verilerine ulaşıp sonuçları tespit ederek literatürdeki az sayıdaki gerçek yaşam verilerine ait çalışmalar ile karşılaştırıp literatüre katkı sağlamaktır. Çalışmamızda hastanemizin İç Hastalıkları ABD, Hematoloji BD’da 1.Ocak.2008-1.Ocak.2023 tarihleri arasında 18 yaş ve üzerinde iken immün (i) TTP tanısı konulan 31 hastanın klinik ve laboratuvar bulguları ile tedavi sonuçları retrospektif olarak değerlendirildi. 31 hastanın 7’si erkek (%22,6) idi. Ortalama yaş 45,13 ±19,07 (16-83) yıl idi. Hastaların tamamında MAHA ve trombositopeni tespit edildi. Tedavide tüm hastalara PD uygulandı. Hastaneye başvurudan sonra PD’ye başlama süresi 24 (12-24) saat idi. Hastaların 16’sında (%57,1) 1.basamak tedaviye direnç görülmezken 12’sinde (%42,9) direnç mevcuttu. Direnç durumunda hastaların 10’una (%83,3) 4 doz 375 mg/m2/hafta rituksimab tedavisi verildi. Refrakter 12 hasta (%42,9) ile non-refrakter 16 hasta (%57,7) arasında yapılan değerlendirme sonucunda tanı anındaki total bilirubin (p=0.019) ve direkt bilirubin (p=0.031) değerleri arasında anlamlı fark saptandı. Mortal 7 hasta (%22,6) ile mortal seyretmeyen 24 hasta (%77,4) arasındaki değerlendirme sonucunda tanı anındaki yaş açısından anlamlı fark saptandı (p=0.034). Sonuç olarak TTP’nin kadınlarda erkeklere göre daha sık izlendiği MAHA ile trombositopeninin tanıda olmazsa olmaz bulgular olduğu; tanı için verilerin kapsamlı analizinin gerektiği saptandı. Plazma ADisintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS-13) aktivite testi tanının doğrulanmasında yardımcı olup TTP tedavisinin köşe taşını PD oluşturmaktadır.

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Renal Involvement in Thrombotic Thrombocytopenic Purpura: Is It Time to Challenge the Old Paradigm?

Issa,

Sandakly,

El Koubayati

et al. 2024

Cureus

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Outcome of Thrombotic Thrombocytopenic Purpura Patients: Single Center Experience

Cited by 3 publications

References 1 publication

Thrombotic Thrombocytopenic Purpura: Revisiting a Miss and an Inevitable Consequence

Thrombotic Thrombocytopenic Purpura: Revisiting a Miss and an Inevitable Consequence

Evaluation Of Clinical, Laboratory and Treatment Results Of Our Patients With Thrombotic Thrombocytopenic Purpura.

Renal Involvement in Thrombotic Thrombocytopenic Purpura: Is It Time to Challenge the Old Paradigm?

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