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Immunol Allergy Clin North Am ; 39(1): 31-47, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30466771


Secondary hypogammaglobulinemia is a common development in patients treated with immunomodulatory agents for autoimmune, connective tissue, and malignant diseases. It has been observed in the medical management of patients undergoing hematopoietic stem cell and solid organ transplantation. Some patients have preexisting immunodeficiency associated with these illnesses; immunosuppressive treatment magnifies their immune defect. This article reviews immunosuppressive medications, including biological treatments that cause secondary hypogammaglobulinemia. It summarizes risk factors for rituximab-induced hypogammaglobulinemia, such as preexisting low immunoglobulin G levels, CD19 levels, host factors, and additive effect of all immunomodulatory drugs used. The evaluation and management of secondary hypogammaglobulinemia are discussed.

Front Immunol ; 8: 798, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28769923


Mutations of the recombinase-activating genes 1 and 2 (RAG1 and RAG2) in humans are associated with a broad range of phenotypes. For patients with severe clinical presentation, hematopoietic stem cell transplantation (HSCT) represents the only curative treatment; however, high rates of graft failure and incomplete immune reconstitution have been observed, especially after unconditioned haploidentical transplantation. Studies in mice have shown that Rag-/- natural killer (NK) cells have a mature phenotype, reduced fitness, and increased cytotoxicity. We aimed to analyze NK cell phenotype and function in patients with mutations in RAG and in non-homologous end joining (NHEJ) genes. Here, we provide evidence that NK cells from these patients have an immature phenotype, with significant expansion of CD56bright CD16-/int CD57- cells, yet increased degranulation and high perforin content. Correlation was observed between in vitro recombinase activity of the mutant proteins, NK cell abnormalities, and in vivo clinical phenotype. Addition of serotherapy in the conditioning regimen, with the aim of depleting the autologous NK cell compartment, may be important to facilitate engraftment and immune reconstitution in patients with RAG and NHEJ defects treated by HSCT.

J Allergy (Cairo) ; 2016: 9040319, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26997960


Physicians underrecognize and undertreat anaphylaxis. Effective interventions are needed to improve physician knowledge and competency regarding evidence-based anaphylaxis diagnosis and management (ADAM). We designed and evaluated an educational program to improve ADAM in pediatrics, internal medicine, and emergency medicine residents from two academic medical centers. Anonymous questionnaires queried participants' demographics, prior ADAM clinical experience, competency, and comfort. A pretest assessing baseline knowledge preceded a 45-minute allergist-led evidence-based presentation, including practice with epinephrine autoinjectors, immediately followed by a posttest. A follow-up test assessed long-term knowledge retention twelve weeks later. 159 residents participated in the pretest, 152 participated in the posttest, and 86 participated in the follow-up test. There were no significant differences by specialty or site. With a possible score of 10, the mean pretest score (7.31 ± 1.50) was lower than the posttest score (8.79 ± 1.29) and follow-up score (8.17 ± 1.72) (P < 0.001 for both). Although participants' perceived confidence in diagnosing or managing anaphylaxis improved from baseline to follow-up (P < 0.001 for both), participants' self-reported clinical experience with ADAM or autoinjector use was unchanged. Allergist-led face-to-face educational intervention improves residents' short-term knowledge and perceived confidence in ADAM. Limited clinical experience or reinforcement contributes to the observed decreased knowledge.

J Allergy Clin Immunol Pract ; 2(5): 594-600, 2014 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25213054


BACKGROUND: As the indications and use of rituximab continue to expand, the reports of long-term effects of anti-CD20--mediated B-cell depletion on the immune system accumulate. OBJECTIVE: We report a group of patients with immunodeficiency who were treated with rituximab and present their immunologic data. METHODS: A retrospective chart review identified patients with immunodeficiency who received rituximab for treatment of their primary disease and required immunoglobulin replacement therapy (IGRT). Pre-IGRT immunoglobulins, specific antibodies, B-cells, and B-cell phenotype were recorded and analyzed. RESULTS: We identified 11 patients with immunodeficiency who received rituximab and required IGRT. Two of these patients were diagnosed with common variable immunodeficiency before rituximab treatment. Nine other patients had hypogammaglobulinemia and did not achieve an adequate response to polysaccharide vaccine. There was a significant delay in B-cell recovery. B-cell phenotypes identified predominantly naive B cells in the blood of these patients with significant decrease in switched and memory B cells. CONCLUSION: There are patients with persistent B-cell dysfunction long after rituximab treatment was discontinued. Some of these patients required IGRT. These patients should be distinguished from patients with primary immunodeficiency diseases. Routine baseline B-cell numbers and serum immunoglobulin levels before starting immunomodulatory therapy are required to help distinguish primary immunodeficiency diseases from secondary rituximab-induced, transient, and, at times, prolonged immune suppression. Periodic monitoring is prudent to identify immune recovery. Post-rituximab B-cell phenotyping may help identify the patients who will develop persistent immune dysfunction caused by an unidentified underlying disease or the prolonged effect of rituximab treatment.

Anticorpos Monoclonais Murinos/uso terapêutico , Síndromes de Imunodeficiência/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Adolescente , Adulto , Idoso , Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Pré-Escolar , Humanos , Imunoglobulinas/sangue , Imunoglobulinas/uso terapêutico , Síndromes de Imunodeficiência/sangue , Síndromes de Imunodeficiência/imunologia , Contagem de Linfócitos , Pessoa de Meia-Idade , Rituximab , Adulto Jovem
Ann Allergy Asthma Immunol ; 97(6): 755-8, 2006 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17201233


BACKGROUND: Severe combined immunodeficiency (SCID) is a rare primary immunodeficiency characterized by abnormal lymphocyte development and lymphopenia. It often presents during the first year of life with recurrent, opportunistic infections, failure to thrive, and malabsorption. OBJECTIVE: To advocate newborn screening for SCID. METHODS: We reviewed the case histories of dizygotic twins with Pneumocystis carinii pneumonia (PCP) at the age of 6 months. RESULTS: Full-term fraternal twin girls were born to nonconsanguineous parents. Twin A developed recurrent oral candidiasis at 2 months, followed by pneumococcal bacteremia and PCP. At 5 months she had failure to thrive, but her absolute lymphocyte count was normal (5,887 cells/mm3). Subsequently, twin B presented with acute respiratory distress and was also diagnosed as having PCP; her absolute lymphocyte count was 5,852 cells/mm3. Flow cytometric analysis of peripheral blood lymphocytes from both girls demonstrated a T-B+NK+ phenotype consistent with interleukin 7 receptor alpha-chain-mutation SCID. Both girls received a haploidentical T-cell-depleted bone marrow transplant from their mother. In the interim, a homozygous point mutation in the interleukin 7 receptor alpha-chain gene was identified in twin B. Both parents were found to be carriers. Twin A died of chronic lung disease 8 months after transplantation; twin B is currently thriving. CONCLUSIONS: Early diagnosis and treatment of SCID are associated with an increased rate of survival and improved long-term outcome. Some patients with SCID can present without lymphopenia. Thus, we advocate that more sensitive screening tests be considered for inclusion in the newborn screening program currently used in most states.

Transplante de Medula Óssea , Receptores de Interleucina-7/genética , Imunodeficiência Combinada Severa/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Linfopenia , Programas de Rastreamento , Mutação , Pneumonia por Pneumocystis/complicações , Imunodeficiência Combinada Severa/complicações , Imunodeficiência Combinada Severa/genética , Linfócitos T
AIDS Read ; 13(1): 39-47, 2003 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-12569894


Over the last several years, virologic assays for the detection and measurement of HIV-1 RNA have become important in the diagnosis and management of perinatal HIV infection. Since AIDS Clinical Trials Group 076, a number of prospective and retrospective analyses have investigated the role of viral load in perinatal transmission. Although no universal virologic threshold for perinatal HIV transmission has been established, much has been learned about the timing of perinatal transmission and the relationship between maternal viral load and disease progression in HIV-infected neonates. Virologic assays have become accepted as standards of care in monitoring viral load during pregnancy, diagnosing neonatal infection, and establishing prognosis and response to therapy in infected infants.

Infecções por HIV/diagnóstico , Infecções por HIV/terapia , HIV-1/genética , HIV-1/fisiologia , Transmissão Vertical de Doença Infecciosa/prevenção & controle , RNA Viral/análise , Carga Viral/métodos , Países em Desenvolvimento , Progressão da Doença , Monitoramento de Medicamentos/métodos , Infecções por HIV/epidemiologia , Infecções por HIV/transmissão , Humanos , Recém-Nascido , Transmissão Vertical de Doença Infecciosa/estatística & dados numéricos , Determinação de Necessidades de Cuidados de Saúde , Valor Preditivo dos Testes , Prognóstico , Pesquisa