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An individual with Lennox-Gastaut syndrome developed pancytopenia. Peripheral smear showed red cell macrocytosis with stomatocytes and well-granulated pseudo Pelger-Huet neutrophils (Figure A ).
Bone marrow biopsy was moderately hypocellular. There was erythroid (bi- and multinucleate forms) (Figure B) and granulocytic atypia (hypolobated neutrophils) and marked dysmegakaryopoiesis (many osteoclast-like and small hypolobated megakaryocytes) (Figures C and D). Cytogenetic studies were normal. Clinical evaluation revealed supratherapeutic total and free serum valproate levels.
Hematologic toxicity of sodium valproate can manifest with trilineage dysplasia and cytopenias.
The blood counts improved within 5 days of valproate discontinuation.
This case highlights a therapy-induced dyspoiesis that mimics myelodysplastic syndrome. Clues supporting drug toxicity include well-granulated pseudo–Pelger-Huet neutrophils and predominance of osteoclast-like megakaryocytes.
Potential Competing Interests
The authors report no potential competing interests.