Fisher-Evans syndrome. Casereport in physician’s practice
https://doi.org/10.20514/2226-6704-2020-10-1-74-80
Abstract
The article presents the clinical features and treatment options for autoimmune thrombocytopenic purpura associated with autoimmune hemolytic anemia in the context of Fisher-Evans syndrome. Patient P. was admitted to the emergency department by an ambulance team with a direct diagnosis of acute pancreatitis. Leading clinical syndromes were hemorrhagic and anemic syndromes. A physical examination also revealed a strip-formed hemorrhagic rash in the area of inguinal folds, the anterior surface of the thighs and lower legs. During the differential diagnostic search, the diagnosis of Fisher-Evans syndrome was established. Initial oral and pulse therapy with prednisone was not effective. The patient received platelet transfusions regularly. When eltrombopag was included in therapy, there was an improvement in the patient’s condition, as well as a tendency to increase the level of platelets. On the 35th day, the patient was discharged from the hospital.
We examined various clinical variants of thrombocytopenia, I meet in real clinical practice.
About the Authors
A. A. YakushevRussian Federation
Andrey А. Yakushev
Moscow
I. G. Fedorov
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
L. Yu. Ilchenko
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
S. S. Shmycova
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
N. V. Ilin
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
G. G. Totolyan
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
I. O. Sirenova
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
I. G. Nikitin
Russian Federation
2nd Department of hospital therapy, N.I. Pirogov Russian national research medical university
Moscow
References
1. Maschan A.A., Rumyantsev A.G., Kovaleva L.G. et al. Recommendations of the Russian council of experts in diagnostics and treatment of patients with primary immune throbocytopenia. Oncogematologiya. 2010; 3: 36-45 [in Russian].
2. Evans R.S., Takahashi K. & Duane R.T. Primary thrombocytopenic purpura and acquired hemolytic anemia. Archives of Internal Medicine. 1951; 87: 48-65.
3. Hansen D.L, Möller S., Andersen K. et al. Evans syndrome in adults incidence, prevalence, and survival in a nationwide cohort. Am. J. Hematol. 2019; 94: 1081-1090.
4. Vaughn J.E., Anwer F., Deeg H.J., Treatment of refractory ITP and Evans syndrome by haematopoietic cell transplantation: is it indicated, and for whom? Vox. Sang. 2016; 110(1): 5-11.
5. Gómez-Almaguer D., Colunga-Pedraza P.R., Gómez-de León A. et al. Eltrombopag, low-dose rituximab, and dexamethasone combination as frontline treatment of newly diagnosed immune thrombocytopaenia. Br.J. Haematol. 2019; 184(2): 288-290.
Review
For citations:
Yakushev A.A., Fedorov I.G., Ilchenko L.Yu., Shmycova S.S., Ilin N.V., Totolyan G.G., Sirenova I.O., Nikitin I.G. Fisher-Evans syndrome. Casereport in physician’s practice. The Russian Archives of Internal Medicine. 2020;10(1):74-80. https://doi.org/10.20514/2226-6704-2020-10-1-74-80