High levels of substance P-like immunoreactivity were demonstrated by radioimmunoassay in the plasma and tumour of a patient with a medullary carcinoma of the thyroid.

An adult patient is described with hepatomegaly and sea-blue histiocytes in the bone marrow. A diagnosis of cholesterol ester storage disease was established following enzyme and lipid analyses on liver biopsy and cultured skin fibroblasts. Acid esterase activity was deficient (approx. 5% of controls) in liver and fibroblasts using [14C]-triolein or 4-methylumbelliferyl palmitate as substrates. Cholesterol ester levels were raised about 70-fold in liver, whereas triglyceride levels were only marginally raised. Marked accumulation of cholesterol esters was also demonstrated in cultured fibroblasts. Clinically, the patient responded favourably to phenobarbitone treatment. However, this was not reflected in liver acid esterase or lipid levels.

A 59-year-old white man had generalized lymphadenopathy, fever, weight loss, and hypercalcemia. Histologic examination of seven lymph nodes from three different anatomic sites revealed a diffuse histiocytic infiltrate containing numerous typical Michaelis-Gutmann (MG) bodies. Histochemical and immunohistochemical results confirmed the histiocytic nature of the predominant cell within the infiltrate. Approximately equal numbers of residual normal B-cells, T-helper, and T-suppressor cells were present. Electron microscopy revealed extracellular and intracellular MG bodies, many of which were membrane-bound. Special stains, electron microscopy, and lymph node culture did not demonstrate microorganisms. The histologic features of the infiltrate were those of malacoplakia. However, the disease behaved like a lymphoma, with the occurrence of lymphadenopathy and a fatal outcome.

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