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GTR Home > Conditions/Phenotypes > Autoimmune lymphoproliferative syndrome type 4

Autoimmune lymphoproliferative syndrome type 4

Synonyms
AUTOIMMUNE LYMPHOPROLIFERATIVE SYNDROME, TYPE IV; RAS-associated autoimmune leukoproliferative disorder
Modes of inheritance
Unknown inheritance (Orphanet)

Summary

RAS-associated leukoproliferative disorder is characterized by lymphadenopathy, splenomegaly, and variable autoimmune phenomena, including autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, and neutropenia. Laboratory studies show an expansion of lymphocytes due to defective apoptosis, as well as significant autoantibodies. Some patients have recurrent infections, and there may be an increased risk of hematologic malignancy (summary by Oliveira, 2013 and Niemela et al., 2010). The disorder shows significant overlap with autoimmune lymphoproliferative syndrome (ALPS; 601859) and was originally designated ALPS IV. [from OMIM]

Available tests

75 tests are in the database for this condition.

Check Related conditions for additional relevant tests.

Clinical tests (75 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: 'C-K-RAS, C-K-RAS, CFC2, K-RAS2A, K-RAS2B, K-RAS4A, K-RAS4B, K-Ras, K-Ras 2, KI-RAS, KRAS1, KRAS2, NS, NS3, OES, RALD, RASK2, c-Ki-ras, c-Ki-ras2, KRAS
    Summary: KRAS proto-oncogene, GTPase

  • Also known as: ALPS4, CMNS, KRAS, N-ras, NCMS, NRAS1, NS6, NRAS
    Summary: NRAS proto-oncogene, GTPase

Clinical features

Help
Imported from Human Phenotype Ontology (HPO)

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