LETTER TO THE EDITOR

Antibody to a high-prevalence blood group antigen Ataassociated with Sjögren’s syndrome

Siddharth Mathur & Harwinder Sawhney &

Maud A. Bertoni & Renuka Gupta

Received: 15 July 2007 /Accepted: 20 November 2007 /Published online: 11 December 2007# Springer-Verlag 2007

Dear Editor,Anti-Augustine antibody (Ata) are a high-prevalence redblood cell (RBC) antigen and are not assigned to a bloodgroup system, and anti-Ata is an extremely rare RBCalloantibody. It was first discovered by Applewhaite et al.in 1967 [1] and has been described only in black personsmost of whom were Caribbean or Southern US ancestry.The anti-Ata is usually IgG and is reactive only by theantiglobulin test. It has been proposed as a phenotypeoriginating as a mutation in West Africa or the West Indies[2]. A recent donor search yielded only one Ata-subjectamong 8,552 black donors in Detroit [3]. In a case reportpublished by Ramsey et al., two patients with anti-Ataantibody were found to have a concurrent autoimmunedisease in the form of Systemic Lupus Erythematosus(SLE) and Type 1 Diabetes mellitus (DM) [4]. Our casereport documents the association of a different autoimmunedisease, Sjögren’s syndrome, with an anti-Ata antibody.

Our patient is a 49-year-old, black female who wasadmitted to the emergency room with complaint of severeshortness of breath of 1-day duration and found to haveleft-side Pneumococcal pneumonia, which responded tointravenous antibiotics. She gave previous history of twosimilar episodes, of which one required hospitalization. Shealso complained of dry eyes and mouth for the past fewyears and parotid region swelling on and off, for which shetook no specific treatment. She had six pregnancies—noneof which required any transfusion or had any neonataljaundice or anemia.

The patient also had low hemoglobin of 9.4 g/dl whichdropped to 6.5 on day 3 of admission. The patient’s bloodsample was sent for typing before transfusion and it camepositive for anti-Ata antibody. This test was performed bythe Immunohematology Laboratory of the New York BloodCenter. The phenotype of our patient is: D+ C− E+ c+ e+K− Fya− Fyb− Jka+ Jkb− S+ s+ M+ N+Coa+ Ata− Lan+.In light of the recurrent pneumonias and history suggestiveof Sjögren’s syndrome, patient was worked up for the same.Laboratory results showed a positive SS-A and SS-Bantibody, with a high titer positive anti-neutrophil antibody,positive Rheumatoid factor, and hypocomplementemia. Allother markers for autoimmune diseases were negativeincluding double-stranded DNA. Patient was diagnosed asa case of Sjögren’s syndrome with anti-Ata antibody andleft-lung Pneumococcal pneumonia. The patient’s hemo-globin improved with erythropoietin injections therapy, andshe did not receive any blood transfusions.

Applewhaite et al. first described anti-Ata in 1967 in ablack woman, Mrs. August, and since then ten otherexamples of anti-Ata have been described so far [2, 4].Although there was uncertain clinical significance previ-ously, Cash et al. reported the first case of a severe delayedhemolytic transfusion reaction secondary to anti-Ata

Ann Hematol (2008) 87:497–498DOI 10.1007/s00277-007-0419-y

S. Mathur (*)Internal Medicine, Wyckoff Heights Medical Center,Brooklyn, NY, USAe-mail: mathur.siddharth@gmail.com

H. SawhneyDepartment of Hematology–Oncology,Wyckoff Heights Medical Center,Brooklyn, USA

M. A. BertoniMedical Blood Bank, Hematology–Pathology,Wyckoff Heights Medical Center,Brooklyn, USA

R. GuptaPGY III, Department of Medicine,Wyckoff Heights Medical Center,Brooklyn, USA

antibody [5]. In addition, two chromium-labeled RBCstudies have demonstrated decreased RBC survival inpatients with anti-Ata suggesting that this antibody is ofsignificance [2, 4].

The occurrence of this rare antibody with autoimmunedisease documented earlier by Ramsey et al., and now inour patient, suggests that there may be a relationshipbetween the two. Of the two cases described by Ramseyet al., the first was a 26-year-old female from Guyana whowas diagnosed SLE since age 11. RBC survival study doneon this patient showed a remarkably shortened half-life.The other patient was a 36-year-old female from NewOrleans who was also a Jehovah’s Witness and was foundto have Type I DM. Patients with autoimmune diseasemight be at increased risk of RBC alloimmunizationbecause of underlying immunogenic propensity.

We report this patient with anti-Ata antibody and itspossible association with autoimmune illness. Family mem-bers may be typed and matched, and autologous transfusionmay be considered, as well as the use of hemoglobinsubstitutes and hematinics [6]. These patients should be

encouraged to register with the American Red Cross RareDonor Registry and be encouraged to donate for themselvesand others.

References

1. Applewhaite F, Ginsburg V, Cunningham CA, Gavin J (1967) Avery frequent red cell antigen Ata. Vox Sang 13:444–445

2. Sweeney JD, Holmes S, McCall L et al (1995) At(a-) phenotype:description of a family and reduced survival of At(a+) red cells inproposita with anti-Ata. Transfusion 35:63–67

3. Winkler MM, Hamilton JR (1990) Previously tested donorseliminated to determine rare phenotype frequencies, abstract. Jointcongress of International Society of Blood Transfusion andAmerican association of Blood Banks. AABB, Arlington, p p 158

4. Ramsey G, Sherman LA, Zimmer AM et al (1995) Clinicalsignificance of anti-Ata. Vox Sang 69:135–137

5. Cash KL, Brown T, Sausais L et al (1999) Severe delayedhemolytic transfusion reaction secondary to anti-Ata. Transfusion39:834–837

6. Woodfield G, Poole J, Nance ST, Daniels G (2004) A review of theISBT rare blood donor program. Immunohematology 20:244–248

498 Ann Hematol (2008) 87:497–498