Haematologica. 1990 Nov-Dec;75(6):546-50.

Successful lectin-separated bone marrow transplantation in adenosine deaminase deficiency-related severe immunodeficiency.

Nespoli L, Porta F, Locatelli F, Aversa F, Carotti A, Lanfranchi A, Gibardi A, Marchesi ME, Abate L, Martelli MF, et al.

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Clinica Pediatrica, Università, IRCCS Policlinico San Matteo, Pavia, Italy.

Abstract

Severe combined immunodeficiency disease (SCID) with adenosine deaminase (ADA) deficiency is a genetic autosomic recessive disorder with profound impairment of T-cell function, invariably complicated by fatal infections. The absence of ADA-enzyme and the accumulation of deoxy-ATP, with toxic effects on the T-lymphocytes is the common feature of this disease. As a consequence, lymphoid precursors failure to develop into mature T-cells, resulting in absolute lymphopenia and atrophy of the thymus. Bone marrow transplantation from an HLA-identical donor is considered the treatment of choice for this disease. We describe the case of a 1-month-old child with ADA deficiency SCID who underwent bone marrow transplantation (BMT) using paternal haploidentical, lectin-separated marrow, as a source of hemopoietic stem cells.

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Reconstitution of T- and B-cell function after T-lymphocyte-depleted haploidentical bone marrow transplantation in severe combined immunodeficiency due to adenosine deaminase deficiency. [Clin Immunol Immunopathol. 1987]
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Review Adenosine deaminase deficiency.
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Review The biology of bone marrow transplantation for severe combined immune deficiency. [Adv Immunol. 1991]
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