Successful nonsibling bone marrow transplantation in severe combined immunodeficiency

K Ramsøe; P Skinhøj; V Andersen; P Ernst; V Faber; P Platz; M Thomsen; A Svejgaard; K R Eriksen; T Plesner; N Morling; J Philip; S A Killmann; C Koch; N Muller-Bérat; K Henningsen; H Axelsen

Successful nonsibling bone marrow transplantation in severe combined immunodeficiency

K Ramsøe, P Skinhøj, V Andersen, P Ernst, V Faber, P Platz, M Thomsen, A Svejgaard, K R Eriksen, T Plesner, N Morling, J Philip, S A Killmann, C Koch, N Muller-Bérat, K Henningsen, H Axelsen

5 Citations (Scopus)

Abstract

Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.

Original language	English
Journal	Transplantation
Volume	26
Issue number	6
Pages (from-to)	369-72
Number of pages	3
ISSN	0041-1337
Publication status	Published - 1978

Cite this

@article{6c05bd90328c11df8ed1000ea68e967b,

title = "Successful nonsibling bone marrow transplantation in severe combined immunodeficiency",

abstract = "Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.",

author = "K Rams{\o}e and P Skinh{\o}j and V Andersen and P Ernst and V Faber and P Platz and M Thomsen and A Svejgaard and Eriksen, {K R} and T Plesner and N Morling and J Philip and Killmann, {S A} and C Koch and N Muller-B{\'e}rat and K Henningsen and H Axelsen",

note = "Keywords: Bone Marrow Transplantation; Female; HLA Antigens; Humans; Immunologic Deficiency Syndromes; Infant; Male; Pedigree; Transplantation, Homologous",

year = "1978",

language = "English",

volume = "26",

pages = "369--72",

journal = "Transplantation",

issn = "0041-1337",

publisher = "Lippincott Williams & Wilkins",

number = "6",

}

TY - JOUR

T1 - Successful nonsibling bone marrow transplantation in severe combined immunodeficiency

AU - Ramsøe, K

AU - Skinhøj, P

AU - Andersen, V

AU - Ernst, P

AU - Faber, V

AU - Platz, P

AU - Thomsen, M

AU - Svejgaard, A

AU - Eriksen, K R

AU - Plesner, T

AU - Morling, N

AU - Philip, J

AU - Killmann, S A

AU - Koch, C

AU - Muller-Bérat, N

AU - Henningsen, K

AU - Axelsen, H

N1 - Keywords: Bone Marrow Transplantation; Female; HLA Antigens; Humans; Immunologic Deficiency Syndromes; Infant; Male; Pedigree; Transplantation, Homologous

PY - 1978

Y1 - 1978

N2 - Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.

AB - Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.

M3 - Journal article

C2 - 366819

SN - 0041-1337

VL - 26

SP - 369

EP - 372

JO - Transplantation

JF - Transplantation

IS - 6

ER -

Successful nonsibling bone marrow transplantation in severe combined immunodeficiency

Abstract

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