A 1-year-old boy with Wiskott-Aldrich Syndrome (WAS) who developed malignant lymphoma is described. He showed various complications such as atypical lymphocytosis, disseminated intravascular coagulation (DIC), intracranial hemorrhage, macroamylasemia, and monoclonal gammopathy (immunoglobulin A kappa chain). Epstein-Barr virus (EBV) DNA was detected in the tumor tissue, and the monoclonality of B cells from the tumor tissue was established. EBV-associated lymphoma is frequently observed in immunocompromised patients including those with WAS. The development of macroamylasemia, which is rare in childhood, is discussed in relation to lymphoma and monoclonal gammopathy. This case is unique in that the EBV-associated malignant lymphoma developed at an early age and was accompanied by macroamylasemia.

Amylases↗

PubMedDate not supplied

T- and B-lymphocytes in immunological disorders.

The lymphocytes from the patients with primary immunodeficiency diseases and those under immunosuppressive conditions such as viral infection or administration of antimetabolites were studied by various parameters of T- and B-lymphocytes. T-lymphocyte specific antigen, spontaneous rosette formation with sheep erythrocytes, phytohaemagglutinin response of the lymphocytes and delayed hypersensivity skin reaction were used to assess T-lymphocytes, while complement receptor, surface immunoglobulin, serum immunoglobulin levels and antibody response to antigens were estimated as parameters of B-lymphocytes. 9 of infantile agammaglobulinemia, 8 severe combined immunodeficiency, 5 ataxia telangiectasia, d Di-George syndrome, 11 common variable immunodeficiency, 3 isolated IgA deficiency and 4 cases thymectomized more than 10 years previously were studied and discussed for the results. The peripheral blood lymphocytes, especially T-lymphocytes were reduced in the acute stage of measles infection, while they were increased in infectious mononucleosis caused by EB (Epstein-Barr) virus. Atypical lymphocytes observed in the later disease seemed to originate from mainly T-lymphocytes. Cyclophosphamide had suppressive effect selectively on B-lymphocytes.

Adult↗