myelokathexis


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ka·thex·is

(kă-theks'is),
A rare disorder characterized by bone marrow retention of myeloid elements leading to severe peripheral neutropenia; neutrophils have a distinctly abnormal appearance; Gm-CSF levels are undetectable and administration of this substance is therapeutically effective.
Synonym(s): myelokathexis

ka·thex·is

(kath-eks'is)
A rare disorder characterized by bone marrow retention of myeloid elements leading to severe peripheral neutropenia; neutrophils have a distinctly abnormal appearance; granulocyte-macrophage colony-stimulating factor levels are undetectable and administration of this substance is therapeutically effective.
Synonym(s): myelokathexis.

myelokathexis

(mī″ĕ-lō-kă-thek′sĭs) [ myelo- + cathexis]
A congenital form of neutropenia in which mature granulocytic white blood cells are not released from the bone marrow to circulate in the bloodstream. It is often inherited as an autosomal dominant condition associated with warts, low levels of gamma globulins, and recurrent infections (WHIM syndrome).

myelokathexis

neutropenia resulting from bone marrow retention in the face of adequate reserves.
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References in periodicals archive ?
WHIM is an abbreviation for the characteristic symptoms of the syndrome: warts, hypogammaglobulinemia, infections, and myelokathexis.
Extensive warts, hypogammaglobulinemia, infections and myelokathexis are found in this syndrome (1, 12, 18).
An example is the spontaneous chromothriptic cure of an individual with a rare congenital immunodeficiency disorder known as warts, hypogammaglobulinemia, infections, and myelokathexis syndrome (WHIM), which is caused by mutations in the chemokine receptor CXCR4 gene.
US-based biotechnology company X4 Pharmaceuticals has initiated a Phase 2/3 study with the first patient dosed with X4P-001-LD for the treatment of WHIM syndrome, a sub-type of a primary immunodeficiency disease characterised by warts, hypogammaglobulinemia, infections, and myelokathexis or "WHIM," the company said.