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Hemolytic disease of the newborn (anti-Kell 1) ranges from mild disease to a very severe disease. It is the second most common cause of severe HDN after Rh disease. Anti-Kell 1 is becoming relatively more important as prevention of Rh disease is becoming more and more effective.
About 91% of the population are Kell 1 negative and about 9% are Kell 1 positive. A fraction of a percentage are homozygous for Kell 1.
Very severe disease can occur as early as 20 wks gestation. Hydrops fetalis can also occur early. The finding of anti-Kell antibodies in an antenatal screening blood test (indirect Coombs test) is an indication for early referral to a specialist antenatal service for assessment, management and treatment. The fathers Kell type is determined which is Kell 1 positive (almost all heterozygous) in 9% of cases. About 4.5% of babies of a Kell 1 negative mother are Kell 1 positive.
The anti-Kell 1 antibodies can cause severe anemia partly because they prevent early RBC's proliferating as well as causing alloimmune hemolysis.
Causes
Mothers who are negative for the blood group K 1 are sensitised to it by RBC's that are positive for the K 1 antigen. Over half of the cases are caused by multiple blood transfusions and the remainder by pregnancy with a Kell 1 positive baby.
Prevention
Suggestions have been made that women of child bearing age or young girls should not be given a transfusion with Kell 1 positive blood (or Rhc positive blood for similar reasons). This would require a lot of extra work in blood transfusion departments and it is considered not economical to do the blood group screening at the present time.
It is theoretically likely that IgG anti-Kell 1 antibody injections would prevent sensitization to RBC surface Kell 1 antigens in a similar way that IgG anti-D antibodies (Rho(D) Immune Globulin) are used to prevent Rh disease, but the methods for IgG anti-Kell 1 antbodies have not been developed at the present time.
anti-Kell 2, anti-Kell 3 and anti-Kell 4 antibodies
Hemolytic disease of the newborn can also be caused by anti-Kell 2, anti-Kell 3 and anti-Kell 4 IgG antibodies. These are rarer and generallly the disease is milder.
References
- Geifman-Holtzman O, Wojtowycz M, Kosmas E, and Artal R. Female allo-immunization with antibodies known to cause hemolytic disease. Obstetrics and Gynecology 1997 89, 272-275
- Wiener CP, and Widness JA. Decreased fetal erythropoiesis and hemolysis in Kell hemolytic anemia. American Journal of Obstetrics and Gynecology. 1996 174: 547-55
See also
Transfusion medicine | Obstetrics | Pediatrics | Blood disorders | hematology
"Hemolytic disease of the newborn (anti-Kell)"