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| EVIDENCE FOR ENGRAFTMENT OF DONOR-TYPE MULTIPOTENT CD34(+) CELLS IN A PATIENT WITH SELECTIVE T-LYMPHOCYTE RECONSTITUTION AFTER BONE-MARROW TRANSPLANTATION FOR B-SCID |
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| Author(s): TJONNFJORD GE, STEEN R, VEIBY OP, FRIEDRICH W, EGELAND T |
| Source: BLOOD Volume: 84 Issue: 10 Pages: 3584-3589 Published: NOV 15 1994 |
| Times Cited: 24 References: 28 |
| Abstract: Severe combined immunodeficiencies (SCID), a heterogeneous group of disorders of infancy, are fatal without treatment directed at immunologic reconstitution. Allogeneic bone marrow transplantation (BMT), which is such a treatment presents some unique features in SCID, especially when T-lymphocyte-depleted HLA haploidentical allografts are used. Donor-type T lymphopoiesis, less often B lymphopoiesis, develops, whereas myelopoiesis remains the recipient-type. Little is known about the engrafting cells in this peculiar lymphohematopoietic chimerism and the pathophysiology of the frequent failure of B-lymphocyte reconstitution. To address these issues, we purified CD34(+) BM cells from a patient with selective T-lymphocyte reconstitution after HLA haploidentical BMT for B-SCID. Phenotypic analysis of CD34(+) cells was performed by flow cytometry, and functional studies of donor- and recipient-type CD34(+) cells were performed in vitro. Donor-type CD34(+) cells, constituting similar to 2% if the CD34(+) cells, were detected; both CD34(+)HLA-DR(-) cells and CD34(+) cells coexpressing B-(CD10 and CD19) and T-(CD2 and CD7) lymphocyte-associated cells surface molecules. Donor-type CD34(+) cells coexpressing myeloid-associated molecules (CD13, CD14, CD15, and CD33) were undetectable. However, donor-type CD34(+) myeloid progenitors could be shown in functional assays. Recipient-type CD34(+) cells coexpressing B- and T-lymphocyte- as well as myeloid-associated molecules were detected, but recipient-type CD34(+) cells could not be driven into T-lymphocyte differentiation in vitro. These findings provide evidence for engraftment of multipotent stem cells in our patient with B-SCID. Furthermore, the failure of B-lymphocyte reconstitution cannot be explained by lack of donor-type B-lymphocyte progenitors. Donor-type B lymphopoiesis and myelopoiesis are prevented by an unidentified mechanism. (C) 1994 by The American Society of Hematology. |
| Document Type: Article |
| Language: English |
| Reprint Address: TJONNFJORD, GE (reprint author), UNIV OSLO, NATL HOSP, INST TRANSPLANTAT IMMUNOL, N-0027 OSLO, NORWAY |
Addresses:
1. UNIV OSLO, NATL HOSP, DEPT MED A, OSLO, NORWAY
2. NYCOMED BIOREG AS, OSLO, NORWAY
3. UNIV ULM, DEPT PEDIAT 2, W-7900 ULM, GERMANY |
| Publisher: W B SAUNDERS CO, INDEPENDENCE SQUARE WEST CURTIS CENTER, STE 300, PHILADELPHIA, PA 19106-3399 |
| Subject Category: Hematology |
| IDS Number: PR221 |
| ISSN: 0006-4971 |
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