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PubMed · 4181812

[Relationship between the decrease of immunoglobulins and the clinically manifest antibody-deficiency syndrome in paraproteinemia].

M Knedel·A Fateh-Moghadam·R Lamerz·U Mayan
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AgammaglobulinemiaBlood Protein DisordersHumansgamma-GlobulinsJournal Article

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M Knedel, A Fateh-Moghadam, R Lamerz, U Mayan. 1968. [Relationship between the decrease of immunoglobulins and the clinically manifest antibody-deficiency syndrome in paraproteinemia].. https://pubmed.ncbi.nlm.nih.gov/4181812/

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PubMed1998

Clinical usage of intravenous immunoglobulins in Auckland.

AIMS: To review the indications for prescription of intravenous immunoglobulin (IVIg), in Auckland and the associated adverse effects. METHODS: All patients who received IVIg infusions in four major hospitals in Auckland in 1996 were identified from blood bank records. Clinical details were recorded from case notes. RESULTS: One hundred and thirty-five cases were identified and 131 records were available for review. Hypogammaglobulinaemia (n = 38) and thrombocytopenia (n = 38) were the two most common indications for 44% of Primary hypogammaglobulinaemia accounted for IVIg. the IVIg prescribed. IVIg was only used in one patient with hypogammaglobulinaemia secondary to chronic lymphocytic leukaemia and in one case of graft-versus-host disease. Adverse reactions were noted in 13 cases (10%). Headache (n = 4) and fever (n = 4) were the most commonly recorded side-effects. One patient developed acute renal failure after IVIg infusion. Written consent was documented in 7 patients (5%). CONCLUSIONS: The indications for IVIg prescription were generally consistent with the recommendations of the Australasian Society of Blood Transfusion. Use of IVIg in haematological conditions other than immune thrombocytopaenic purpura was infrequent. Most of the IVIg infusions were administered uneventfully but some minor side effects were recorded and one significant clinical event may have been causally related to IVIg infusion.

Agammaglobulinemia↗
PubMed1998

Mutations in the human lambda5/14.1 gene result in B cell deficiency and agammaglobulinemia.

B cell precursors transiently express a pre-B cell receptor complex consisting of a rearranged mu heavy chain, a surrogate light chain composed of lambda5/14.1 and VpreB, and the immunoglobulin (Ig)-associated signal transducing chains, Igalpha and Igbeta. Mutations in the mu heavy chain are associated with a complete failure of B cell development in both humans and mice, whereas mutations in murine lambda5 result in a leaky phenotype with detectable humoral responses. In evaluating patients with agammaglobulinemia and markedly reduced numbers of B cells, we identified a boy with mutations on both alleles of the gene for lambda5/14.1. The maternal allele carried a premature stop codon in the first exon of lambda5/14.1 and the paternal allele demonstrated three basepair substitutions in a 33-basepair sequence in exon 3. The three substitutions correspond to the sequence in the lambda5/14. 1 pseudogene 16.1 and result in an amino acid substitution at an invariant proline. When expressed in COS cells, the allele carrying the pseudogene sequence resulted in defective folding and secretion of mutant lambda5/14.1. These findings indicate that expression of the functional lambda5/14.1 is critical for B cell development in the human.

Agammaglobulinemia↗