Three males in one family (two siblings and one maternal cousin) had an illness with cervical adenopathy, hepatosplenomegaly, and a fulminant febrile course. In the two survivors agammaglobulinemia developed. One of them became ill at the age of six months and had an Epstein-Barr-virus antibody titer of 1:10 during illness and convalescence. The white-cell count was 120,000 with 90 per cent lymphocytes, most being atypical and forming increased numbers of sheep erythrocyte rosettes. IgM was elevated, IgA normal and IgG decreased. Subsequently, all immunoglobulins were absent, and the Epstein-Barr-virus antibody titer became negative. Peripheral B-cell number remained normal, but abnormal lymph-node architecture associated with failure to respond to antigenic challenge indicated B-cell dysfunction. The pathogenesis of this entity may involve an abnormal T-cell response to transformation of B cells by Epstein-Barr virus, leading to B-cell dysfunction and agammaglobulinemia.

The fibromatoses are a heterogeneous group of disorders characterized by proliferation of fibroblasts. Infantile myofibromatosis is a variant that is distinctive because of its multicentric origin, appearance at birth, and cellular composition, which is predominantly myofibroblasts. We treated a patient with infantile myofibromatosis with the interesting clinical presentation of a linear lesion involving the left arm and shoulder, and aggressive hepatomegaly with jaundice secondary to fibroblastic infiltration of the common bile duct and gallbladder. Diagnosis was confirmed histologically and ultrastructurally. Excision of the cutaneous lesion was facilitated by tissue expansion of uninvolved regional tissue.

Diploid DNA content, advanced stage, unfavorable histology, and N-myc amplification are all associated with aggressive disease andpoorprognosis in childhood neuroblastoma. DNA diploidy is associated with advanced stage and unfavorable histology, but the relationships amongploidy, N-myc amplification, and proliferative activity are not known. To determine ifDNA diploidy is associated with N-myc amplification, we studied 29 neuroblastomas with flow cytometric analysis and Southern blot analysis. Clinical and histologicfeatures were also evaluated. Sixty percent of the Nmyc-amplified tumors were diploid, compared to 26% of the neuroblastomas, which lacked N-myc amplification (P = 0.11). In our analysis ofproliferative activity and N-myc amplification, a higher mean percentage ofcells in S phase was seen in the N-myc-amplified tumors (13.4%) than in the unamplified tumors (10%), but again the result was not statistically significant (P = 0.14). Significant associations were seen between unfavorable histology and DNA diploidy (P = 0.05), and between unfavorable histology and high proliferative activity (P = 0. 007). Our data suggest that Supported in part by The Deborah Goldfine Reva Smilgoff Memorial Fund for Cancer Research; the Shellie Harris Memorial Fund for Cancer Research; the American Cancer Society, Illinois Division, Inc. 88-33; and the Children's Memorial Institute for Education and Research. Flow cytometry at Northwestern University is supported by an ongoing gift from the Coleman Foundation.

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A 2 8/12 year old girl with acute mucocutaneous lymph node syndrome (Kawasaki disease), presented 17 months later with a new onset of mitral and aortic insufficiency. Congestive heart failure rapidly developed and double valve replacement was performed. Pathologic study of the excised valve tissue disclosed active valvulitis. This is the first report of late onset aortic and mitral valvulitis associated with Kawasaki disease.