Nov 1, 1975

Diagnosis and therapy of primary defects of immunity (author's transl)

Klinische Pädiatrie
R J HaasE Kleihauer

Abstract

Immunological responses are divided into those mediated by humoral antibody (B-Cells) and those by cells (T-Cells). Both depend upon the activity of small lymphocytes which become thymus or bursa analogue dependent during embryonic development. Primary immundeficiencies are therefor defects of lymphocytes attributed in most cases to gene defects. Early diagnosis is the necessary prerequisit for possible treatment. Tests for assessing the cellular immune status are: skin testes and in vitro tests. Clinical syndroms are: lymphocytopenia, hypoplasia, deficiency of lymphocytes in lymphatic tissues normal cortical germinal centres. Recurrent infections with viruses, candida or pneumocystis carinii are common. Tests for assesing the humoral immune status are: measurement of immunglobulin levels in serum, isohaemagglutinins, tests for antibody formation following active immunization, in vitro tests. Clinical syndroms are: no germinal centres in lymphnodes, no plasma cells. Recurrent bacterial infections. Treatment of B-cell deficiencies: immunglobulin replacement therapy. Treatment of T-cell deficiencies by grafting of thymus transplants. Combined immune deficiencies are treated with bone marrow transplants from matched donors. For bo...Continue Reading

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Mentioned in this Paper

T-Lymphocyte
Immunologic Deficiency Syndromes
Gnotobiotics
Immune System Diseases
Lymphocytes as Percentage of Blood Leukocytes (Lab Test)
Entire Bursa
Lymphopenia
Neoplasm of Uncertain or Unknown Behavior of Thymus
Hypoplasia
Bone Marrow

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