PURPOSE: Although an increasing number of children with cancer survive as a result of more intense chemotherapy with advanced supportive care, they may be immunosuppressed because of the anticancer chemotherapy with radiotherapy and are susceptible to severe infections. We surveyed the pattern of immunoglobulin deficiency after chemotherapy in childhood acute leukemia with severe infections. METHODS: We reviewed the medical records and laboratory reports of 11 acute leukemia patients who developed immunoglobulin deficiency after chemotherapy at the Department of Pediatrics, Kyungpook National University Hospital in Daegu, Korea from January 1995 to September 2003. RESULTS: Among 11 acute leukemia patients, the median time interval from the diagnosis of leukemia to that of immunoglobulin deficiency was 21 (2~44) months. At the diagnosis of leukemia, 10 patients had normal levels of IgG, IgA, and IgM. At the diagnosis of immunoglobulin deficiency, 9 patients had low level of IgG (median, 339 mg/dL), IgA (median, 14.1 mg/dL), IgM (median, 24.4 mg/dL), and the other 2 patients had isolated IgG deficiency and isolated IgA deficiency, respectively. They were treated with antibiotics and high dose intravenous immunoglobulin G. CONCLUSION: Acquired immunoglobulin deficiency is one of the causes of frequent and serious infections which develops in childhood acute leukemia with chemotherapy. We suggest that periodic monitoring of immunoglobulin levels is important for early detection and treatment.
Anti-Bacterial Agents ; Child ; Daegu ; Diagnosis ; Drug Therapy* ; Gyeongsangbuk-do ; Humans ; IgA Deficiency ; IgG Deficiency ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins* ; Korea ; Leukemia* ; Medical Records ; Pediatrics ; Radiotherapy

Rituximab, an anti-CD20 monoclonal antibody, is an effective target agent against the B lymphocytes in B-cell lymphoid malignancies and various lymphoproliferative diseases. Moreover, the toxicity of rituximab is less severe than that of conventional cytotoxic agents, which has promoted the widespread application of rituximab in the treatment of B-cell lymphoma. However, depletion of B lymphocytes by rituximab, which leads to secondary hypogammaglobulinemia, can cause deterioration of humoral immunity. Although immune reconstitution after hematopoietic stem cell transplantation is known to prevent prolonged hypogammaglobulinemia, very few cases of long-standing hypogammaglobulinemia have been reported. We report herein a case of prolonged hypogammaglobulinemia after rituximab-containing chemotherapy and splenectomy in a patient with non-Hodgkin's lymphoma and discuss the clinical significance and pathogenetic mechanism of this phenomenon with a literature review.
Agammaglobulinemia* ; B-Lymphocytes ; Cytotoxins ; Drug Therapy* ; Hematopoietic Stem Cell Transplantation ; Humans ; IgG Deficiency ; Immunity, Humoral ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin ; Splenectomy ; Rituximab

Newborn premature babies have lwo levels of transplacentally acquired maternal immunoglobulin which is mostly transferred after 32~34 weeks gestaton, therefore they may have IgG deficiencies that increase their susceptibility to bacterial infection. We performed this study to determine whether intravenous immunoglobulin (IVIG) therapy improves mortality or infection occurrance rate. From 1 october 1991 to 31 July 1992, 73premature newborn infants with gestational age< or =34weeks were enrolled: the theatment group, consisting of 43infants who received prophylactic intravenous immunoglobulin therapy (500mg/kg/week) and the control group, consisting of 30infants who did not receive. prophylactic intravenous administration of immunoglobulin to preterm infants with a gestational ageage< or =34week, at a dose of 500mg/kg/week, results in maintenance of a satisfactory serum IgG level throughout the high-risk period for infection. But the incidence rates of proven or very probable sepsis, mortality for sepsis and total mortality in the infants receiving intravenous immunoglobulin were not significant differences when compared with those in the control infants. No adverse effects were noted after immunoglobulin transfusions in our subjects. In conclusion, our study does not show any decrease in bacterial infection rate or in mortality rate, and no study in the literature has shown absolute proof of the prophylactic efficacy of IVIG in premature newborns. Larger studies are necessary to confirm these observations and to determine more effective dosing schedules and the optimal levels of orhanism-spectific antibodies. And specific hyperimmnue of monoclonal antibody preparations may be required to provide reliable sources of effective prophylactic to premature neonate with high risk in bacterial sepsis.
Administration, Intravenous ; Antibodies ; Appointments and Schedules ; Bacterial Infections ; Humans ; IgG Deficiency ; Immunization, Passive ; Immunoglobulin G ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature* ; Mortality ; Sepsis*

The IgG subclass deficiency is defined as a significant decrease in the serum concentrations of one or more subclasses of IgG in a patient whose total IgG concentration is normal. IgG subclass deficiency can predispose to recurrent sinopulmonary infections. A 29-year-old female patient with a 4-year history of bronchial asthma presented with cough, sputum, dyspnea, and recurrent respiratory infections. She had frequently been treated with antibiotics and systemic steroids for recurrent respiratory infections and acute asthma exacerbations. Chest X-ray and computed tomography showed pectus excavatum and bronchial wall thickening without lung parenchymal abnormalities. On immunological evaluation, she was found to have a low serum IgG3, with normal total IgG concentration. Under diagnosis of selective IgG3 deficiency, she was started on monthly infusions of intravenous immunoglobulin (IVIG) therapy. The frequency and severity of respiratory infections and acute asthma exacerbations were markedly decreased during 3 years of IVIG therapy. Our case report suggests that a patient who has underlying selective IgG3 deficiency and asthma may benefit from IVIG therapy as this can significantly reduce the incidence and severity of recurrent respiratory infections and acute asthma exacerbations.
Adult ; Anti-Bacterial Agents ; Asthma* ; Cough ; Diagnosis ; Dyspnea ; Female ; Funnel Chest ; Humans ; IgG Deficiency ; Immunization, Passive* ; Immunoglobulin G* ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Incidence ; Lung ; Respiratory Tract Infections* ; Sputum ; Steroids ; Thorax

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea.
Adolescent ; Agammaglobulinemia/congenital/epidemiology ; Age Distribution ; Child ; Child, Preschool ; Common Variable Immunodeficiency/epidemiology ; Female ; Genetic Diseases, X-Linked/epidemiology ; Humans ; IgA Deficiency/epidemiology ; IgG Deficiency/epidemiology ; Immunologic Deficiency Syndromes/*epidemiology ; Infant ; Infant, Newborn ; Job's Syndrome/epidemiology ; Male ; Prevalence ; Questionnaires ; Registries ; Republic of Korea/epidemiology ; Severe Combined Immunodeficiency/epidemiology ; Sex Distribution ; Wiskott-Aldrich Syndrome/epidemiology ; Young Adult

OBJECTIVETo explore the correlation between Fc γ RIII A (CD16A) and aortic atherosclerotic plaque destabilization in apoE knockout (apoE KO) mice and the intervention effects of effective components of chuanxiong rhizome and red peony root.

METHODSEight 8-week-old male C57BL/6J mice were selected as the control group. Forty 8-week-old male apoE KO mice were randomly divided into the model group, apoE KO + intraperitoneal injection immunoglobulin group (IVIG), apoE KO + simvastatin group (Sm), apoE KO + high dosage of xiongshao capsule (XSC) group (XSCH), and apoE KO + low dosage of XSC group (XSCL), 8 mice in each group. Mice in the control group were put on a normal diet, and others were fed with a high-fat diet. After 10-week different interventions, monocyte CD16 expression was detected by flow cytometry, aortic matrix metalloproteinase-9 (MMP-9) mRNA expression was detected using reverse transcription polymerase chain reaction, and serum tumor necrosis factor (TNF)-α level was detected using enzyme-linked immunosorbent assay.

RESULTSCompared with the control group, monocyte CD16 expression, aortic MMP-9 mRNA expression, and serum TNF-α level in the model group increased obviously (P<0.01). Injections of apoE KO mice with intraperitoneal immunoglobulin during a 5-day period significantly reduced the monocyte CD16 expression, aortic MMP-9 mRNA expression, and serum TNF-α level (P<0.01 or 0.05) over a 10-week period of high-fat diet. Indices above in the Sm group, XSCH group, and XSCL group decreased in a different degree. Of them, the aortic MMP-9 mRNA expression in XSCH group was lower than that in Sm group (P<0.05) and the monocyte CD16 expression and serum TNF-α level showed no significant difference between XSCH group and Sm group (P>0.05). Correlation analyses suggested positive correlation between monocyte CD16 expression and aortic MMP-9 mRNA expression or serum TNF-α level in IVIG group, XSCH group, and XSCL group.

CONCLUSIONSFcγR III A mediates systemic inflammation in the progression of coronary heart disease with blood stasis syndrome. XSC could stabilize atherosclerotic plaque by suppressing inflammation and its target was relative with FcγRIII A.

Animals ; Aorta ; drug effects ; enzymology ; pathology ; Apolipoproteins E ; deficiency ; metabolism ; Drugs, Chinese Herbal ; pharmacology ; therapeutic use ; Flow Cytometry ; Gene Expression Regulation, Enzymologic ; drug effects ; Lipopolysaccharide Receptors ; metabolism ; Male ; Matrix Metalloproteinase 9 ; genetics ; metabolism ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Monocytes ; drug effects ; metabolism ; Paeonia ; chemistry ; Phytotherapy ; Plant Roots ; chemistry ; Plaque, Atherosclerotic ; blood ; drug therapy ; enzymology ; pathology ; RNA, Messenger ; genetics ; metabolism ; Receptors, IgG ; metabolism ; Tumor Necrosis Factor-alpha ; blood