Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 2000 Aug;83(2):165–169. doi: 10.1136/adc.83.2.165

An unusual concurrence of graft versus host disease caused by engraftment of maternal lymphocytes with DiGeorge anomaly

J Ocejo-Vinyals 1, M Lozano 1, P Sanchez-Velasco 1, J E de Diego 1, J Paz-Miguel 1, F Leyva-Cobian 1
PMCID: PMC1718418  PMID: 10906029

Abstract

We describe a girl with DiGeorge anomaly and normal cytogenetic and molecular studies, whose clinical course was complicated by graft versus host disease caused by intrauterine materno-fetal transfusion, and several immunohaematological alterations including a monoclonal gammapathy of undetermined significance (first IgG, which subsequently changed to IgM). The main clinical features and pathological findings are discussed.



Full Text

The Full Text of this article is available as a PDF (148.5 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Appleton A. L., Curtis A., Wilkes J., Cant A. J. Differentiation of materno-fetal GVHD from Omenn's syndrome in pre-BMT patients with severe combined immunodeficiency. Bone Marrow Transplant. 1994 Jul;14(1):157–159. [PubMed] [Google Scholar]
  2. Archer E., Chuang T. Y., Hong R. Severe eczema in a patient with DiGeorge's syndrome. Cutis. 1990 Jun;45(6):455–459. [PubMed] [Google Scholar]
  3. Aubry M., Demczuk S., Desmaze C., Aikem M., Aurias A., Julien J. P., Rouleau G. A. Isolation of a zinc finger gene consistently deleted in DiGeorge syndrome. Hum Mol Genet. 1993 Oct;2(10):1583–1587. doi: 10.1093/hmg/2.10.1583. [DOI] [PubMed] [Google Scholar]
  4. Baybick J. H. Reticuloendotheliosis with eosinophilia (Omenn's syndrome). Findings in fine needle aspirate of a lymph node. Acta Cytol. 1987 Jan-Feb;31(1):20–24. [PubMed] [Google Scholar]
  5. Bonnet D., Cormier-Daire V., Kachaner J., Szezepanski I., Souillard P., Sidi D., Munnich A., Lyonnet S. Microsatellite DNA markers detects 95% of chromosome 22q11 deletions. Am J Med Genet. 1997 Jan 20;68(2):182–184. [PubMed] [Google Scholar]
  6. Brooks E. G., Filipovich A. H., Padgett J. W., Mamlock R., Goldblum R. M. T-cell receptor analysis in Omenn's syndrome: evidence for defects in gene rearrangement and assembly. Blood. 1999 Jan 1;93(1):242–250. [PubMed] [Google Scholar]
  7. Collard H. R., Boeck A., Mc Laughlin T. M., Watson T. J., Schiff S. E., Hale L. P., Markert M. L. Possible extrathymic development of nonfunctional T cells in a patient with complete DiGeorge syndrome. Clin Immunol. 1999 May;91(2):156–162. doi: 10.1006/clim.1999.4691. [DOI] [PubMed] [Google Scholar]
  8. Gerritsen E., Vossen J., van Tol M., Jol-van der Zijde C., Van der Weijden-Ragas R., Radl J. Monoclonal gammopathies in children. J Clin Immunol. 1989 Jul;9(4):296–305. doi: 10.1007/BF00918661. [DOI] [PubMed] [Google Scholar]
  9. Glover M. T., Atherton D. J., Levinsky R. J. Syndrome of erythroderma, failure to thrive, and diarrhea in infancy: a manifestation of immunodeficiency. Pediatrics. 1988 Jan;81(1):66–72. [PubMed] [Google Scholar]
  10. Hong R. The DiGeorge anomaly (CATCH 22, DiGeorge/velocardiofacial syndrome). Semin Hematol. 1998 Oct;35(4):282–290. [PubMed] [Google Scholar]
  11. Jouan H., Le Deist F., Nezelof C. Omenn's syndrome--pathologic arguments in favor of a graft versus host pathogenesis: a report of nine cases. Hum Pathol. 1987 Nov;18(11):1101–1108. doi: 10.1016/s0046-8177(87)80376-3. [DOI] [PubMed] [Google Scholar]
  12. Kyle R. A., Lust J. A. Monoclonal gammopathies of undetermined significance. Semin Hematol. 1989 Jul;26(3):176–200. [PubMed] [Google Scholar]
  13. Levy-Mozziconacci A., Wernert F., Scambler P., Rouault F., Metras D., Kreitman B., Depetris D., Mattei M. G., Philip N. Clinical and molecular study of DiGeorge sequence. Eur J Pediatr. 1994 Nov;153(11):813–820. doi: 10.1007/BF01972889. [DOI] [PubMed] [Google Scholar]
  14. Lozano M. D., Tierens A., Greiner T. C., Wickert R. S., Weisenburger D. D., Chan W. C. Clonality analysis of B-lymphoid proliferations using the polymerase chain reaction. Cancer. 1996 Apr 1;77(7):1349–1355. doi: 10.1002/(SICI)1097-0142(19960401)77:7<1349::AID-CNCR19>3.0.CO;2-1. [DOI] [PubMed] [Google Scholar]
  15. Notarangelo L. D., Villa A., Schwarz K. RAG and RAG defects. Curr Opin Immunol. 1999 Aug;11(4):435–442. doi: 10.1016/S0952-7915(99)80073-9. [DOI] [PubMed] [Google Scholar]
  16. Parkman R., Weinberg K. DiGeorge syndrome: still an enigma. J Pediatr. 1998 Jan;132(1):3–4. doi: 10.1016/s0022-3476(98)70475-5. [DOI] [PubMed] [Google Scholar]
  17. Pupo R. A., Tyring S. K., Raimer S. S., Wirt D. P., Brooks E. G., Goldblum R. M. Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive. J Am Acad Dermatol. 1991 Aug;25(2 Pt 2):442–446. doi: 10.1016/0190-9622(91)70225-q. [DOI] [PubMed] [Google Scholar]
  18. Ramos J. T., López-Laso E., Ruiz-Contreras J., Giancaspro E., Madero S. B cell non-Hodgkin's lymphoma in a girl with the DiGeorge anomaly. Arch Dis Child. 1999 Nov;81(5):444–445. doi: 10.1136/adc.81.5.444. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Tachinami T., Koizumi S., Yachie A., Yamagami M., Yokoi T., Ohno I., Taniguchi N., Takada I., Kawashima A., Okada Y. Immune status in two brothers with Omenn's syndrome: no discernible chimerism on FACS analysis using a monoclonal antibody specific for a maternally restricted HLA antigen. Am J Pediatr Hematol Oncol. 1990 Fall;12(3):343–350. [PubMed] [Google Scholar]
  20. Thompson L. F., O'Connor R. D., Bastian J. F. Phenotype and function of engrafted maternal T cells in patients with severe combined immunodeficiency. J Immunol. 1984 Nov;133(5):2513–2517. [PubMed] [Google Scholar]
  21. Van Esch H., Groenen P., Daw S., Poffyn A., Holvoet M., Scambler P., Fryns J. P., Van de Ven W., Devriendt K. Partial DiGeorge syndrome in two patients with a 10p rearrangement. Clin Genet. 1999 Apr;55(4):269–276. doi: 10.1034/j.1399-0004.1999.550410.x. [DOI] [PubMed] [Google Scholar]
  22. Van Esch H., Groenen P., Fryns J. P., Van de Ven W., Devriendt K. The phenotypic spectrum of the 10p deletion syndrome versus the classical DiGeorge syndrome. Genet Couns. 1999;10(1):59–65. [PubMed] [Google Scholar]
  23. Villa A., Santagata S., Bozzi F., Imberti L., Notarangelo L. D. Omenn syndrome: a disorder of Rag1 and Rag2 genes. J Clin Immunol. 1999 Mar;19(2):87–97. doi: 10.1023/a:1020550432126. [DOI] [PubMed] [Google Scholar]
  24. Wirt D. P., Brooks E. G., Vaidya S., Klimpel G. R., Waldmann T. A., Goldblum R. M. Novel T-lymphocyte population in combined immunodeficiency with features of graft-versus-host disease. N Engl J Med. 1989 Aug 10;321(6):370–374. doi: 10.1056/NEJM198908103210606. [DOI] [PubMed] [Google Scholar]
  25. de Saint-Basile G., Le Deist F., de Villartay J. P., Cerf-Bensussan N., Journet O., Brousse N., Griscelli C., Fischer A. Restricted heterogeneity of T lymphocytes in combined immunodeficiency with hypereosinophilia (Omenn's syndrome). J Clin Invest. 1991 Apr;87(4):1352–1359. doi: 10.1172/JCI115139. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES