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.
A case of methemoglobinemia (MHb) in a teenage woman, triggered by an acute ingestion of approximately 120 to 180 mg of cetirizine, allegedly, with no suicidal intent is described. The patient presented with anxiety and tremors and rapidly developed central cyanosis unresponsive to oxygen supplementation. There was a history of recurrent, spontaneously remitting, unprovoked “blue discoloration of hands.” Investigations confirmed the diagnosis of MHb, and the patient responded to ascorbic acid and methylene blue, although the baseline methemoglobin level remained slightly high. The exact enzymatic deficiency could not be ascertained as the patient refused to undergo complete testing. To the best of our knowledge, this is the first documentation of cetirizine as a causative agent for drug induced MHb. Cetirizine, a selective histamine H1 receptor antagonist is eliminated via oxidation and conjugation processes, which use pathways other than cytochrome P450 enzyme system. The metabolism could potentially create by-products, like superoxide or hydrogen peroxide, which could act as strong reducing agents and oxidize hemoglobin into ferric containing methemoglobin. In this case, an unusually high systemic load of the drug speculatively saturated and overwhelmed the protective enzyme systems, which resulted in clinical manifestation of MHb.
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
An 11-year-old girl presented with episodic abdominal pain of 2 years' duration. CT scan of the abdomen showed a mass in the tail of the pancreas. A distal pancreatectomy was done and the tumor was excised. Macroscopic and immunohistochemical studies were compatible with a solid and papillary epithelial neoplasm. This is a rare neoplasm with a decidedly female predominance. It has a very low malignant potential with a good prognosis. Surgical removal of the tumor is usually curative.
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