Case Report: A Case of Primary Evans Syndrome Involving the Three Lines of Blood Cells

Layth Al-Karaja^1, , Lamees Khalil¹, Besan M. Alsayied¹, Shahd T. Natsheh², Malak S. Khalil¹, Wajd A.D.Natsheh² and Alaa Abdulwahab¹

¹Al-Quds University, Jerusalem, Palestine

²Palestinian Polytechnic University, Hebron, Palestine

Cite this paper:
Layth Al-Karaja, Lamees Khalil, Besan M. Alsayied, Shahd T. Natsheh, Malak S. Khalil, Wajd A.D.Natsheh and Alaa Abdulwahab. Case Report: A Case of Primary Evans Syndrome Involving the Three Lines of Blood Cells. American Journal of Medical Case Reports. 2023; 11(4):74-76. doi: 10.12691/ajmcr-11-4-3

Abstract

Evans Syndrome is a rare autoimmune disorder characterized by the sequential presence of autoimmune hemolytic anemia and thrombocytopenia. Knowing whether an ES is primary or secondary is crucial. The coexistence of ES with other conditions such as hematological malignancies, systemic lupus erythematosus, and infections can affect how it is managed or affect its prognosis. Here we report Evans Syndrome case in a 6 years old female patient who presented with cough, shortness of breath, tachypnea, fever, epistaxis, prolonged bleeding, ecchymosis, and petechia all over the lower jaundice, limbs and trunk, pancytopenia, autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP), reticulocytosis, and hemolysis of transfused blood, with elevated inflammatory markers. There was no family history of hemolytic diseases. The patient was treated with IV IG, steroids (prednisone) with slight progression for one year, then cyclosporine with better response, and finally mycophenolate mofetil.

Keywords:
Evans syndrome hemolytic anemia thrombocytopenia

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References:

[1]	McCarthy MD, Fareeth AGM. Evans syndrome in a young man with rare autoimmune associations and transplanted liver. BMJ Case Rep. 2022 Sep 28; 15(9): e251252.
[2]	Dhakal S, Neupane S, Mandal A, Parajuli SB, Sapkota S. Evans Syndrome: A Case Report. JNMA J Nepal Med Assoc. 2022 May 5; 60(249): 482-484.
[3]	Cortesi M, Soresina A, Dotta L, Gorio C, Cattalini M, Lougaris V, Porta F, Badolato R. Pathogenesis of Autoimmune Cytopenias in Inborn Errors of Immunity Revealing Novel Therapeutic Targets. Front Immunol. 2022 Apr 6; 13: 846660.
[4]	Dhakal S, Neupane S, Mandal A, Parajuli SB, Sapkota S. Evans Syndrome: A Case Report. JNMA J Nepal Med Assoc. 2022 May 5; 60(249): 482-484.
[5]	Evans RS, Takahashi K, Duane RT. Primary thrombocytopenic purpura and acquired hemolytic anemia. Archives of Internal Medicine 1951; 87:48-65.
[6]	Bashir BA, Othman SA, Malik AA. A rare haematological disorder in a Sudanese child: Evans syndrome, case report and literature review. Sudan J Paediatr. 2021; 21(1): 89-94.
[7]	Dhakal S, Neupane S, Mandal A, Parajuli SB, Sapkota S. Evans Syndrome: A Case Report. JNMA J Nepal Med Assoc. 2022 May 5; 60(249): 482-484.
[8]	Savasan S, Warrier I, Ravindronath Y. The spectrum of Evan's syndrome. Archives of Disease in Childhood 1997; 77(3): 245-248.
[9]	Bashir BA, Othman SA, Malik AA. A rare haematological disorder in a Sudanese child: Evans syndrome, case report and literature review. Sudan J Paediatr. 2021; 21(1): 89-94.
[10]	Allison AC. Mechanisms of action of mycophenolate mofetil. Lupus. 2005; 14 Suppl 1: S2-S8.
[11]	Dale DC, Crawford J, Klippel Z, Reiner M, Osslund T, Fan E, Morrow PK, Allcott K, Lyman GH. A systematic literature review of the efficacy, effectiveness, and safety of filgrastim. Support Care Cancer. 2018 Jan; 26(1): 7-20.