A case of hemoglobin SC disease with cold agglutinin‐induced hemolysis

Children with sickle cell disease commonly require red blood cell (RBC) transfusion. We report the first case of hemoglobin (Hb) SC disease with development of severe anemia induced by cold agglutinin hemolysis after Mycoplasma infection. Complete blood count (CBC) showed falsely decreased RBC count and hematocrit and falsely elevated MCV and MCHC. Peripheral blood smear showed RBC clumping at room temperature; this disappeared after warming to 37°C. Anti C3b‐C3d was present on red cells, and indirect antiglobulin test revealed a circulating cold agglutinin. Furthermore, anti‐Mycoplasma pneumoniae IgM antibody was detected in serum. Careful evaluation of CBCs and peripheral blood smears is required in cases of worsening anemia among sickle cell patients and consideration should be given to cold hemagglutinin disease as an etiology. Am. J. Hematol. 78:37–40, 2005. © 2004 Wiley‐Liss, Inc.

[1]  P. Swerdlow,et al.  Mycoplasma disease and acute chest syndrome in sickle cell disease. , 2003, Pediatrics.

[2]  P. Lane Sickle cell disease. , 1996, Pediatric clinics of North America.

[3]  Scott T. Miller,et al.  Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. , 1994, Archives of pediatrics & adolescent medicine.

[4]  C. Chapman,et al.  Autoanti-red cell antibodies synthesized by patients with infectious mononucleosis utilize the VH4-21 gene segment. , 1993, Journal of immunology.

[5]  G. Maude,et al.  Clinical presentation of sickle cell-hemoglobin C disease. , 1986, The Journal of pediatrics.

[6]  J. Paulson,et al.  Erythrocyte receptors for Mycoplasma pneumoniae are sialylated oligosaccharides of Ii antigen type , 1984, Nature.

[7]  A. Schechter,et al.  Molecular and cellular pathogenesis of hemoglobin SC disease. , 1982, Proceedings of the National Academy of Sciences of the United States of America.

[8]  David Allan,et al.  Release of spectrin-free spicules on reoxygenation of sickled erythrocytes , 1982, Nature.

[9]  S. Jones,et al.  OUTBREAK OF APLASTIC CRISES IN SICKLE CELL ANAEMIA ASSOCIATED WITH PARVOVIRUS-LIKE AGENT , 1981, The Lancet.

[10]  T. Papayannopoulou,et al.  Fetal characteristics of erythrocytes in sickle cell anemia: an immunofluorescence study of individual cells. , 1979, Blood.

[11]  S. Hakomori,et al.  Cell surface modification by endo-beta-galactosidase. Change of blood group activities and release of oligosaccharides from glycoproteins and glycosphingolipids of human erythrocytes. , 1979, The Journal of biological chemistry.

[12]  J. Bertles,et al.  COLD AGGLUTININS AND SICKLE-CELL DISEASE , 1977, The Lancet.

[13]  J. Atkinson,et al.  The role of complement in the clearance of cold agglutinin-sensitized erythrocytes in man. , 1976, The Journal of clinical investigation.

[14]  F. Rosner,et al.  Pancytopenia in a patient with sickle cell anemia. , 1975, American journal of diseases of children.

[15]  J. Stuart,et al.  Anaemic crisis in sickle cell disease. , 1975, Journal of clinical pathology.

[16]  Bender Ae Letter: Protein requirement. , 1974 .

[17]  P. Heller,et al.  The mechanism of indction of cold agglutinins by mycoplasma pneumoniae. , 1971, Journal of immunology.

[18]  S. Shohet,et al.  Excessive binding of natural anti-alpha-galactosyl immunoglobin G to sickle erythrocytes may contribute to extravascular cell destruction. , 1986, The Journal of clinical investigation.

[19]  H. Schubothe The cold hemagglutinin disease. , 1966, Seminars in hematology.