The authors report on 2 children with pernicious anemia, sisters, who presented with hypermelanosis as one of the clinical manifestations. The hypermelanosis disappeared with adequate treatment of vitamin B12 deficiency. Vitamin B12 deficiency should be considered in the differential diagnosis of a child presenting with hyperpigmentation and macrocytic red cell indices.
The case of a 7-year-old girl with acquired immunodeficiency syndrome treated for 5 years with AZT and intravenous gamma globulin is reported. Shortly before her demise she developed a pulmonary leiomyosarcoma and leiomyoma. Does prolonged survival in pediatric acquired immune deficiency syndrome increase the incidence of secondary malignancies?
Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder associated with hepatosplenomegaly, cytopenias, lymphohistiocytic accumulation in the reticuloendothelial system and hemophagocytosis. Immunological dysregulation can occur with defective Tand NK-cell function; macrophage activation and cytokine production. Elevated transforming growth factor beta (TGF/3) produced by activated T cells and monocytes can be anti-inflammatory, whereas interleukin-6 (IL-6) induces T-cell proliferation and activation. We report the clinical and immunological evaluation of a 5-month-old Hispanic infant with FHLH. The evaluation included TGF/3, IL-6, nitric oxide (NO), and extended lymphocyte phenotypes, CD3+, CD4+, CD8+, CD28+, CD95+, CD45RA+, CD62L+, HLA-DR+, CD38+, CD69+ using enzyme-linked immunoadsorbent assay (ELISA), Greiss reagent, and 4' color flow cytometry pre-and postchemotherapy with VP16 @ 65 mg/M2 and dexamethasone over 10 courses of treatment. TGF/3 (924.4-4286.8 pg/mL), IL-6 (215.4-4225 pg/mL) in PHA-stimulated supernatant mononuclear cells and serum NO (4.267-11.660 fiM) dramatically increased post-treatment along with the percentage of CD8+ T cells (22-30%) and NK+ cells (2-14%). Resting naive cells (CD45RA+CD62L+) decreased in CD4+ (75-52%) and CD8+ (64^47%) subsets. T-cell activation was suggested by increases in CD95 expression on CD4+ cells (1-19%) and co-expression of CD38+CD69+ (1-23%) and HLA-DR+CD69+(l-25%) on CD8+ cells. Immunoregulatory cytokine changes along with T-cell and NK phenotypes may indicate a direct or indirect effect of chemotherapy on the pathogenesis of FHLH. Thus, a favorable clinical and immunological effect was noted following chemotherapy for FHLH.'The
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