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Sickle Cell Syndromes. I. Hemoglobin SC-A-Thalassemia

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Sickle Cell Syndromes. I. Hemoglobin SC-A-Thalassemia HEMOGLOBIN SC-a-THALASSEMIA 613 3. Belle, A.. and Ktm. Y S: Rat \mall ~nte\llnallilucln Is~ilat~onand ch;trilcterl~.~- 5x5 (1967) tlon of ;I water-soluble mucln fract~on.Arch. Blochem. H~oph!s. 150- 079 14. Jakow\ka. S Cystic F~hrostaand Rclitted Human and Animal I)~\ea\c\, p I62 (1972). Gordon and Breach Scientific Publ~sh~ngCo.. New York) 4. Bettelheim. F. A,: [Light-sc:tttcr~ng studlea of hov~ne suhm;~rlllar! mucln. IS. Joe\. R. W.. and Carr. C W. The b~ndlngof ci~lctum in mlrture\ of Biochim. Biophys eta, 236- 702 (1971) pho\phol~pids.Proc. Soc 'xp Blol. Med.. 1.74 1268 ( 1967). 5. Boat.T. F., W~esrnan.U. N.. and Pilllaviclni. .I C.: I'urtf~cat~oni~nd propcrtiea of 16. Negua. V.. Mucus Proc KO!. Soc. Med.. 60. 75 (1967). the c:tlc~um-prec~p~tnbleprotcln In \uhmar~llar! \:t11\:1 of normal .tnd c!\t~c 17 P~gman. W.. and tlerp. ,A: Aspects blochlmlquea et ph!\~olog~qucs des fihros~ssubjects. I'cdiat. Re\. 8. 531 (1974). gl)coproteldc\ des ?ecretlon\ muqucuaea. !Ann. Anat. Pitthol . 17 227 (1972). 6 Buddccke. I:.: In: A. Ciottachalk: M~scellnneoo\gl)coprotetns In gl)coprote~ns. IX. Roherts. (i. P. Isolation and ch:~r;~cter~/ittton~~fglycopr~lle~ns from sputu~n. bur. thetr cornporit~on.structure and function. p 558 (l:lse\~c.r Puhl~sh~ngC'O., J. Blochem . 30 265 ( 1974). Amsterdam. 1966) 14 Kou\\el. P.. I amhl~n.(; , and Degand. P.: lleterogene~t) of the c;~rhoh)drntr 7. di Sant'Agnesc. P. A,. and 'T:il;~mo, K. C : P:~thogcne\~\and ph!stop;~tholog) of chain\ of sull';tted hronch~algl!coproteins ~solatedfrom ;I patlent \ul'fer~ng cyat~cfihrow of the p;tncreas h' I-ngl J. Med.. 277 12x7 ( 1967) from c!\tic fthroz~\..I. Biol Chcm.. 2.51) 21 I4 (1975). X Foratncr. I. 1,- .. and kor\tncr. (i. (i.: C'i~lciu~iihlnd~ng to ~nte\ttn;tl ohlet ccll 20. Ta!lor. hl. The ongin\ and function\ of naul mucu\ I.ar!ngorcopc. 34 612 mucln Rlochtm. Hloph!\. act:^. 386 2x3 (1975) (1974). 9. Fcir\tner. .I F . Jahhitl. I.. itnd t.or\lner. Ci. (i: <;ohlet ccll mucln of r,lt smitll 21. \h arner. R R.. and Coleman. J. K . tlectron prohe anal!\^\ olcalc~urntran\port Inte\tlnc. Chcm~c;~litnd ph!s~c;~lch:~r:tcterl/;~tlon Can J. H~,ichcm..51. 1154 by \mCill tntc\tlnc. J Cell Blol., 64 54 (1975). (1973). 22 W:~rton. K. L.. and Blo~nficld.J H!droxydp:ttttc In the p:ithogcnez~\ tllc!\tlc 10. For\tner. .I. k . and Milncr!. 1. 1. Ci~lc~ulnb~nd~ng h) human er!throc!tc fthro\~a.Br~t. Med. .I . J: 570 ( I97 l ) rncrnhranca. Hlochcm J.. 124 563 ( 1971). 23. The i~uthora;ire ~ndehtedto the Medlc;tl Rc\carch Counc~lof C.tnad.~ and the I I Forstncr. I. k.. Tn~chtnnn.N.. haln~n\,V.. and For\tncr. (i. (i. Inre\tln;tl gohlet C;ln:ldl;ln C)sr~ct~hroslh l.oundat~on f11r fin:tncl;~l aupport. to Dr Ditv~dKelh cell mucu\ l\olatl<~nand ~dcnt~flcntlonh) ~~nmun~~lluore~cenccof it gohlut ccll of the 0ep;lrtmcnt of B~ochcm~str!.Iln~vcr\~t! of Torontl~.I)r perfornm~ng gl)coprolc~n..I. Cell Scl . 12 585 (1973) anitlyt~cultr:~centr~lugat~on \rudlc\. and 10 Mr\. Cec~l~akcng lor technical I?. (iihson. L. t.. Mi~tthe~s.W. .I . Mlnlh;ln. I' T . and I'att~, J. A - Kclat~ng 3b\l\l:lIlCt. muc~us,cnlc~um and sue;lt In a ncu concept of c)\tlc f~hro\~\Pcdiatrlcs. 48 14 Kequc\t\ for reprint\ should he addre\\cd t<l- I. F. tor\tnur. M.I).. Research 695 ( 197 1 ). In\t~tutc.The Hosp~talfor Slch Ch~ldrcn.35 I;n~\eratt! .\be.. loronto. 13. (;uglcr, E C.. Pallav~cin~.J. C.. Swcrdlow, ti.. and dl Sitnt'Agnew, P.: I'he role Ontarlo M5(i IXX (C:~nadi~). of ci~lciu~nIn \uhrn;~\~llar! si~l~ba 111 p:ltlent\ u~thc)\t~c fihro\l\. J Pedlat.. 71 25 Accepted for puhl~c;~t~on1)ecenihcr IX. 1'175. ~ediat.Res. 10: 61 3 620 (1976) Erythrocytes sickle cell anemia hemoglobin a-thalassemia Sickle Cell Syndromes. I. Hemoglobin SC-a-Thalassemia (iEOK<iI: K. HONI(;. 5' UNSAI. GUNAY. K. <;EOKGL: MASON. LOYI)A N. VII>A. AN[> C'IIKISI'INI: I;I:KI..N( 111r\~c,r\rn.01 Illrr~or.\.Sr(,hIc, cc.11 (c31rrc~rcrrrcl L)c~prrrrr~rc~r~rof lJrdrrr/rrc,\. IIrc, Ahraha~~r1.irrc~olrr Sc,hool oJ Medrcitrr. L'rri\,er.rin. of 1lli1roi.r M~dicuICetrrer, C'hic,ago. Illirrois. L'S.4 Extract months typical findings of mild hemoglobin H disease appeared. with HbH making up 6.5% of the total hemoglobin. Hematologic and globin synthesis $tudie$ were performed in a hlack American family in which the genes for x-thalassemia and Speculation hemoglobins t Hb) S and C' were segregating. 'l'he following distribution of these abnormalities was found: father. sickle cell Ihe prewnce of tu-thalassemia in the proband of thi\ rtudy trait + n-thalassemia; mother, HbC trait + a-thalaswmia, proposi- appeared to modif, her HbSC direare ro as to reduce it$ clinical tus, HbSC + a-thalassemia; older sibling, n-thalasscmia trait; seterit!, ar nell ar its pathologic potential. The amelioratite effect of and younger sibling, hemoglobin H disease. tu-thalarremia would appear to he related to a reduction in the 'The child with HbSC'-e-thalasremia demonstrated more serere intracellular hemoglobin concentration in the patient'$ erythrocvter. anemia and a more hemolytic picture than is typical of HhS<' hut other factors may alro be responriblc for there change$. Further disease. Her erythrocytes exhibited decreased osmotic fragility in study of genetically modified sickle hemoglobinopathy ryndromer comparison with HbSC' er! throcytes, but had an indistinguishable may ultimatcl! aid in the detelopment of effectite mcanr for the oxygen equilibrium curre and 2.3-diphosphoglycerate (2,3-I)P(;) treatment of there di$orderr. letel. Erythrocyte sickling in the patient, honeter, was significantly reduced, with less than 35% sickle forms observed at nearly com- plete oxygen desaturation. The clinical expression of sickling disorders ma! var! widel?. The sibling with hemoglobin H disease exhibited 26'2 Bart's even aniong individuals having an apparently identical form of (y,) hemoglobin at birth, a level comparable with that seen in in- sickle cell disease. This variability has stimulated investigation of fants with HbH disease in Far Eastern populations. At age 5 the role of genetic as well as nongenetic factors in the hematologic 614 HONIG ETAL and clinical expression of the sickle hemoglobinopathies. In a samples also were drawn into syringes containing 5% formalin and number of atypical sickling disorders specific modifying factors 0.01 M Na,HPO, in isotonic saline. The formalin-fixed erythro- have been identified that seem to alter the ability of the er) throcyte cytes were examined in a hemocytometer counting chamber and to undergo sickle transformation. Other genetic traits appear to classified according to the criteria of Rampling and Sirs (31). For modify the clinical expression of sickling disorders by different. each sample. 1,000 cells were counted. but thus far unidentified mechanisms. All clinical studies were carried out with informed consent Abnormalities that have a genetic basis and are known to according to the provisions of the Declaration of Helsinki. modify sickle cell diseases inciude: associated hemoglobin (?-chain (19, 40) and P-chain (4. 23. 24) structural abnormalities, thalas- semias and thalassemia-like disorders (1, 28. 35. 46): variability in R ESU L.TS the production of fetal hemoglobin (2. 17): and the presence of other associated hematologic disorders (22, 38). CASE REPORTS In this report we describe the clinical and hematologic findings of a child having an apparently modified form of hemoglobin SC The patient, RC', a 7-year-old girl, was referred for hematologic disease related to the association of tr-thalassemia. evaluation in preparation for an adenoidectomy. She had had multiple episodes of otitis media. and had developed severe METHODS nasopharyngeal obstruction with persistant mouth-breathing. She was noted to he anemic. and was found from a hemoglobin HEMATOL.OGIC AND Ht.MOGLOBIN STUDlhS electrophoresis stud! to have hemoglohins S and C. Apart from Hematologic measurements were made with a Coulter model S the problems attributable to her tonsillar hypertrophy, the child electronic cell counter which was standardized daily using a had generallj, been health!.. She hird never been noted to have commercial standard. Other determinations were performed by jaundice, fatigue, pain crisis, or other clinical manifestation of standard methods (5). Osmotic fragility tests were carried out with sickle cell disease. She appeared somewhat pale and her spleen was fresh samples of defibrinated blood as described bk Dncie (8). enlarged extending 2 cm below the costal margin. Blood samples for hemoglobin analysis were collected in EDTA. After completion of the hematologic evaluation the child was The erythrocytes were isolatetl by centrifugation. washed three given a transfusion of packed red cells sufficient to increase her times in isotonic saline, and lysed with 2-3 volumes of water blood hemoglobin concentration to I I .O g/dl. An adenoidectomy without the addition of an organic solvent. Stroma-free lysates under general aneqthesia was performed without con~plication.and were prepared and subjected to electrophoresis on cellulose-acetate her recovery and subsequent course were uneventful.
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