Korean J Blood Transfus.  2012 Apr;23(1):78-83.

A Case of Immune Hemolytic Anemia due to Autoantibodies Against C and e Antigens in a Patient with Paroxysmal Nocturnal Hemoglobinuria and Myelodysplastic Syndrome

• Park MJ
• Seo YH
• Park PW
• Kim KH
• Park SH
• Jeong JH
• Lee JH
• Hong JS
• Ahn JY

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.

Keyword

Paroxysmal nocturnal hemoglobinuria; Myelodysplastic syndrome; Immune hemolytic anemia

MeSH Terms

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
Autoantibodies
Hepatitis B e Antigens