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Aplastic Anemia in Neonatal Lupus Erythematosus

Aplastic Anemia in Neonatal Lupus Erythematosus Abstract • Objective. —To describe an infant with neonatal lupus erythematosus associated with aplastic anemia. Setting. —The pediatric department in a tertiary-care hospital. Interventions. —Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy. Measurements/Main Results. —The patient presented with severe anemia and a circumscribed, reticular, macular rash on the face and neck at 5 months of age. Skin lesion biopsy revealed epidermic hyperkeratosis, hydropic degeneration of the basal layer, and deposition of immunoglobulins and granular C1 q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythyroid progenitor burst-forming unit) colonies in bone marrow increased by about tenfold when suppressor CD8+ T lymphocytes were removed, indicating immune suppression of hematopoiesis. High-dose steroid therapy failed. The infant subsequently developed gram-negative sepsis, severe metabolic acidosis, and consumptive coagulopathy and died. Conclusions. —Neonatal lupus erythematosus may present as part of a spectrum. The disease may range from mild and transient to a severe, life-threatening condition requiring immediate intervention, as in the case reported here. This is the first report of neonatal lupus associated with aplastic anemia due to immune-mediated suppression of hematopoiesis.(AJDC. 1993;147:941-944) References 1. Watson RM, Lane AT, Barnett NK, Bias WB, Arnett FC, Provost TT. Neonatal lupus erythematosus: a clinical, serological and immunogenetic study with review of the literature . Medicine . 1984;63:362-378.Crossref 2. Lee LA, Weston W. Neonatal lupus erythematosus . Semin Dermatol . 1988;7:66-72. 3. Buyon JP, Ben-Chetrit E, Karp S, et al. Acquired congenital heart block: pattern of maternal antibody response to biochemically defined antigens of the SSA/Ro-SSB/La system in neonatal lupus . J Clin Invest . 1989;84:627-634.Crossref 4. Provost TT, Watson R, Gaither KK, Harley JB. The neonatal lupus erythematosus syndrome . J Rheumatol . 1987;14( (suppl 13) ):199-205. 5. Esterly NB. Neonatal lupus erythematosus: special symposium . Pediatr Dermatol . 1986;3:417-424.Crossref 6. Lee LA, Weston WL. New findings in neonatal lupus syndrome . AJDC . 1984;138:233-236. 7. Kaneko F, Tanji O, Hasegawa T, Ohto H, Yamazaki K. Neonatal lupus erythematosus in Japan . J Am Acad Dermatol . 1992;26:397-403.Crossref 8. Laxer RM, Roberts EA, Gross KR, et al. Liver disease in neonatal lupus erythematosus . J Pediatr . 1990;116:238-242.Crossref 9. Petri M, Watson R, Hochberg MC. Anti-Ro antibodies and neonatal lupus . Rheum Dis Clin North Am . 1980;15:335-360. 10. Reichlin M, Harley JB. Antibodies to Ro (SSA) and the heterogeneity of systemic lupus erythenlatosus . J Rheumatol . 1987;14( (suppl 13) ):112-117. 11. Harley JB, Kaine JL, Fox OF, Reichlin M, Gruber B. Ro (SS-A) antibody and antigen in a patient with congenital complete heart block . Arthritis Rheum . 1985;28:1321-1325.Crossref 12. Lucky AW, Prendiville JS. Thrombocytopenia and hepatosplenomegaly in a newborn . Pediatr Dermatol . 1989;6:346-348.Crossref 13. Watson R, Kang JE, May M, Hudak M, Kickler T, Provost TT. Thrombocytopenia in the neonatal lupus syndrome . Arch Dermatol . 1988;124:560-563.Crossref 14. Vonderheid EC, Koblenzer PJ, Ming PML, Burgoon CF Jr. Neonatal lupus erythematosus: report of four cases with review of the literature . Arch Dermatol . 1976;112:698-705.Crossref 15. Weston WL, Harmon C, Peebles C, et al. A serological marker for neonatal lupus erythematosus . Br J Dermatol . 1982;107:377-382.Crossref 16. Provost TT, Watson R, Gammon WR, Radowsky M, Harley JB, Reichlin M. Neonatal lupus syndrome associated with U1RNP (nRNP) antibodies . N Engl J Med . 1987;316:1135-1138.Crossref 17. Reichlin M. Significance of the Ro antigen system . J Clin Immunol . 1986;6:339-348.Crossref 18. Lee LA, Harmon CE, Huff JC, Norris DA, Weston WL. The demonstration of SS-A/Ro antigen in human fetal tissues and in neonatal and adult skin . J Invest Dermatol . 1985;85:143-146.Crossref 19. Alexander E, Byon JP, Provost TT, Guarnieri T. Anti-Ro/SS-A antibodies in the pathophysiology of congenital heart block in neonatal lupus syndrome, an experimental model: in vitro electrophysiologic and immunocytochemical studies . Arthritis Rheum . 1992;35:176-189.Crossref 20. Lumpkin LR, Hall J, Hogan JD, Tucker SB, Jordon RE. Neonatal lupus erythematosus: a report of three cases associated with anti-Ro/SSA antibodies . Arch Dermatol . 1985;121:377-381.Crossref 21. Ramsey-Goldman R, Horn D, Deng JS, et al. Anti-SS-A antibodies and fetal outcome in maternal systemic lupus erythematosus . Arthritis Rheum . 1986;29:1269-1273.Crossref 22. Watson RM, Scheel JN, Petri M, Lee LA, Bias WB, McLean RH. Neonatal lupus erythematosus syndrome: analysis of C4 allotypes and C4 genes in 18 families . Medicine . 1992;71:84-95.Crossref 23. O'Neill PE, Grimwood RE. Neonatal lupus erythematosus syndrome . Arch Dermatol . 1988;124:1869-1870.Crossref 24. Nathan DJ, Snapper I. Simultaneous placental transfer of factors responsible for LE cell formation and thrombocytopenia . Am J Med . 1958;25:647-653.Crossref 25. Seip M. Systemic lupus erythematosus in pregnancy with haemolytic anaemia, leucopenia and thrombocytopenia in the mother and her newborn infant . Arch Dis Child . 1960;35:364-366.Crossref 26. Budman DR, Steinberg AD. Hematologic aspects of systemic lupus erythematosus: current concept . Ann Intern Med . 1977;86:220-229.Crossref 27. Michael SR, Vural IL, Bassen FA, Schaefer L. The hematologic aspects of disseminated (systemic) lupus erythematosus . Blood . 1951;6:1059-1072. 28. Cassileth PA, Myers AR. Erythroid aplasia in systemic lupus erythematosus . Am J Med . 1973;55:706-710.Crossref 29. Daughaday WH. Lupus erythematosus with severe anemia, selective erythroid hypoplasia and multiple red blood cell iso-antibodies . Am J Med . 1968;44:590-598.Crossref 30. Meyer RJ, Hoffman R, Zanjani ED. Autoimmune hemolytic anemia and periodic pure red cell aplasia in systemic lupus erythematosus . Am J Med . 1978;65:342-345.Crossref 31. Fitchen JJ, Cline MJ, Saxon A, Golde DW. Serum inhibitors of hematopoiesis in a patient with aplastic anemia and systemic lupus erythematosus: recovery after exchange plasmapheresis . Am J Med . 1979;66:537-542.Crossref 32. Dainiak N, Hardin J, Floyd V, Callahan M, Hoffman R. Humoral suppression of erythropoiesis in systemic lupus erythematosus (SLE) and rheumatoid arthritis . Am J Med . 1980;69:537-544.Crossref 33. Kagan WA, Ascensao JA, Pahwa RN, et al. Aplastic anemia: presence in human bone marrow of cells that suppress myelopoiesis . Proc Natl Acad Sci USA . 1976;73:2890-2894.Crossref 34. Champlin R, Ho W, Gale RP. Antithymocyte globulin treatment in patients with aplastic anemia: a prospective randomized trial . N Engl J Med . 1983;308:113-118.Crossref 35. Mangan KF, Chikkappa G, Bieler LZ, Scharfman WB, Parkinson DR. Regulation of human blood erythroid burst-forming unit (BFU-E) proliferation by T-lymphocyte subpopulations defined by Fc receptors and monoclonal antibodies . Blood . 1982;59:990-996. 36. Mangan KF, Hartnett ME, Matis SA, Winkelstein A, Abo T. Natural killer cells suppress human erythroid stem cell proliferation in vitro . Blood . 1984;63:260-269. 37. Zoumbos NC, Gascon P, Djeu JY, Trost SR, Young NS. Circulating activated suppressor T lymphocytes in aplastic anemia . N Engl J Med . 1985;312:257-265.Crossref 38. Wisniewski D, Strife A, Wachter M, Clarkson B. Regulation of human peripheral blood erythroid burst-forming unit growth by T lymphocytes and T lymphocyte sub-populations defined by OKT4 and OKT8 monoclonal antibodies . Blood . 1985;65:456-463. 39. Nielsen B, Jessen P; Hokland P. Regulation of autologous T-lymphocyte clones on in vitro growth of BFU-e: enhancing effects of both CD4 and CD8—positive clones . Eur J Haematol . 1991;46:26-32.Crossref 40. Haq AU, Rinehart JJ, Balcerzak SP. T-cell subset modulation of blood erythroid burst-forming unit proliferation . J Lab Clin Med . 1983;101:53-57. 41. Smith MA, Knight SM, Maddison PJ, Smith JG. Anaemia of chronic disease in rheumatoid arthritis: effect of the blunted response to erythropoietin and of interleukin-1 production by marrow macrophages . Ann Rheum Dis . 1992;51:753-757.Crossref 42. Hoffman R, Lu L, Jansen J, et al. Influence of T lymphocytes on hematopoiesis in a patient with T cell hypoplasia . Am J Hematol . 1988;27:118-124.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1993 American Medical Association. All Rights Reserved.
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0002-922X
DOI
10.1001/archpedi.1993.02160330031012
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Abstract

Abstract • Objective. —To describe an infant with neonatal lupus erythematosus associated with aplastic anemia. Setting. —The pediatric department in a tertiary-care hospital. Interventions. —Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy. Measurements/Main Results. —The patient presented with severe anemia and a circumscribed, reticular, macular rash on the face and neck at 5 months of age. Skin lesion biopsy revealed epidermic hyperkeratosis, hydropic degeneration of the basal layer, and deposition of immunoglobulins and granular C1 q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythyroid progenitor burst-forming unit) colonies in bone marrow increased by about tenfold when suppressor CD8+ T lymphocytes were removed, indicating immune suppression of hematopoiesis. High-dose steroid therapy failed. The infant subsequently developed gram-negative sepsis, severe metabolic acidosis, and consumptive coagulopathy and died. Conclusions. —Neonatal lupus erythematosus may present as part of a spectrum. The disease may range from mild and transient to a severe, life-threatening condition requiring immediate intervention, as in the case reported here. This is the first report of neonatal lupus associated with aplastic anemia due to immune-mediated suppression of hematopoiesis.(AJDC. 1993;147:941-944) References 1. Watson RM, Lane AT, Barnett NK, Bias WB, Arnett FC, Provost TT. Neonatal lupus erythematosus: a clinical, serological and immunogenetic study with review of the literature . Medicine . 1984;63:362-378.Crossref 2. Lee LA, Weston W. Neonatal lupus erythematosus . Semin Dermatol . 1988;7:66-72. 3. Buyon JP, Ben-Chetrit E, Karp S, et al. Acquired congenital heart block: pattern of maternal antibody response to biochemically defined antigens of the SSA/Ro-SSB/La system in neonatal lupus . J Clin Invest . 1989;84:627-634.Crossref 4. Provost TT, Watson R, Gaither KK, Harley JB. The neonatal lupus erythematosus syndrome . J Rheumatol . 1987;14( (suppl 13) ):199-205. 5. Esterly NB. Neonatal lupus erythematosus: special symposium . Pediatr Dermatol . 1986;3:417-424.Crossref 6. Lee LA, Weston WL. New findings in neonatal lupus syndrome . AJDC . 1984;138:233-236. 7. Kaneko F, Tanji O, Hasegawa T, Ohto H, Yamazaki K. Neonatal lupus erythematosus in Japan . J Am Acad Dermatol . 1992;26:397-403.Crossref 8. Laxer RM, Roberts EA, Gross KR, et al. Liver disease in neonatal lupus erythematosus . J Pediatr . 1990;116:238-242.Crossref 9. Petri M, Watson R, Hochberg MC. Anti-Ro antibodies and neonatal lupus . Rheum Dis Clin North Am . 1980;15:335-360. 10. Reichlin M, Harley JB. Antibodies to Ro (SSA) and the heterogeneity of systemic lupus erythenlatosus . J Rheumatol . 1987;14( (suppl 13) ):112-117. 11. Harley JB, Kaine JL, Fox OF, Reichlin M, Gruber B. Ro (SS-A) antibody and antigen in a patient with congenital complete heart block . Arthritis Rheum . 1985;28:1321-1325.Crossref 12. Lucky AW, Prendiville JS. Thrombocytopenia and hepatosplenomegaly in a newborn . Pediatr Dermatol . 1989;6:346-348.Crossref 13. Watson R, Kang JE, May M, Hudak M, Kickler T, Provost TT. Thrombocytopenia in the neonatal lupus syndrome . Arch Dermatol . 1988;124:560-563.Crossref 14. Vonderheid EC, Koblenzer PJ, Ming PML, Burgoon CF Jr. Neonatal lupus erythematosus: report of four cases with review of the literature . Arch Dermatol . 1976;112:698-705.Crossref 15. Weston WL, Harmon C, Peebles C, et al. A serological marker for neonatal lupus erythematosus . Br J Dermatol . 1982;107:377-382.Crossref 16. Provost TT, Watson R, Gammon WR, Radowsky M, Harley JB, Reichlin M. Neonatal lupus syndrome associated with U1RNP (nRNP) antibodies . N Engl J Med . 1987;316:1135-1138.Crossref 17. Reichlin M. Significance of the Ro antigen system . J Clin Immunol . 1986;6:339-348.Crossref 18. Lee LA, Harmon CE, Huff JC, Norris DA, Weston WL. The demonstration of SS-A/Ro antigen in human fetal tissues and in neonatal and adult skin . J Invest Dermatol . 1985;85:143-146.Crossref 19. Alexander E, Byon JP, Provost TT, Guarnieri T. Anti-Ro/SS-A antibodies in the pathophysiology of congenital heart block in neonatal lupus syndrome, an experimental model: in vitro electrophysiologic and immunocytochemical studies . Arthritis Rheum . 1992;35:176-189.Crossref 20. Lumpkin LR, Hall J, Hogan JD, Tucker SB, Jordon RE. Neonatal lupus erythematosus: a report of three cases associated with anti-Ro/SSA antibodies . Arch Dermatol . 1985;121:377-381.Crossref 21. Ramsey-Goldman R, Horn D, Deng JS, et al. Anti-SS-A antibodies and fetal outcome in maternal systemic lupus erythematosus . Arthritis Rheum . 1986;29:1269-1273.Crossref 22. Watson RM, Scheel JN, Petri M, Lee LA, Bias WB, McLean RH. Neonatal lupus erythematosus syndrome: analysis of C4 allotypes and C4 genes in 18 families . Medicine . 1992;71:84-95.Crossref 23. O'Neill PE, Grimwood RE. Neonatal lupus erythematosus syndrome . Arch Dermatol . 1988;124:1869-1870.Crossref 24. Nathan DJ, Snapper I. Simultaneous placental transfer of factors responsible for LE cell formation and thrombocytopenia . Am J Med . 1958;25:647-653.Crossref 25. Seip M. Systemic lupus erythematosus in pregnancy with haemolytic anaemia, leucopenia and thrombocytopenia in the mother and her newborn infant . Arch Dis Child . 1960;35:364-366.Crossref 26. Budman DR, Steinberg AD. Hematologic aspects of systemic lupus erythematosus: current concept . Ann Intern Med . 1977;86:220-229.Crossref 27. Michael SR, Vural IL, Bassen FA, Schaefer L. The hematologic aspects of disseminated (systemic) lupus erythematosus . Blood . 1951;6:1059-1072. 28. Cassileth PA, Myers AR. Erythroid aplasia in systemic lupus erythematosus . Am J Med . 1973;55:706-710.Crossref 29. Daughaday WH. Lupus erythematosus with severe anemia, selective erythroid hypoplasia and multiple red blood cell iso-antibodies . Am J Med . 1968;44:590-598.Crossref 30. Meyer RJ, Hoffman R, Zanjani ED. Autoimmune hemolytic anemia and periodic pure red cell aplasia in systemic lupus erythematosus . Am J Med . 1978;65:342-345.Crossref 31. Fitchen JJ, Cline MJ, Saxon A, Golde DW. Serum inhibitors of hematopoiesis in a patient with aplastic anemia and systemic lupus erythematosus: recovery after exchange plasmapheresis . Am J Med . 1979;66:537-542.Crossref 32. Dainiak N, Hardin J, Floyd V, Callahan M, Hoffman R. Humoral suppression of erythropoiesis in systemic lupus erythematosus (SLE) and rheumatoid arthritis . Am J Med . 1980;69:537-544.Crossref 33. Kagan WA, Ascensao JA, Pahwa RN, et al. Aplastic anemia: presence in human bone marrow of cells that suppress myelopoiesis . Proc Natl Acad Sci USA . 1976;73:2890-2894.Crossref 34. Champlin R, Ho W, Gale RP. Antithymocyte globulin treatment in patients with aplastic anemia: a prospective randomized trial . N Engl J Med . 1983;308:113-118.Crossref 35. Mangan KF, Chikkappa G, Bieler LZ, Scharfman WB, Parkinson DR. Regulation of human blood erythroid burst-forming unit (BFU-E) proliferation by T-lymphocyte subpopulations defined by Fc receptors and monoclonal antibodies . Blood . 1982;59:990-996. 36. Mangan KF, Hartnett ME, Matis SA, Winkelstein A, Abo T. Natural killer cells suppress human erythroid stem cell proliferation in vitro . Blood . 1984;63:260-269. 37. Zoumbos NC, Gascon P, Djeu JY, Trost SR, Young NS. Circulating activated suppressor T lymphocytes in aplastic anemia . N Engl J Med . 1985;312:257-265.Crossref 38. Wisniewski D, Strife A, Wachter M, Clarkson B. Regulation of human peripheral blood erythroid burst-forming unit growth by T lymphocytes and T lymphocyte sub-populations defined by OKT4 and OKT8 monoclonal antibodies . Blood . 1985;65:456-463. 39. Nielsen B, Jessen P; Hokland P. Regulation of autologous T-lymphocyte clones on in vitro growth of BFU-e: enhancing effects of both CD4 and CD8—positive clones . Eur J Haematol . 1991;46:26-32.Crossref 40. Haq AU, Rinehart JJ, Balcerzak SP. T-cell subset modulation of blood erythroid burst-forming unit proliferation . J Lab Clin Med . 1983;101:53-57. 41. Smith MA, Knight SM, Maddison PJ, Smith JG. Anaemia of chronic disease in rheumatoid arthritis: effect of the blunted response to erythropoietin and of interleukin-1 production by marrow macrophages . Ann Rheum Dis . 1992;51:753-757.Crossref 42. Hoffman R, Lu L, Jansen J, et al. Influence of T lymphocytes on hematopoiesis in a patient with T cell hypoplasia . Am J Hematol . 1988;27:118-124.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Sep 1, 1993

References