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Treatment Of Refractory Aplastic Anemia With Plasmapheresis: Report Of A Case In Childhood With Review Of The Literature

Austgulen et al., 1990Other/Unknown

Austgulen, R., Moe, P. J., Jørstad, S., & Widerøe, T. E. (1990). Treatment of refractory aplastic anemia with plasmapheresis: report of a case in childhood with review of the literature. Pediatric hematology and oncology, 7(3), 285–296. https://doi.org/10.3109/08880019009033404

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Abstract

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.

Case Details

Disease Location

Bone marrow

Personal Characteristics

An 11 years old male

Clinical Characteristics

Patient admitted for evaluation of anemia and thrombocytopenia. Two years before, he developed ecchymosis and was under observation. Evaluation showed no blood cells in bone marrow. Diagnosis of idiopathic aplastic anemia in a severe form.

Remission Characteristics

Hematological parameters remained constantly low until he had a sr one year after admission. When 1st sr occurred (lasted for 4 months), hb and white blood cell count normalized first, followed by normalizing of the platelet count six weeks afterwards. During the 2nd sr on the same year, all the cell lines normalized (lasted for nearly one year). Afte that he relapsed

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

Methylprednisolone (20 mg/kg per day for three days). Prednisolone medication every second day for a half year, antilymphocyte globulin. Steroids tretament (anasteron, 100 mg daily). Cyclosporine a (100 mg daily). None reported treatment gave response. In addition, he was given platelets and red blood cells once a week. After second relapse, he was treated with plasmapherisis achieving complete remission