The Kidd Minus-Minus Phenotype in a Caucasian

L. N. SUSSMAN, R. SOLOMON, AND S. GROCAN

From the Beth Israel Medical Center, New York. New York

This is the first reported example in a Caucasian with the phenotype Jk(a-b-). A strongly reacting antibody was present which was of a single specificity and which could be completely absorbed by either Jk(a+b-) or Jk(a -b+) cells. Since no compatible donors could be found, autotransfusion was done. Family study failed to reveal any other members with the Jk(a-b-) phenotype. The question of a third allele at the Kidd locus is considered.

THE KIDD BLOOD GROUP System Was first reported by Allen, Diamond, and Niedziela.' They identified a new antigen called Jka which was found to be present in 77 per cent of the white population tested from Boston. Within two years, the anticipated anti-Jkb was described by Plant, Ikin, Mourant, Sanger, and Race.* A . minus-minus phenotype, Jk(a-b-) was demonstrated in a Filipino of Oriental ancestry.' The patient's serum contained a crossreacting antibody against Jk(a+b-) and Jk(a-b+) cells and was called anti-Jk" Jkb. Additional examples of the phenotype Jk(a-b-) were reported in Chinese,z Hawaiians,I2 and in South American Indians.lo No example of Jk(a-b-) in individuals of European or Negro ancestry has been reported. Race and Sangerg state that no homozygous J k J k Eu- ropean has yet been found, though heterozy- gotes certainly exist.

In routine preoperative studies, a strongly reacting antibody was found in the serum of a Group of A Rh (negative) female patient. All test cells in the screening panel were ag- glutinated by the patient's serum in a high protein medium and with ficin-treated cells at room temperature. The reactions in saline were negative, but became moderately

Received for publication July 8, 1974; accepted December 10, 1974.

positive when antiglobulin serum was added. The agglutination was strongest with enzyme treated test cells. The reactions of the patient's cells with several anti-Jka and anti-Jkb serums were negative. The patient's serum agglutinated all test cells known to be Jk(a+b+), Jk(a+b-), or Jk(a-b+).

The problem of blood for transfusion during surgery for a femoro-popliteal bypass was solved by collecting a unit of blood from the patient two weeks prior to surgery. The patient was then transfused with this unit without incident during the surgical procedure.

The propositus, a Jewish female, had a history of transfusion without reaction about 30 years ago following the birth of a full-term, normal child of her second preg- nancy. The child died at age 20 of a reported aplastic anemia. Her first and third pregnancies terminated spontaneously at 1 1 and at 16 weeks of gestation. The propositus was Group A, rh, MSs, Fy(a+b+), kk, Kp(a-b+), Lu(a+) Diego(a-), Le(a+b-), Vel+, Jk(a- b -).

Her serum reacted against all cells except her own. The antibody in the serum was identified as anti-JkaJkb. This was confirmed by Mr. Marsh at the New York Blood Center. The antibody titer against Jk(a- b+) and Jk(a+ b-) cells is shown in Table 1.

Absorption of the serum with Jk(a+b-) or Jk(a-b+) red blood cells completely removed all anti-Kidd activity. The serum was then completely inactive against Jk(a+b-), Jk(a-b+), Jk(a+b+) cells. This crossreacting antibody, of a single specificity, was most active at room temperature in a high protein medium and

Transfusion July-Aug. 1975

356 Volume 15 Number4

Volume 15 Number4 KIDD M I N U S - M I N U S P H E N O T Y P E 357

Table 1. Antibody Titer Using Jk (a+b-) and Jk (a-b+1 Cells

Cells Test System 1 2 4 7 16 32 64 Titer

Jk (a+b-) Saline ++ 2 Albumin ++ 2 Albumin Coombs ++ ++ + + + k 32 Ficin ++++ +++ ++ + 16 Ficin Coombs +++ ++ ++ ++ + + 32

Jk (a-b+) Saline ++ 4 Albumin ++ 2 Albumin Coombs ++ ++ 4 Ficin ++++ ++++ ++ ++ + + 32 Ficin Coombs +++ +++ ++ ++ ++ + + 64

+ +

- - - - - - - - - -

- + - -

-

+ + +

- - - - - - - - - + - - - -

-

the reactions could be enhanced by anti- globulin serum and ficin (Table I ) . The anti- body, as originally named was called anti-Jk” Jkb, although Erskine4 suggests the name anti-Jk. Some confusion might arise since this suggestion would require the genotype of the propositus to be indicated a s j k jk. Until clarified further, perhaps it would be best at present, to retain the original designation of the “silent” allele as Jk and the antibody as anti-Jk” Jkb.

Of interest, is the suspected duration of the activity of the antibody in vivo, assuming

it was stimulated by the transfusion of 30 years earlier.

Inquiries among other blood bank colleagues revealed the existence of several other known Jk(a-b-) people in the United States, none of whom had been previously reported. In each case, they had been identified because of crossmatching difficulty caused by the antibody in their serum. One case was found in Bellefonte, Pennsylvania5; another in Minneapolis, Min- nesota6; one in Buffalo, New York6; and one in Greenfield, Massachusetts. In none of

ml N. Test

a= Jha

a= Jk’

0- JkYk

FIG. I . Family study of propositus.

358 SUSSMAN, ET AL. Transfusion July-Aug. 1975

these was extensive family studies recorded. Our current case was investigated and a family tree could be constructed indicating the relevant Kidd grouping (Fig. 1). It is of interest that no other Jk(a-b-) individual could be identified in this limited family study.

The possible origin of the minus-minus phenotype in the Kidd system has been ex- plained as either a silent recessive gene ( J k ) or an inhibitor gene responsible for the sup- pression of Jk" or Jkb.3 The fact that the family described at present contains mem- bers who are Jk(a+b-) or Jk(a-b+) but none who are Jk(a+b+) suggests that a third silent allele may be present, making their probable genotype Jk" J k or Jkb J k . Thus, the propositus could have inherited a J k gene from each of her parents. This would be an extremely rare occurrence because of the infrequency of J k genes in the popu- lation. Consanguinity, however, may still produce an occasional Jk(a-b-) member. Although both deceased parents did come from a small Russian town with a small Jewish community, consanguinity was vigorously denied.

A Caucasian family is presented in which one member is of the rare Jk(a-b-) phenotype. Previous report^^.^.'* have pro- vided no positive evidence for inhibitor genes in the Kidd system. The report of two members in a family with the rare Jk(a-b-) phenotype'2 and the absence of Jk(a+b+) siblings in the family reported suggest the existence of a silent allele at the Kidd locus. Thorough study of such families should be pursued to further clarify the Kidd system.

Acknowledgments

The authors are grateful to W. L. Marsh of the Greater New York Blood Program who confirmed the Kidd grouping of the propositus as well as the single specificity of the antibody. Dr. A. M. Schwartz, Chief of Vascular Surgery a t Beth Israel Medical Center,

graciously permitted access to his patient and her family for this study.

References

I . Allen, F. H., Jr., L. K. Diamond, and B. Niedziela: A new blood group antigen. Nature 167:482, 1951.

Day, D., H. A. Perkins, and B. Sams: The minus- minus phenotype in the Kidd system. Trans- fusion5:315, 1965.

3. Crawford, M. N., T. J. Greenwalt, T. Sasaki, P. Tippett, R. Sanger, and R. R. Race: The phenotype Lu(a-b-) together with unconven- tional Kidd groups in one family. Transfusion 1:228, 1961.

4. Erskine, A. E.: The Principles and Practice of Blood Grouping. St. Louis, C. V. Mosley, 1973, p. 136.

Magnani, T. J.: Personal communication, Centre Community Hospital, Bellefonte, Pennsylvania.

Moulds, J.: Personal communication, Minneapolis War Memorial Blood Bank, Minneapolis, Min- nesota.

Pinkerton, F. J., L. E. Mermod, B. A. Liles, J. A. Jack, Jr., and J. Noades: The phenotype Jk(a- b-) in the Kidd blood group system. Vox Sang. 4:155, 1959.

Plaut, G., E. W. Ikin, A. E. Mourant, R. Sanger, and R. R. Race: A new blood-group antibody, anti-Jkb. Nature 171:431, 1953.

Race, R. R., and R. Sanger: Blood Groups in Man, 5th ed., Philadelphia, F. A. Davis, 1968, pp. 349 and 353.

Silver, R. T., J. M. Haber, and A. Kellner: Evi- dence for a new allele in the Kidd blood group system in Indians of northern Mato Grosso, Brazil. Nature 186:481, 1960.

I I . Stroup, M.: Personal communication, Philip Levine Laboratory, Ortho Diagnostics, Raritan, New Jersey.

Yokoyama, M., L. E. Mermod. and A. Stegmaier: Further examples of Jk(a-b-) in blood in Hawaii. Vox Sang, 12:154, 1967.

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L. N. Sussman, Director of the Ralph Gore Blood Bank, Beth Israel Medical Center, Mount Sinai School of Medicine of City University of New York, New York, New York.

R. Solomon, Supervisor of the Ralph Gore Blood Bank, Beth lsrael Medical Center, New York, New York.

S. Grogan, Technologist of the Ralph Gore Blood Bank, Beth Israel Medical Center.