Myelokathexis
Myelokathexis | |
---|---|
Classification and external resources | |
ICD-9-CM | 288.0[1] |
DiseasesDB | 32164 |
Myelokathexis is a congenital disorder of the white blood cells that causes severe, chronic leukopenia (a reduction of circulating white blood cells) and neutropenia (a reduction of neutrophil granulocytes).[2] The disorder is believed to be inherited in an autosomal dominant manner.[2][3] Myelokathexis refers to retention (kathexis) of neutrophils in the bone marrow (myelo).
Treatment
Myelokathexis is amongst the diseases treated with bone marrow transplantation and cord blood stem cells.
References
- ↑ "ICD-9-CM Disease Index: M". Retrieved 2008-02-17.
- 1 2 Aprikyan AA, Liles WC, Park JR, Jonas M, Chi EY, Dale DC (Jan 2000). "Myelokathexis, a congenital disorder of severe neutropenia characterized by accelerated apoptosis and defective expression of bcl-x in neutrophil precursors" (Free full text). Blood 95 (1): 320–327. PMID 10607719.
- ↑ Hord, JD; Whitlock, JA; Gay, JC; Lukens, JN (Sep–Oct 1997). "Clinical features of myelokathexis and treatment with hematopoietic cytokines: a case report of two patients and review of the literature". Journal of Pediatric Hematology/Oncology : official journal of the American Society of Pediatric Hematology/Oncology 19 (5): 443–448. doi:10.1097/00043426-199709000-00007. PMID 9329467.
See also
- WHIM syndrome Warts, hypogammaglobulinemia, infections, and myelokathexis OMIM: 193670
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