Hemolytic disease of newborn (HDN) due to blood group incompatibility is the cause of hyperbilirubinemia in a large number of
neonates(1). With the decline in Rh(D) HDN due to anti-Q immunoprophylaxis, HDN due tc:> antibodies to other blood group antigens such as non (D) rhesus (C, c, E, e, C e(f),
Cx, EW, Rh 36, Rh 37), Kell (K, Jsa JSb), Duffy (Fyb) Kidd
Jka, Jkb, JK3), MNS (M, S, s, U), Diego (Dia, Dib) and P Blood group systems have become a relatively greater problem(2). Heddle et al.(3) found
that the most frequently produced red cell alloantibodies were to the antigens of Rhesus, Kell and Kidd system. We report a case of HDN
due to the presence of anti-Jkb antibodies in the mother.
A 28-year-old fourth-gravida mother delivered a full term appropriate-for-gestation female baby. The birth weight was 2.7 kg and the apgar score was 7, 8, 8 at 1, 5 and 15 minutes, respectively. The baby was noticed to be icteric at 30 hours of age. There was no history of neonatal hyper- bilirubinemia in any of the siblings. The mother had no history of antepartum
hemorrhage, abortion or intrauterine death in earlier pregnancies. She had a history of blood transfusion fifteen years back.
On
physical examination of the neonate, no significant finding was observed. The investigation revealed a total serum bilirubin of 14.4 mg/dl, with a predominance of unconjugated bilirubin (13. 8
mg/dl). The blood group was B Rh(D) positive and di- rect antiglobulin test was negative. Hemoglobin concentration was found to be 18.8 g/dl with a reticulocyte count of 6%. Peripheral blood smear showed polychro-matophilia with nucleated red cells (10/100 WBC). The blood glucose level was 90 gl
dl. G6PD screening done by methylene blue reduction test showed normal activity. Septic screen did not reveal any abnormality.
Blood group of the mother was 0 Rh (D) positive. A partial Witebsky test was done to look for evidence of ABO-HDN by performing IgG anti-A and IgG anti-B titres. IgG titres of more than 1:32 were considered significant. The titres of IgG anti-A and IgG anti-B were low (1:2 and 1:4, respectively). Maternal serum was , tested against screening red cells consisting of two OR1R1
and one
OR2R2
red cells carrying most of the clinically significant blood group antigens in the homozygous state. The techniques used for antibody screening were saline at 22.C (room temperature), saline at 37°C, albumin layering, enzyme (pa- pain-cystein) and indirect antiglobulin test (IA T). An antibody was detected with en- zyme
technique against one of the screening cells. The antibody Was
identified using a red cell panel of 10 'O' cells fully typed for
antigens of clinical importance. The antibody was identified as, anti-Jkb. The baby responded to phototherapy.
Antibodies in Kidd system are generally IgG with potential to cross placenta and these show dosage effect. There are three known antibodies to Kidd antigens, i.e., anti-Jka, anti-Jkb and .anti-Jk3 (anti-Jka-b-)(4). Anti-Jka is the most commonly encountered
of these. Both anti-Jka and anti-Jkb can cause HDN and severe hemolytic transfusion reaction. Anti Jk-3 has been known to cause mild HDN. These antibodies have a propensity to disappear rapidly from the patient's sera. In a study(S), 10 out of 17 anti-Jka and 4 out of 11 anti-Jkb which
were detected during initial testing disappeared on long term follow up. Anti-Jkb has been frequently detected in association with other antibodies such as anti-S, anti-Fya, anti-C, anti-M and other kidd antibodies(6). Forty one per cent of women with anti-Jkb had autoimmune disease in a series(6).
This case emphasises the importance of antibody screening in sera of mothers of hyperbilirubinemic neonates. Anti-Jkb is a clinically significant antibody which may be associated with significant risk of HDN and 1 or stillbirth in subsequent pregnancies and, therefore a close monitoring of future pregnancies by clinical, laboratory and radiologic investigation is required. As the baby has persistent maternal antibodies for ,a period of first four months, the red cell transfusions
1
exchange transfusion during this period should be Jkb negative. Ex-change transfusion using Jkb positive red cells will worsen the jaundice by causing increased hemolysis.
We would also like to highlight that blood transfusion department should
develop red cell panel for antiqody screening. The preparation of red cell panel is cost effective
as once prepared and properly frozen at -800 C, these can be
used for all future investigation for maternal antibody screening for a
period of more than 10 years. Maternal antibody screening of hyperbilirubinemic neonates may therefore not only help in diagnosis of the case but also assist in the management of the case and future pregnancies.
V. Tomar,
N.Dhingra,
N. Madan,
M.M.A. Faridi*,
Departments of Pathology and Pediatrics*,
University College of Medical Sciences
and G. T.B. Hospital,
Delhi 110 095,
India.
1.
Valaes T, Kolipoulos C, Koltsidopoulos A. The impact of phototherapy in
the management of neonatal hyperbilirubinaemia: Comparison of historical cohorts. Acta Paediatr 1996; 85: 273-276.
2.
Alverson DL, Izquierdo LA. Hemolytic disease of newborn: Treatment and Prevention. In: Principles of Transfusion Medicine, 2nd edn. Eds. Rossi EC, Simon TL, Moss GS, Gould SA. Williams and Wilkins, 1996; pp 127-140.
3.
Heddle NM, Soutar RL, 'O' Hoski PL, Singer J, McBride JA, Ali MAM, et al, A
prospective study to determine the frequency and clinical significance of
alloimmunization post-transfusion. Br
J
Haematol1995; 91: 1000-1005.
4.
Fratelli AL, Silberstein LE, Spitalnik SL. Human blood group antigens and anti- bodies. In: Principles of Transfusion Medicine, 2nd edn. Eds. Rossi EC, Simon TL, Moss GS, Gould SA. Williams and Wilkins, 1996; pp 67-68.
5.
Ramsey G, Smietana SJ. Long term follow up testing of red cell antibodies. Transfusion 1994; 34: 122-124.
6.
Ramsey G, Smietana SJ. Multiple or un-common red cell antibodies in women. Association with autoimmune disease. Transfusion 1995;
35: 582-586.
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