Malignant histiocytosis. The diagnosis based on chromosomal, immunologic and histiopathologic analysis.
A 38-year-old female suffered for several months from persistent fever without signs of infectious or immunologic disease. 57Co-bleomycin scintigraphy revealed abnormal concentration in the liver. Following a diagnostic laparotomy, the patient died from abdominal haemorrhage. Malignant cell infiltration was demonstrated in a biopsy from the liver. Histiocytic cells were demonstrated in a bone marrow smear obtained shortly before death. Investigation of cultured lymphocytes obtained from the patient the day before she died showed deficiency of T-lymphocytes. Investigations of chromosomes delineated several clones with polyploidi. The diagnosis of malignant histiocytosis was based on erythrophagocytosis, and immunologic and chromosomal aberrations. If malignant histiocytosis is the primary disease, a terminal stage of different diseases or a sign of complication secondary to immuno incompetence (e.g. virus infection) is an open question.
D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007621:Karyotyping; D007958:Leukocyte Count; D008099:Liver / Q000473:pathology; D008213:Lymphocyte Activation; D011123:Polyploidy; D013601:T-Lymphocytes / Q000276:immunology