A 56 year-old female was admitted because of exertional dyspnea and general fatigue. Five years before admission, her chest roentgenogram showed linear, micronodular and reticular shadows in the both lower lung fields. Corticosteroid and antibiotics were given with slight remission. Two months before entry, she complained of exertional dyspnea.
On physical examination, dullness and diminished breath sounds were present over the lower area of the right lung. The liver, spleen and peripheral lymphnode were not palpable. The glove and stocking type of paresthesia and hypesthesia were present and deep tendon reflexes were diminished or absent. The hemoglobin was 11.2g/ml, the white-cell count was 11600, with 2% basophils, 1% eosinophils, 2% neutrophils, 5% monocytes, 66% lymphocytes and 2% abnormal lymphocytes with indented or segmented nuclei (ATL cells). The bone marrow examination showed 13.2% plasma cells with no myeloma cells and 1% abnormal lymphocytes. IgG was 4426mg/dl with M-protein of IgG-κ type. The other immunoglobulins were not suppressed. Anti-ATLA (ATL associated antigen) antibody was detected. Chest roentgenogram on admission showed increased linear, micronodular and reticular shadows in the same area as five years previously and a mass shadow in the right lower lung.
A transbronchial lung biopsy of the right lower lung revealed infiltration of ATL cells in the alveolar septa. The ATL cells were also detected in the bone marrow. A biopsy of the eczematous skin eruption showed Pautrier's microabscess in the epidermis and infiltration of ATL cells in the dermis.
One month after the beginning of treatment with vincristine, cyclophophamide and prednisolone, the symptoms, laboratory data and chest roentgenogram were moderately improved.
During the hospital course, vaginal cancer was detected. Two years after admission, she died because of vaginal cancer without exacerbation of ATL. Autopsy was not possible.
The causative agent of ATL is thought to be type C retrovirus and that of vaginal cancer is suspected to be herpes virus type 2. Therefore, she might have susceptibility to viral infections.
In summary, there are three intersting points in this report. (1) Pulmonary involvement of ATL cells may be over seven years, but luekemic change in peripheral blood was mild. (2) The pathogenic agents of ATL and vaginal cancer may be both viral infections. (3) This is the second case of the M-protein associated with ATL, and M-protein induced polyneuropathy.
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