Antibody-mediated pure neutrophil aplasia, recurrent myasthenia gravis and previous thymoma: case report and literature review.
We describe a case of neutrophil aplasia in a woman with recurrent myasthenia gravis and a past history of thymoma. Bone marrow showed virtually absent granulopoiesis but normal erythropoiesis and megakaryopoiesis. Bone marrow cultures showed no growth of granulocyte/mononuclear cell progenitors (CFU-GM). She was treated with immunosuppression including azathioprine, and her neutrophil count returned to normal. Serum before treatment, and also an IgG fraction thereof, inhibited CFU-GM growth both in autologous 'remission' marrow and in allogeneic marrow. She remains in complete remission 36 months after starting azathioprine. This association is extremely rare, and was formerly associated with a grim prognosis.
Mathieson
P W
PW
Department of Clinical Neurology, National Hospital for Nervous Diseases, Queen Square, London.
O'Neill
J H
JH
Durrant
S T
ST
Henderson
S J
SJ
Green
P J
PJ
Newsom-Davis
J
J
D000328:Adult; D000380:Agranulocytosis / Q000150:complications*; D000906:Antibodies / Q000276:immunology; D001379:Azathioprine / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D009157:Myasthenia Gravis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D009503:Neutropenia / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D013234:Stem Cells / Q000473:pathology; D013945:Thymoma / Q000150:complications*; D013953:Thymus Neoplasms / Q000150:complications*
D000906:Antibodies; D001379:Azathioprine