A Case of Immune Hemolytic Anemia due to Autoantibodies Against C and e Antigens in a Patient with Paroxysmal Nocturnal Hemoglobinuria and Myelodysplastic Syndrome.
- Author:
Mi Jung PARK
1
;
Yiel Hea SEO
;
Pil Whan PARK
;
Kyung Hee KIM
;
Soon Ho PARK
;
Ji Hun JEONG
;
Jae Hoon LEE
;
Jun Shik HONG
;
Jeong Yeal AHN
Author Information
1. Department of Laboratory Medicine, Gachon University Gil Hospital, Incheon, Korea. jyahn@gilhospital.com
- Publication Type:Case Report
- Keywords:
Paroxysmal nocturnal hemoglobinuria;
Myelodysplastic syndrome;
Immune hemolytic anemia
- MeSH:
Anemia, Aplastic;
Anemia, Hemolytic;
Anemia, Hemolytic, Autoimmune;
Anemia, Refractory;
Autoantibodies;
Bone Marrow;
Coombs Test;
Hemoglobinuria, Paroxysmal;
Hemolysis;
Hepatitis B e Antigens;
Humans;
Hydrogen-Ion Concentration;
Male;
Mass Screening;
Middle Aged;
Myelodysplastic Syndromes;
Osmotic Fragility;
Pulmonary Embolism;
Thrombophilia
- From:Korean Journal of Blood Transfusion
2012;23(1):78-83
- CountryRepublic of Korea
- Language:English
-
Abstract:
Antiglobulin test-negative hemolytic anemia, thrombophilia, and marrow failure, such as aplastic anemia and myelodysplastic syndrome - refractory anemia (MDS-RA), are the primary clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH). Here, we report on a case of a 56-year-old male patient diagnosed with PNH, MDS-RA, and immune hemolytic anemia (IHA). The patient was transferred to the hospital with an impression of hemolytic anemia and pulmonary embolism. Positive results were observed on direct and indirect antiglobulin tests, and alloantibody, anti-C and anti-e, autoantibodies were identified. In addition, C and e antigens were found in Rh subgrouping. Therefore, due to the presence of autoantibodies against C and e antigens, we assumed that the cause of IHA was autoimmune reaction. Spherocytosis, increased osmotic fragility test, and positivity on direct and indirect antiglobulin tests were not considered characteristics of PNH. Therefore, without the presence of pulmonary embolism and MDS-RA, it is possible that autoimmune hemolytic anemia was considered the only reason for the hemolytic anemia, and that PNH could be overlooked. In patients with PH, use of washed RBCs during transfusion is not necessary. PNH screening test is recommended for patients who have experienced a thromboembolic event and intravascular hemolysis or MDS-RA. In order to obtain accurate information regarding the percentage of GPI-AP-deficient RBCs, flow cytometric analysis should be performed prior to transfusion.
