Marked eosinophilia and the accumulation of eosinophils in diseased tissues occur with various disorders and physiologic conditions, such as allergic diseases, bronchial asthma, parasitic infection, hypereosinophilic syndrome, and cancer. In normal individuals, the level of blood eosinophils is tightly regulated and eosinophil localization is limited primarily to the gastrointestinal tracts. However, after the onset of disease, eosinophils increase in the blood and infiltrate into various organs in the body, such as lungs and skin. Importantly, selective tissue eosinophilia can be observed even in the absence of marked blood eosinophilia. For example, in patients with bronchial asthma, eosinophils, but not neutrophils, infiltrate into the airway tissues, release various inflammatory mediators, and cause damage to the airway mucosa. Eosinophilia may be harmful because of the proinflammatory effects of the eosinophils, but it may also be beneficial because of the anti-parasitic effects of these cells. This article focuses on the recent advances in our understanding of the mechanisms of eosinophilia and tissue accumulation of eosinophils as well as the roles of these cell types in various human diseases. We hope that this article will help to better understand the biology of eosinophils and the pathologic mechanisms of eosinophilic disorders, and may lead to the development of new therapeutic approaches for patients.

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Measuring minimal residual disease (MRD) in children with acute lymphoblastic leukemia (ALL) is becoming increasingly important. Several studies have shown that MRD assays can provide unique and important information about treatment response and risk of relapse. Flow cytometry is a practical and widely applicable approach for monitoring MRD in patients with ALL. It relies on the identification of immunophenotypes that are expressed in leukemic cells but not in normal cells in bone marrow and peripheral blood. These phenotypes can identify one leukemic cell among 10, 000 normal cells, and are currently applicable to approximately 95% of children with ALL. There is a strong correlation between flow cytometric measurements of MRD during clinical remission and treatment outcome, suggesting that these assays should be useful to guide the intensity of therapy.

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Sixteen children with hematological disease who had undergone allogeneic stem cell transplantation (SCT) were evaluated to determine the adverse effect of anthracycline (ATC) and cyclophosphamide (CY) used as the conditioning regimen on pre-and post-transplant cardiac function. Methods employed were resting ECG, echocardiography and ¹²³I-BMIPP (beta-methyl-iodophenyl-pentadecanoic acid) imaging. A cumulative ATC dose over 300 mg/m², especially over 400 mg/m², was predictable for pre-transplant abnormal findings by parameters such as uptake score (US) and heart mediastinum ratio (H/M). However, the cumulative ATC dose and pre-transplant mild abnormal cardiac findings did not correlate with post-transplant cardiac function. A 200 mg/kg dose of CY was predictable for decreased summated QRS amplitude (QRS sum) and left ventricular mass index (LVMI), however, there was no correlation between the CY dose and the values obtained through BMIPP imaging. Moreover, the CY dose was not a risk factor for worsening post-transplant fractional shortening (FS) as evaluated by echocardiography. In summary, ²³¹I-BMIPP imaging was useful for evaluating subclinical cardiac damage due to ATC before transplant, but not for predicting cardiac damage during the course of SCT.

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We investigated the clinical aspects of nine families and 12 children with hereditary spherocytosis, including diagnosis, complication, justification of splenectomy, and clinical course after splenectomy. Median age at diagnosis was 5.5 years (1 month to 11 years). Seven cases (58%) had a family history. In the neonatal period, 10 patients received phototherapy and 1 patient had exchange transfusion due to neonatal hyperbilirubinemia. Laboratory findings at diagnosis were : Hb of 7.4 2.1 g/dl, reticulocyte count of (27.6±22.1) ×10⁴/μl, and total bilirubin of 2.2±0.9 mg/dl. Seven cases (58%) concurred aplastic crisis due to human parvovirus B19 infection. Six patients received erythrocyte transfusions. Of the seven patients who underwent splenectomy, 6 were more than 5 years of age. All patients recovered Hb and had elevated platelet counts after splenectomy. All patients received pneumococcal vaccination (Pneumovax^®) before splenectomy and subsequent postsplenectomy infection was not observed. Splenectomy, on one hand, contributes not only to the recovery of anemia, but also to prevention of gallstone and hemolytic crisis. On the other hand, there is a small risk of subsequent postsplenectomy infection and ischemic heart disease. So it is requisite for practitioners to follow up their patients carefully.

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A girl with exocrine pancreatic insufficiency and deletion of mitochondrial DNA (mtDNA) has been observed clinically for 7 years. When the girl was diagnosed to have deletion of mtDNA, the deletion was seen in 68% of the muscular cells and 45% of the peripheral lymphocytes. Treatment with Coenzyme Q10 and other medicines was chosen because of the similarity of a deleted portion of the mtDNA to Pearson syndrome. The treatment induced transient symptomatic improvement. However, not only have her exocrine pancreatic function and renal function gradually become worse, but achromatic pigmentary retinal dystrophy and anemia have also developed. Significant ringed sideroblasts have been observed in bone marrow cells. It has been reported that most patients with Pearson syndrome die in the early stage of the disease and that, in surviving patients, a majority of the accompanying anemia recovers spontaneously in spite of the severity of anemia. Development of sideroblastic anemia after long survival is a specific finding to this case and this finding is very important to speculate the pathogenesis of deletion of mtDNA.

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We report the case of a 2-year-old boy with acute lymphoblastic leukemia who presented Charcot-Marie-Tooth (CMT) disease following the administration of vincristine (VCR) for acute lymphoblastic leukemia. After the patient received a total dose of 8 mg/m² of VCR, he demonstrated marked muscle weakness, distal muscle atrophy in the arms and legs, pes cavus, foot drops, claw hand, tremor, and diminished deep tendon reflex, suggesting the presence of CMT disease. By genetic analysis, he was shown to have duplication of the PMP22 gene, and was diagnosed as CMT type 1A. Although his mother and maternal grandmother were asymptomatic, they were also diagnosed as CMT type 1A. He has been on complete remission by chemotherapy without VCR. Care should be taken to pay attention to the possibility of masked CMT disease when administering VCR.

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