Lithium therapy of children with chronic neutropenia.
We treated five children with chronic neutropenia using lithium carbonate and studied the effect in vivo on granulopoiesis. Granulocyte precursors (CFU-C) from blood and marrow, and colony-stimulating activity (CSA) from peripheral blood leukocytes, were assayed in a methylcellulose tissue culture system. Three patterns of response to lithium were seen. In patients with aplastic anemia (one acquired and two Fanconi's aplastic anemia) despite increased colony-stimulating activity, CFU-C numbers remained very low and the neutropenia persisted. In a patient with Kostmann neutropenia colony-stimulating activity, and blood and marrow CFU-C numbers increased, but the agranulocytosis was unchanged. An impressive therapeutic effect was seen in one patient with idiopathic neutropenia with low colony-stimulating activity who responded to lithium with an increase in colony-stimulating activity and CFU-C resulting in persisting normal neutrophil counts. Lithium appears useful in treating a select group of neutropenic patients in whom colony-stimulating activity production is responsive to lithium, and the granulocytic progenitor compartment is capable of producing mature neutrophils.
D000293:Adolescent; D000380:Agranulocytosis / Q000188:drug therapy*; D002648:Child; D002908:Chronic Disease; D003114:Colony-Forming Units Assay; D005260:Female; D006098:Granulocytes / Q000502:physiology; D006410:Hematopoiesis / Q000187:drug effects; D006801:Humans; D007223:Infant; D008094:Lithium / Q000627:therapeutic use*; D009503:Neutropenia / Q000188:drug therapy*
D008094:Lithium