Technical Report No. 5-87
Autopsy case of histiocytic sarcoma with transition to malignant histiocytosis complicated with various immunologic abnormalities
Eto R, Fujii H, Kitamura YSummary
An autopsy case is reported of histiocytic sarcoma with transition to malignant histiocytosis, complicated with various immunologic abnormalities as determined in clinical laboratory examinations.
The patient was a 1-year and 5-month-old female infant, who at age 6 months had developed skin eruption and dyspnea following oral administration of Amoxycillin for treatment of mild fever and cough. Laboratory studies revealed polyclonal hypergammaglobulinemia, strongly positive rheumatoid arthritis (RA) reaction, positive antismooth-muscle antibody, and a small amount of M protein in the serum and urine. She experienced a transient episode of Kaposi’s varicelliform eruption, but antiviral antibodies were negative. She expired due to respiratory failure. The clinical impression was juvenile RA.
An autopsy was performed 11 hours after death. No pathological evidence of RA or other collagen diseases was found in the joints and visceral organs. The tumor tissue was composed of round naked nucleus cells involving the bilateral lungs, left main bronchus, descending colon, and liver in a nodular fashion. Lymph nodes were affected diffusely, but in spleen and bone marrow, an infiltrating pattern. Tumor cells rarely showed a picture of erythrophagocytosis. These pathological findings were consistent with a diagnosis of histiocytic sarcoma with transition to malignant histiocytosis.
The various antemortem immunologic abnormalities can be best explained if it is assumed that these abnormalities are attributable to the dysfunction of Tgamma lymphocytes. Erythrophagocytic Tgamma lymphoma was also suspected, but it could not be confirmed because the essential immunologic data on surface markers of tumor cells was not obtained.
An autopsy case is reported of histiocytic sarcoma with transition to malignant histiocytosis, complicated with various immunologic abnormalities as determined in clinical laboratory examinations.
The patient was a 1-year and 5-month-old female infant, who at age 6 months had developed skin eruption and dyspnea following oral administration of Amoxycillin for treatment of mild fever and cough. Laboratory studies revealed polyclonal hypergammaglobulinemia, strongly positive rheumatoid arthritis (RA) reaction, positive antismooth-muscle antibody, and a small amount of M protein in the serum and urine. She experienced a transient episode of Kaposi’s varicelliform eruption, but antiviral antibodies were negative. She expired due to respiratory failure. The clinical impression was juvenile RA.
An autopsy was performed 11 hours after death. No pathological evidence of RA or other collagen diseases was found in the joints and visceral organs. The tumor tissue was composed of round naked nucleus cells involving the bilateral lungs, left main bronchus, descending colon, and liver in a nodular fashion. Lymph nodes were affected diffusely, but in spleen and bone marrow, an infiltrating pattern. Tumor cells rarely showed a picture of erythrophagocytosis. These pathological findings were consistent with a diagnosis of histiocytic sarcoma with transition to malignant histiocytosis.
The various antemortem immunologic abnormalities can be best explained if it is assumed that these abnormalities are attributable to the dysfunction of Tgamma lymphocytes. Erythrophagocytic Tgamma lymphoma was also suspected, but it could not be confirmed because the essential immunologic data on surface markers of tumor cells was not obtained.