Myasthenia gravis remission and anti-AChR ab reduction after immunosuppressive and anti-neoplastic therapy in a patient with thymic Hodgkin’s disease

Excerpt

Sirs: We report a case of thymic Hodgkin’s disease (HD) in a patient with sero-positive generalized myasthenia gravis (MG) whose successful treatment for HD was associated with reduction of anti-acetylcholine receptor antibodies (anti-AChR-ab) and a complete remission of myasthenic symptoms. The female patient developed MG at the age of 52; the diagnosis was in accordance with clinical, pharmacological and electrophysiological criteria; anti-AChR-ab were 21.3 pmoles/ml. Chest CT and MRI suggested the presence of a thymoma, though anti-titin and anti-ryanodine receptor (RyR) antibody detection was negative. In January 1995, the patient began anticholinesterase and immunosuppressive treatment (prednisone and azathioprine) and a clinical improvement was observed. In December 1995 she was thymectomized by Video Assisted Thoracoscopic Extended Thymectomy without sternotomy [1]. Pre-tracheal, anterior mediastinal and costo-phrenic fat plus a thymic residue with a 1.5 cm diameter mass were removed; histological examination revealed an unexpected nodular sclerosing syncytial Hodgkin’s lymphoma within an involuted thymus. Total-body CT and bone marrow biopsy confirmed a clinically stage II A HD with mediastinal bulk. In February 1996, immunosuppressants were discontinued and adriamycin (90 mg) plus vinblastine (11 mg) started. Chemotherapy was temporary interrupted after 3 sessions owing to the onset of serious side effects; it was then resumed in May 1996 (5 cycles of low doses of adriamycin-bleomycin-vinblastine-dacarbazine) followed by mediastinal radiotherapy (30 gray plus a burst 10 gray) 6 months after. Clinical follow-up demonstrated a complete remission of both MG and HD (see Fig. 1); by November 1996 no further therapy was necessary. Anti-AChR-ab progressively decreased to 1 pmol/ml in December 1998 (see Figure). In June 2003, while no signs or symptoms of MG and HD were present, the patient developed an intractable cough; a lung adenocarcinoma was soon diagnosed and 1 month later she died for heart failure.

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