Steven M. Holland, Author at Cancer Therapy Advisor

Steven M. Holland

All articles by Steven M. Holland

Hyper-IgE syndromes

Hyper-IgE syndromes What every physician needs to know: There are two major diseases included under the heading of hyper-IgE syndromes (HIES): STAT3 deficiency (autosomal dominant HIES or Job’s syndrome) and DOCK8 deficiency (autosomal recessive HIES). Job’s syndrome is due to heterozygous dominant-negative mutations in STAT3, a major transcription factor, that lead to impaired inflammation with…

Disorders of neutrophils: GATA2 Deficiency (MonoMAC2 DCML, Emberger, familial AML/MDS, classical NK cell deficiency)

Disorders of neutrophils: GATA2 Deficiency (also known as MonoMAC, DCML, Emberger, familial AML/MDS, classical NK cell deficiency) What every physician needs to know: GATA2 is a critical transcription factor in early hematopoietic ontogeny. Haploinsufficiency for GATA2 causes conditions ranging from deafness to lymphedema to acute myelogenous leukemia to disseminated mycobacterial infection, with ages of onset…

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