Experience of successful diagnosis and first-stage treatment of a patient with Moschcowitz syndrome
https://doi.org/10.34215/1609-1175-2024-2-87-91
Abstract
Thrombotic thrombocytopenic purpura (TTP), also known as Moschcowitz syndrome, is an orphan, life-threatening, acute-onset disease caused by a deficiency of the metalloprotease that cleaves extra large multimers of von Willebrand factor (ADAMTS13). TTP is a difficult pathology to diagnose due to the pronounced polymorphism of symptoms. In this article, a clinical case of successful diagnosis and first stage therapy of a patient with laboratory-confirmed TTP is presented. Afterwards, the patient was safely transported to the National Medical Research Center for Hematology (Moscow).
About the Authors
P. A. Knyazenko
Vladivostok Clinical Hospital No.1
Russian Federation
Russian Federation
Vladivostok
M. E. Svislotskaya
Vladivostok Clinical Hospital No.1
Russian Federation
Russian Federation
Vladivostok
N. S. Klochkov
Vladivostok Clinical Hospital No.1
Russian Federation
Russian Federation
Vladivostok
A. V. Chernyshenko
Vladivostok Clinical Hospital No.1
Russian Federation
Russian Federation
Vladivostok
A. V. Talko
Regional Clinical Hospital No. 2
Russian Federation
Russian Federation
Angelina V. Talko, hematologist
55 Russian str., Vladivostok, 690105
+7 (914) 707-13-03
V. A. Nevzorova
Pacific State Medical University
Russian Federation
Russian Federation
Vladivostok
E. Yu. Shestopalov
Ministry of Health of Primorsky Krai
Russian Federation
Russian Federation
Vladivostok
References
1. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654–66. doi: 10.1056/NEJMra1312353
2. Galstyan GM, Klebanova EE, Mamleeva SY, Fedorova S.Yu., Drokov M.Yu., Yatsyk G.A. Neurological disorders in patients with thrombotic thrombocytopenic purpura. Clinical Medicine (Russian Journal). 2023;101(1):41–9 (In Russ.)]. doi: 10.30629/0023-2149-2023101-1-41-49
3. Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. An undescribed disease. Am J Med.1952;13(5):567–9. doi: 10.1016/0002-9343(52)90022-3
4. Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD, Kremer Hovinga J, Lämmle B, Matsumoto M, Pavenski K, Sadler E, Sarode R, Wu H, International Working Group for Th rombotic Th rombocytopenic Purpura. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Th romb Haemost. 2017;15(2):312–22. doi: 10.1111/jth.13571
5. Galstyan GM, Klebanova EE, Mamlee va SYu, Grzhimolovsky AV, Kovrigina AM, Dvir nyk VN, Bessmertny DK, Fidarova ZT, Melikyan AL. Immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura – diffi culties and diagnostic errors. Russian Journal of Hematology and Transfusiology. 2023;68(3):317–34 (In Russ.)]. doi: 10.35754/0234-5730-2023-68-3-317-334
6. Mariotte E, Azoulay E, Galicier L, Rondeau E, Zouiti F, Boisseau P, Poullin P, de Maistre E, Provôt F, Delmas Y, Perez P, Benhamou Y, Stepanian A, Coppo P, Veyradier A., French Reference Center for Th rombotic Microangiopathies. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 defi ciency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol. 2016;3(5):e237–45. doi: 10.1016/S2352-3026(16)30018-7
7. George J.N. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood. 2000;96(4):1223–9.
8. Filatov LB. Th rombotic thrombocytopenic purpura (Moschcowitz syndrome). In: M.A. Volkova (Ed.). Rare hematological diseases and syndromes. Moscow: Prakticheskaya meditsina, 2011;32–51 (In Russ.)].
Review
For citations:
Knyazenko P.A., Svislotskaya M.E., Klochkov N.S., Chernyshenko A.V., Talko A.V., Nevzorova V.A., Shestopalov E.Yu. Experience of successful diagnosis and first-stage treatment of a patient with Moschcowitz syndrome. Pacific Medical Journal. 2024;(2):87-91. (In Russ.) https://doi.org/10.34215/1609-1175-2024-2-87-91
Views:
261
Email this article 