A rare case of immune hydrops in an “Elevated D” pregnancy

Abstract

The D-/-phenotype is a rare variant of the Rh system, in which red blood cells express only D antigen. Sensitized D-/-women carry a risk of hemolytic disease of fetus and newborn (HDFN) and pose a challenge in management due to nonavailability of antigen negative blood for intra-uterine/exchange-transfusion. A 23y old G2P1L1A0, blood group “A” Rh D+C-c-E-e- presented at 20 weeks' period of gestation (POG) with a positive indirect antiglobulin test. Cordocentesis at 23 weeks' POG revealed fetal's blood group as “B” Rh D+, Direct Coombs Test 4+, Hemoglobin 2.8 g/dL. USG with MCA-PSV indicated rapid development of fetal anemia and hydrops. Patient's sibling had D-/-phenotype, but was ABO-incompatible with the fetus. Rapidity of progression of case resulted in intrauterine fetal demise at 27 weeks' POG. HDFN, in rare blood groups, leads to problems in arranging compatible units in developing countries due to lack of centralized rare donor registries.

Keywords

• antibody screening
• elevated d
• hemolytic disease of fetus and newborn