Biomaterial Database

Malignant histiocytosis in childhood. Clinicopathological study of 4 cases. 1986

K Ornvold, and M H Nielsen, and N Clausen

Clinicopathological features of four paediatric cases of malignant histiocytosis (MH) are described. The patients all presented with fever, lymphadenopathy and anaemia. Two died from widespread disease within one month. Two patients, who received combination chemotherapy, were brought into remission and are currently without disease after 30 and 60 months, respectively. Diagnosis was made on the basis of infiltration by a mixture of anaplastic and well-differentiated phagocytic histiocytes in skin, lymph node, bone marrow and spleen. Immunohistochemical studies showed variable presence of lysozyme, alpha 1-antitrypsin (AT), and alpha 1-antichymotrypsin (ACT). Concanavalin A (Con A) receptors, recently suggested as a histiocyte marker, were found in the proliferating cells in all cases. S-100 protein was absent. The findings suggest that the prognosis in childhood MH remains guarded but not hopeless with aggressive combination chemotherapy. The presence of Con A receptors in paediatric MH cells with a more constant occurrence than lysozome, AT and ACT seems promising as an additional diagnostic tool although further studies are needed to examine the specificity of Con A receptors in relation to various other disorders of histiocytic proliferation.

UI	MeSH Term	Description	Entries
D007136	Immunoglobulins	Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.	Globulins, Immune,Immune Globulin,Immune Globulins,Immunoglobulin,Globulin, Immune
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008206	Lymphatic Diseases	Diseases of LYMPH; LYMPH NODES; or LYMPHATIC VESSELS.	Lymphatism,Status Lymphaticus,Disease, Lymphatic,Diseases, Lymphatic,Lymphatic Disease
D008297	Male		Males
D008854	Microscopy, Electron	Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.	Electron Microscopy
D009418	S100 Proteins	A family of highly acidic calcium-binding proteins found in large concentration in the brain and believed to be glial in origin. They are also found in other organs in the body. They have in common the EF-hand motif (EF HAND MOTIFS) found on a number of calcium binding proteins. The name of this family derives from the property of being soluble in a 100% saturated ammonium sulfate solution.	Antigen S 100,Nerve Tissue Protein S 100,S100 Protein,S-100 Protein,S100 Protein Family,Protein, S100,S 100 Protein
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D004791	Enzyme Inhibitors	Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.	Enzyme Inhibitor,Inhibitor, Enzyme,Inhibitors, Enzyme
D005260	Female		Females
D006651	Histocytochemistry	Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.	Cytochemistry