Myelokathexis — A New Form of Chronic Granulocytopenia

Leukocyte migration from a fish eye's view

“…Absent or reduced neutrophil function in response to infection is associated with primary immunodeficiency (Zuelzer et al, 1964), whereas overactivation of the neutrophil response underlies the pathogenesis of various diseases, including ischemia, reperfusion-induced tissue damage and autoinflammatory diseases, amongst others (Summers et al, 2010). This Commentary discusses recent progress in our understanding of leukocyte forward and reverse chemotaxis in vivo in zebrafish.…”

Genetic Basis of Inherited Bone Marrow Failure Syndromes

“…Myelokathexis is a rare autosomal dominant disorder with recurrent bacterial infections caused by reduced number and function of neutrophils. (Zuelzer 1964) Neutropenia is typically moderate to severe. Degenerative changes in the granulocytes are characteristic and include pyknotic nuclear lobes, fine chromatin filaments and hypersegmentation.…”

“…Degenerative changes in the granulocytes are characteristic and include pyknotic nuclear lobes, fine chromatin filaments and hypersegmentation. (Zuelzer 1964) Bone marrow specimens are usually hypercellular with granulocytic hyperplasia. The pathophysiology of myelokathexis has been attributed to a defective release of marrow cells into the peripheral blood.…”

“…The pathophysiology of myelokathexis has been attributed to a defective release of marrow cells into the peripheral blood. (Zuelzer 1964) Neutrophil precursors are characterized by depressed expression of BCL-X and accelerated apoptosis. (Aprikyan, Liles et al 2000) G-CSF ameliorate the neutropenia and lead to clinical improvement during episodes of bacterial infection.…”

“…(Aprikyan, Liles et al 2000) G-CSF ameliorate the neutropenia and lead to clinical improvement during episodes of bacterial infection. (Zuelzer 1964;Wetzler, Talpaz et al 1992) WHIM syndrome refers to an association of myelokatheksis with other features (warts, hypogammaglobulinemia, infections and myelokalthexis). Most cases studied are caused by mutations in the chemokine receptor gene CXCR4.…”