This paper reported a case of malignant histiocytosis with laryngeal involvement as an initial sign.
A male, aged 37, who developed hoarseness and nasal obstruction in November, 1976, and visited us on April 8, 1977.
On examination, there were granulomatous changes in the right arytenoid area and the false cord, and inflammation of the oropharynx. Repeated biopsies of the lesion, however, showed inflammatory changes only. The patient continued a chronic course unresponsive to antibiotics and steroids.
In February, 1978, he became febrile and developed granulomatous changes extending to the aryepiglottic fold and epiglottis, and right side of the latter became necrotic and ulcerative.
Radiotherapy was initiated and dramatic regression of the lesion was seen. One month later, the patient developed a painful swelling on the left nasal wing, which was biopsied and diagnosed as malignant lymphoma (histiocytic type).
Radiationtherapy was again instituted and 2800 rads was given with dramatic improvement. Two months later, he developed red crusted subcutaneous nodules of about 2cm in diameter on the chest, back and both thighs.
Biopsy of the nodule on the chest revealed infiltration of atypical histiocytes. Further biopsies showed findings more compatible with malignant histiocytosis.
In October, 1978, he became icteric, febrile and leucopenic, and bleeding from the oropharynx was seen. He died of profuse bleeding from the oropharynx after 2 years from the onset of the disease. Autopsy was not obtained.
The characteristics of malignant histiocytosis in the upper respiratory tract (especially nose) are follows:
1) The initial lesion is seen at non-lymphnode bearing areas.
2) Localized and generalized lymphadenopathy and splenic enlargement are uncommon.
3) The histological features observed in the initial lesion usually polymorphic cell infiltration accompanied by lymphocytes, plasma cells and histiocytes. The degree of histiocyte proliferation is in the spectrum of several cells in a high power field to an almost monotonous pattern. In the monotonous proliferation, histiocytic cells show lack of cohesiveness. The erythrophagocytosis is also seen.
4)The specific esterase activity by α-Naphthyl-acetate method is observed in the atypical cells.
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