Öz
Kaynakça
- 1. Peyvandi F, Mannucci, PM, Valsecchi, C, Pontiggia, S, Farina, Retzios AD. ADAMTS13 content in plasma‐derived factor VIII/von Willebrand factor concentrates. American Journal of Hematology. 2013;88(10):895–898.
- 2. Chen J, Jin J X, Xu XF, Zhang XX, Ye XN, Huang J. Successful treatment of plasma exchange-refractory thrombotic thrombocytopenic purpura with rituximab: A case report. World Journal of Clinical Cases. 2020;8(12):2617.
- 3. Stanley M, Killeen RB and Michalski JM. Thrombotic thrombocytopenic purpura. StatPearls [Internet]. StatPearls Publishing, 2022.
- 4. https://www.thd.org.tr/thdData/Books/130/bolum-itrombotik-trombositopenik-purpura-tani-ve-tedavi-kilavuzu. pdf
- 5. Piers B and Scully M. “Management of thrombotic thrombocytopenic purpura: current perspectives. ” Journal of blood medicine. 2014;15–23.
- 6. Reese JA, Muthurajah DS, Hovinga JAK, Vesely SK, Terrell DR, George JN, Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features. Pediatric Blood & Cancer. 2013;60(10):1676–1682.
- 7. Moatti-Cohen M, Garrec C, Wolf M, Boisseau P, Galicier L, Azoulay E, et al. French Reference Center for Thrombotic Microangiopathies. Unexpected frequency of UpshawSchulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura. Blood. 2012;119(24), 5888–5897.
- 8. Hassan A, Iqbal M, George JN. Additional autoimmune disorders in patients with acquired autoimmune thrombotic thrombocytopenic purpura. Am J Hematol. 2019;94:E172.
- 9. Perez Botero J, Reese JA, George JN, McIntosh JJ. Severe Thrombocytopenia and microangiopathic hemolytic anemia in pregnancy: A guide for the consulting hematologist. Am J Hematol. 2021;96:1655.
- 10. George JN. TTP. long-term outcomes following recovery. Hematology Am Soc Hematol Educ Program. 2018;2018:548.
- 11. George JN. The remarkable diversity of thrombotic thrombocytopenic purpura: a perspective. Blood Adv. 2018;2:1510.
- 12. Jamme M, Rondeau E. The PLASMIC score for thrombotic thrombocytopenic purpura. Lancet Haematol. 2017;4:e148.
- 13. Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020;60:2047.
- 14. George JN. Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: when, how, and why? Transfusion. 2015;55:11.
- 15. Rieger M, Mannucci PM, Hovinga JAK, Herzog A, Gerstenbauer G, Konetschny C, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood. 2005;106:1262.
- 16. Ayanambakkam A, Kremer Hovinga JA, Vesely SK, George JN. Diagnosis of thrombotic thrombocytopenic purpura among patients with ADAMTS13 Activity 10%-20. Am J Hematol. 2017;92:E644.
A Review on Differential Diagnosis and Diagnostic Criteria of Complement-Mediated Thrombotic Microangiopathy with a PLASMIC Score Below Six and Coexisting Hepatitis B Positivity in a Male Patient
Öz
Microangiopathic Hemolytic Anemia (MAHA); Congenital Thrombotic thrombocytopenic purpura (TTP), Acquired (Immune) TTP, Shiga toxin associated Endemic hemolytic uremic syndrome (HUS) and Complement-Mediated TMA (CM-TMA), which may present with different clinical findings, Thrombocytopenia is a severe condition that affects multiple organ systems with anemia. In the congenital form, ADAMTS13 (von Willebrand Factor-Cleaving Protease or a metalloprotease that belongs to the “α disintegrin and metalloprotease with a thrombospondin type I motif) is diagnosed by the deficiency of the enzyme and the absence of antibodies. While in autoimmune TTP, the enzyme deficiency is associated with antibodies, endemic HUS associated with Shiga toxin is characterized by decreased ADAMTS13 levels due to endothelial damage. CM-TMA is associated with complement factor H (CFH) inhibitory dysfunction and increased complement levels due to a genetic mutation. On March 27, 2023, the patient with complaints of shortness of breath, headache, dizziness, weakness, and numbness in the hands and arms was admitted to the internal medicine clinic. The patient, presenting with Thrombocytopenia, reduced haptoglobin levels, elevated reticulocyte count, increased LDH, indirect hyperbilirubinemia, and a PLASMIC score of 6 in peripheral blood smear, was hospitalized and treated with the prediagnosis of TTP. Later, ADAMTS13 level was found to be 73% (normal range: 40– 130), and the diagnosis of CM-TMA was considered. In addition, we discussed the clinical distinction and treatment of TTP by reviewing the literature.
Anahtar Kelimeler
microangiopathic hemolytic anemia thrombotic thrombocytopenic purpura complement-related thrombotic thrombocytopenic purpura ADAMTS13 protein schistocyte plasmic score
Kaynakça
- 1. Peyvandi F, Mannucci, PM, Valsecchi, C, Pontiggia, S, Farina, Retzios AD. ADAMTS13 content in plasma‐derived factor VIII/von Willebrand factor concentrates. American Journal of Hematology. 2013;88(10):895–898.
- 2. Chen J, Jin J X, Xu XF, Zhang XX, Ye XN, Huang J. Successful treatment of plasma exchange-refractory thrombotic thrombocytopenic purpura with rituximab: A case report. World Journal of Clinical Cases. 2020;8(12):2617.
- 3. Stanley M, Killeen RB and Michalski JM. Thrombotic thrombocytopenic purpura. StatPearls [Internet]. StatPearls Publishing, 2022.
- 4. https://www.thd.org.tr/thdData/Books/130/bolum-itrombotik-trombositopenik-purpura-tani-ve-tedavi-kilavuzu. pdf
- 5. Piers B and Scully M. “Management of thrombotic thrombocytopenic purpura: current perspectives. ” Journal of blood medicine. 2014;15–23.
- 6. Reese JA, Muthurajah DS, Hovinga JAK, Vesely SK, Terrell DR, George JN, Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features. Pediatric Blood & Cancer. 2013;60(10):1676–1682.
- 7. Moatti-Cohen M, Garrec C, Wolf M, Boisseau P, Galicier L, Azoulay E, et al. French Reference Center for Thrombotic Microangiopathies. Unexpected frequency of UpshawSchulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura. Blood. 2012;119(24), 5888–5897.
- 8. Hassan A, Iqbal M, George JN. Additional autoimmune disorders in patients with acquired autoimmune thrombotic thrombocytopenic purpura. Am J Hematol. 2019;94:E172.
- 9. Perez Botero J, Reese JA, George JN, McIntosh JJ. Severe Thrombocytopenia and microangiopathic hemolytic anemia in pregnancy: A guide for the consulting hematologist. Am J Hematol. 2021;96:1655.
- 10. George JN. TTP. long-term outcomes following recovery. Hematology Am Soc Hematol Educ Program. 2018;2018:548.
- 11. George JN. The remarkable diversity of thrombotic thrombocytopenic purpura: a perspective. Blood Adv. 2018;2:1510.
- 12. Jamme M, Rondeau E. The PLASMIC score for thrombotic thrombocytopenic purpura. Lancet Haematol. 2017;4:e148.
- 13. Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020;60:2047.
- 14. George JN. Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: when, how, and why? Transfusion. 2015;55:11.
- 15. Rieger M, Mannucci PM, Hovinga JAK, Herzog A, Gerstenbauer G, Konetschny C, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood. 2005;106:1262.
- 16. Ayanambakkam A, Kremer Hovinga JA, Vesely SK, George JN. Diagnosis of thrombotic thrombocytopenic purpura among patients with ADAMTS13 Activity 10%-20. Am J Hematol. 2017;92:E644.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Klinik Tıp Bilimleri (Diğer) |
| Bölüm | Olgu Sunumu |
| Yazarlar | |
| Yayımlanma Tarihi | 30 Aralık 2023 |
| Yayımlandığı Sayı | Yıl 2023 Cilt: 13 Sayı: 3 |
