Treatment of refractory aplastic anemia with plasmapheresis: report of a case in childhood with review of the literature.
Treatment of aplastic anemia may raise considerable problems in some patients. This report concerns a boy whose illness started at 11 years of age. At first admission laboratory data were: hemoglobin 7.5 g/l, and counts of leucocytes, neutrophils and platelets were 2.3, 0.6, and 8 x 10(9)/l, respectively. His bone marrow was hypoplastic with sparse erythropoiesis. The patient did not respond to traditional medical treatment. Serum contained a high concentration of erythropoietin but no antibodies against erythropoietin. The patient's serum did neither alone, nor supported with recombinant erythropoietin, stimulate erythropoiesis in a bioassay, suggesting that some factor(s) inhibiting erythropoietic activity was present. Based on this hypothesis, plasma exchange was performed. After 26 weeks of plasmapheresis the hematological parameters were normalized. We conclude that plasmapheresis might be an alternative in treatment of resistant aplastic anemia. Further diagnostic tools to identify patients who might benefit from such a treatment are required.
Department of Paediatrics, Regionsykehuset, Trondheim, Norway.
R
R
Austgulen
P J
PJ
Moe
S
S
Jørstad
T E
TE
Widerøe
D045930:Anabolic Agents / Q000627:therapeutic use; D000741:Anemia, Aplastic / Q000175:diagnosis / Q000628:therapy*; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D001772:Blood Cell Count; D002648:Child; D003524:Cyclosporins / Q000627:therapeutic use; D004921:Erythropoietin / Q000096:biosynthesis; D006454:Hemoglobins / Q000096:biosynthesis; D006801:Humans; D007167:Immunotherapy; D008297:Male; D010956:Plasmapheresis; D011241:Prednisone / Q000627:therapeutic use; D012075:Remission, Spontaneous
D045930:Anabolic Agents; D000961:Antilymphocyte Serum; D003524:Cyclosporins; D006454:Hemoglobins; D004921:Erythropoietin; D011241:Prednisone