elliptocytosis in blood donors

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TRANSFUSION 19x7 VoI 27. No I CORRESPONDENCE 113 Table 1. Procedural variables' in use of Model CS 3000 Blood Product processed Procedure net MFG Flow rate Authors (mu time (mn) weight (9) used (mi) rPm (mi/mn) Ratio Huestis et at. 9730 170 209 737 1000 60 1:13 LefranCois 8575 180 252 780 750 50 1:11 Figures in the columns are arithmetic means. CS 3000, Fenwal Laboratories, Deerfield, IL). In compari- son, they did not find any difference with the use of a Model 30 (Haemonetics Corp., Braintree, MA) and a slightly lower, but not significant, granulocyte yield with a Model 2997 (IBM Biomedical Systems, Princeton, NJ). We report our experience with 235 leukaphereses using the CS 3000 with the same MFG. For all donors we used one of two steroid regimens: Two oral doses of dexameth- asone, 6 mg 12 to 18 hours and 2 mg 2 to 6 hours before the donation; or, three intravenous injections of hydrocor- tisone, 200 mg 2 hours before the procedure, 50 mg at the beginning of the procedure, and 50 mg I hour after the beginning of the procedure. Compared with the manufacturer's guidelines we de- creased the speed of centrifugation (750 rpm versus 1000) and increased the interface (300 versus 150). Table 1 shows the procedural variables we used during these leukaphereses compared with those Huestis et al. reported. As shown in Table 2, our results with the MFG are sim- ilar to those obtained by Huestis et al. with HES. The dif- ference observed between our results with MFG and those of Huestis et al. with HES for the WBC and granulocyte yields can be explained by the difference in milliliters of blood processed (8575 versus 9730). The comparison of granulocytes removed per liter of blood processed was identical with a similar 95 percent confidence range. All our results were obtained from the final product after removal of red cells by sedimentation at 37°C. We believe that the difference observed by Huestis et al. when they used the MFG as the macromolecular agent instead of HES compared with our results with the MFG may be explained by a difference in procedural variables. These data indicate that after some modification of the manufacturer's procedures for leukaphereses on model CS 3000, it is possible to obtain the same results with MFG as with HES. G. LEFRANCOIS, MD Unite cytaphkrese C. N. T. S. Centre de prelevement Saint-Antoine 53 Boulevard Diderot 75012 Paris France Reference I. Huestis DW, Loftus TJ, Gilcher R, et al. Modified fluid gelatin: an alternative macromolecular agent for centrifugal leukapheresis. Transfusion 1985;25:343-8. Eliiptocytosis in blood donors To the Editor: Hereditary elliptocytosis is an autosomal dominant condition in which an abnormality of the red cell cytoskel- eton leads to an oval appearance of at least 25 percent, and often greater than 75 percent, of the person's red cells. The incidence of this red cell membrane defect is estimated at between 2.5 and 5 persons per 10,000 in the United States.' Many people with this condition have associated mild hemolytic anemias that are usually well-compensated. Borderline or mildly abnormal red cell survival studies are frequent; severely decreased red cell survival is less com- mon. Because the condition is usually benign, many healthy people are unaware of the abnormality of their red cells. We recently found elliptocytosis in two unsuspecting donors when we read their indirect antiglobulin compati- bility tests (IAT) microscopically. The first donor was a 25-year-old white woman who had no significant medical history. She had donated blood at the regional blood center on three previous occasions. Her fourth donation was drawn with the AS-I (Adsol) preser- vation system, a segment from the packed red cells was Table 2. Cell yield in leukaphereses using Model CS 3000' Macromolecular Number of Steroids Authors agent WBCt X PNN$ X PNN per LBPS X lop9 procedures given (3.42-3.89) (2.88-3.32) (3.03-3.49) 122 Yes (1.54-2.48) (1.oo-2.02) (1.03-2.04) 14 Yes (2.07-3.93) (1.88-3.58) (3.03-3.90) 253 Yes Huestis et al. H ES 3.65 3.10 3.26 Huestis et al. M FG 2.01 1.51 1.54 Lef ranGois MFG 3 2.73 3.18 Figures in parentheses are 95 percent confidence ranges. t WBC-White blood cells. $ PNN-Polymorphonuclear leukocytes (granulocytes). 5 LBP-Liters of blood processed.

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Page 1: Elliptocytosis in blood donors

TRANSFUSION 19x7 VoI 27. No I

CORRESPONDENCE 113

Table 1. Procedural variables' in use of Model CS 3000

Blood Product processed Procedure net MFG Flow rate

Authors (mu time (mn) weight (9) used (mi) rPm (mi/mn) Ratio

Huestis et at. 9730 170 209 737 1000 60 1:13 LefranCois 8575 180 252 780 750 50 1:11

Figures in the columns are arithmetic means.

CS 3000, Fenwal Laboratories, Deerfield, IL). In compari- son, they did not find any difference with the use of a Model 30 (Haemonetics Corp., Braintree, MA) and a slightly lower, but not significant, granulocyte yield with a Model 2997 (IBM Biomedical Systems, Princeton, NJ).

We report our experience with 235 leukaphereses using the CS 3000 with the same MFG. For all donors we used one of two steroid regimens: Two oral doses of dexameth- asone, 6 mg 12 to 18 hours and 2 mg 2 to 6 hours before the donation; or, three intravenous injections of hydrocor- tisone, 200 mg 2 hours before the procedure, 50 mg at the beginning of the procedure, and 50 mg I hour after the beginning of the procedure.

Compared with the manufacturer's guidelines we de- creased the speed of centrifugation (750 rpm versus 1000) and increased the interface (300 versus 150).

Table 1 shows the procedural variables we used during these leukaphereses compared with those Huestis et al. reported.

As shown in Table 2, our results with the MFG are sim- ilar to those obtained by Huestis et al. with HES. The dif- ference observed between our results with MFG and those of Huestis et al. with HES for the WBC and granulocyte yields can be explained by the difference in milliliters of blood processed (8575 versus 9730). The comparison of granulocytes removed per liter of blood processed was identical with a similar 95 percent confidence range. All our results were obtained from the final product after removal of red cells by sedimentation at 37°C.

We believe that the difference observed by Huestis et al. when they used the MFG as the macromolecular agent instead of HES compared with our results with the MFG may be explained by a difference in procedural variables.

These data indicate that after some modification of the manufacturer's procedures for leukaphereses on model CS 3000, it is possible to obtain the same results with MFG as with HES.

G. LEFRANCOIS, M D Unite cytaphkrese

C. N. T. S. Centre de prelevement Saint- Antoine

53 Boulevard Diderot 75012 Paris

France

Reference

I. Huestis DW, Loftus TJ, Gilcher R, et al. Modified fluid gelatin: an alternative macromolecular agent for centrifugal leukapheresis. Transfusion 1985;25:343-8.

Eliiptocytosis in blood donors

To the Editor: Hereditary elliptocytosis is an autosomal dominant

condition in which an abnormality of the red cell cytoskel- eton leads to an oval appearance of at least 25 percent, and often greater than 75 percent, of the person's red cells. The incidence of this red cell membrane defect is estimated at between 2.5 and 5 persons per 10,000 in the United States.' Many people with this condition have associated mild hemolytic anemias that are usually well-compensated. Borderline or mildly abnormal red cell survival studies are frequent; severely decreased red cell survival is less com- mon. Because the condition is usually benign, many healthy people are unaware of the abnormality of their red cells. We recently found elliptocytosis in two unsuspecting donors when we read their indirect antiglobulin compati- bility tests (IAT) microscopically.

The first donor was a 25-year-old white woman who had no significant medical history. She had donated blood at the regional blood center on three previous occasions. Her fourth donation was drawn with the AS-I (Adsol) preser- vation system, a segment from the packed red cells was

Table 2. Cell yield in leukaphereses using Model CS 3000'

Macromolecular Number of Steroids Authors agent WBCt X PNN$ X PNN per LBPS X lop9 procedures given

(3.42-3.89) (2.88-3.32) (3.03-3.49) 122 Yes

(1.54-2.48) (1 .oo-2.02) (1.03-2.04) 14 Yes

(2.07-3.93) (1.88-3.58) (3.03-3.90) 253 Yes

Huestis et al. H ES 3.65 3.10 3.26

Huestis et al. M FG 2.01 1.51 1.54

Lef ranGois MFG 3 2.73 3.18

Figures in parentheses are 95 percent confidence ranges. t WBC-White blood cells. $ PNN-Polymorphonuclear leukocytes (granulocytes). 5 LBP-Liters of blood processed.

Page 2: Elliptocytosis in blood donors

114 CORRESPONDENCE TRANSFUSION Vol 27. No I 19117

FIG. 1. Microscopic examination of the antiglobulin phase of the crossmatch. Left: first donor’s elliptocytic cells; right: random donor’s cells. The elliptocytes are readily distinguished from nor- mal cells (magnification X400).

tested for compatibility when the blood was 30 days old. Room temperature, 37OC albumin, and macroscopic IAT portions of the compatibility test were negative. Micro- scopic examination in the antiglobulin test with a 40 X lens (400 X magnification) showed no agglutination; how- ever, it was noticed that most of the cells were cigar- shaped (Fig. I ) . On a subsequent Wright’s stained smear of blood from the segment, more than 90 percent of the red cells were elliptocytes. Occasional red cell fragments and spherocytes were also seen. Many of the cells had regular spicule-like projections consistent with echinocytes (Fig. 2). The unit of blood was not transfused. A fresh sample obtained from the donor confirmed the presence of elliptocytes.

The second donor was a 23-year-old white man who had no previous medical problems. He had donated blood at the regional blood center twice. The third donation was drawn with Adsol; the packed red cells were 31 days old when a segment was used for compatibility testing. All tests were negative, but elliptocytes were noted during microscopic examination of the IAT. When a Wright’s stained smear of the segment was examined, approxi- mately 75 percent of the cells were ovalocytes and ellipto- cytes; some of the cells were echinocytic. The unit was dis-

FIG. 2. Wright’s stained smears of donor cells. Left: first donor’s elliptocytic cells; right: random donor’s cells. Although echinocytosis has altered the appearance of all cells, the ellipto- cytes remain readily identifiable (magnification X IOOO).

carded; a fresh sample of this donor’s blood also confirmed the presence of elliptocytes.

Elliptocytes have a variety of membrane defects, includ- ing lack of blood group antigens such as Gerbich (the Leach phenotype); testing the cells from these two donors’ for Ge phenotype is pending.* In addition, elliptocytes have more rapid declines than normal red cells in levels of adenosine triphosphate (ATP) and 2,3-diphisphoglycerate when incubated in vitro.’ A defect in glycolytic metabolism that results in lower levels of ATP is postulated to target the cell for premature destruction in the spleen. This defect can be expected to occur during refrigerator storage in the blood bank, resulting in shortened posttransfusion survival of the cells. This has been demonstrated: in one study, posttransfusion survival of one-half of the red cells from two elliptocytic donors in normal recipients with intact spleens was abnormal (19 and 20 days, respectively; nor- mal, 27 days, ”Cr) despite the absence of clinical hemoly- sis in either donor.4 In another report, elliptocytes from each of two donors had reduced survival in normal people with spleens, but normal survival in asplenic people.’

Because of the low incidence of elliptocytosis, the likeli- hood of a multiply transfused patient receiving more than 1 unit containing elliptocytes is small, and the risk of requiring additional transfusions to compensate for the shortened survival of these cells is negligible. Thus, there appears to be no need to screen blood donors for ellipto- cytes. On the other hand, when the donor knows that he or she has this condition or when elliptocytes are found ser- endipitously, as in the cases reported here, it may be advis- able to avoid using the red cells for transfusion.

MARGOT S. KRUSKALL, M D DEAN S. MESSIER, MT(ASCP)

BARBARA V. DOHERTY, MT(ASCP) DONNA G. PACINI, MT(ASCP)SBB

Blood Bank Beth lsrael Hospital

Bosron, MA MARK A. POPOVSKY, M D

American Red Cross Northeast Region Blood Services Dedham, MA

References

I . Bannerman R M , Renwick JH. The hereditary elliptocytosis: clinical and linkage data. Ann Hum Genet 1962;26:23-38.

2. Anstee DJ, Parsons SF, Ridgwell K , et al. Two individuals with elliptocytic red cells apparently lack three minor erythro- cyte membrane sialoglycoproteins. Biochem J l984;2 I8:615-9.

3. Quaife MA, Gregorius C, Kelly R. Characteristics of red blood cell survival from subjects with hereditary elliptocytosis. Clin Nucl Med 1978;3:398-400.

4. DeGruchy GC, Loder PB, Hennessey IV. Haemolysis and gly- colytic metabolism in hereditary elliptocytosis. Br J Haematol 1962;8: 168-79.

Incidence of ABO subgroups in Chinese in Taiwan

To the Editor: For the last 2 years we have studied the incidence of

ABO subgroups in Chinese in Taiwan. The data show some significant differences between Chinese and whites.

Among approximately 400,000 donors tested during a 2-year period at Taipei Blood Donation Center, the follow- ing phenotypes were observed: 32 B,, six AlB3, five A,I,