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Hypogammaglobulinemia cll

Sotirios Sachanas, Gerassimos A Pangalis, Petros Karouzakis, Efstathios Koulieris, Maria Moschogiannis, Christina Kalpadakis, Xanthi Yiakoumis, Dimitra Rontogianni
Malakoplakia, a rare granulomatous disease of infectious etiology, is commonly observed in immunocompromised patients. Chronic lymphocytic leukemia (CLL) is characterized by profound immune dysregulation resulting in significant infection-related morbidity and mortality, and several drugs used in CLL treatment have a severe immunosuppressive effect. Ibrutinib, has become a new standard-of-care in patients with CLL, especially for those harboring unfavorable genetic characteristics such as 17 p deletion, with however, unknown long-term immunological consequences...
September 2016: Anticancer Research
Elif Gülsüm Ümit, Figen Kuloğlu, Ahmet Muzaffer Demir
BACKGROUND: Vancomycin resistant enterococci (VRE) are common pathogens of hospital-acquired infection. Long hospitalization periods, use of broad-spectrum antibiotics and immunosuppression are major risks for VRE colonization. AIMS: We aimed to evaluate patients' characteristics and factors which may contribute to VRE colonization. METHODS: Data of 66 patients with colonization and 112 patients without colonization who have been hospitalized in the Hematology clinic were collected...
August 16, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Kenneth K Yu, Constantin A Dasanu
Alemtuzumab is FDA-approved for the treatment of chronic lymphocytic leukemia (CLL). Nonetheless, its use for this indication has fallen out of favor due to serious concerns for infectious complications and increased risks of second malignancies from the profound and lasting immunosuppression. We report here in a patient with a rapidly progressive metastatic Merkel cell carcinoma (MCC) who was previously treated with alemtuzumab and fludarabine for CLL. He developed profound lymphopenia and hypogammaglobulinemia...
June 2016: Connecticut Medicine
Felipe Gomes Campos Amaral, Luiz Guilherme Cernaglia Aureliano Lima, Veruska Menegatti Anastacio Hatanaka, Sheila Aparecida Coelho Siqueira, Amaro Nunes Duarte-Neto
Chronic lymphocytic leukemia (CLL) is a lymphoid neoplasia with the B immunophenotype, which corresponds to the leukemic form of lymphocytic lymphoma. This entity is characterized, in most cases, by immunosuppression due to impaired function of immune cells, hypogammaglobulinemia, bone marrow infiltration, and immune dysfunction due to the neoplasia and the chemotherapy, when prescribed. We describe the case of a 63-year-old woman with a previous diagnosis of advanced CLL, refractory to treatment, who presented respiratory failure at the emergency department and died soon after hospital admission...
January 2016: Autopsy & case reports
Carlos A Ramos, Barbara Savoldo, Vicky Torrano, Brandon Ballard, Huimin Zhang, Olga Dakhova, Enli Liu, George Carrum, Rammurti T Kamble, Adrian P Gee, Zhuyong Mei, Meng-Fen Wu, Hao Liu, Bambi Grilley, Cliona M Rooney, Malcolm K Brenner, Helen E Heslop, Gianpietro Dotti
BACKGROUND: Treatment of B cell malignancies with adoptive transfer of T cells with a CD19-specific chimeric antigen receptor (CAR) shows remarkable clinical efficacy. However, long-term persistence of T cells targeting CD19, a pan-B cell marker, also depletes normal B cells and causes severe hypogammaglobulinemia. Here, we developed a strategy to target B cell malignancies more selectively by taking advantage of B cell light Ig chain restriction. We generated a CAR that is specific for the κ light chain (κ...
July 1, 2016: Journal of Clinical Investigation
S Lachance, A L Christofides, J K Lee, L H Sehn, B C Ritchie, C Shustik, D A Stewart, C L Toze, E Haddad, D C Vinh
Infections are a major cause of morbidity and mortality in patients with chronic lymphocytic leukemia (cll), who typically have increased susceptibility because of hypogammaglobulinemia (hgg) related to their disease and its treatment. Immunoglobulin replacement therapy (igrt) has been shown to reduce the frequency of bacterial infections and associated hospitalizations in patients with hgg or a history of infection, or both. However, use of igrt in cll is contentious. Studies examining such treatment were conducted largely before the use of newer chemoimmunotherapies, which can extend lifespan, but do not correct the hgg inherent to the disease...
February 2016: Current Oncology
Michael Asger Andersen, Fie Juhl Vojdeman, Mette Klarskov Andersen, Peter de Nully Brown, Christian Hartmann Geisler, Ole Weis Bjerrum, Carsten Utoft Niemann
Hypogammaglobulinemia is the most common immune deficiency in chronic lymphocytic leukemia (CLL). However, the prognostic significance in terms of morbidity and mortality remains controversial. We here evaluate the significance of hypogammaglobulinemia in terms of infections, treatment-free survival (TFS), and overall survival (OS). A total of 159 consecutive, newly diagnosed patients were included for analysis. Twenty-five patients (16%) had a moderate or severe infection within one year of diagnosis, but no associations were found between low immunoglobulin (Ig) levels and infections...
July 2016: Leukemia & Lymphoma
Eric M Ammann, Michael P Jones, Brian K Link, Ryan M Carnahan, Scott K Winiecki, James C Torner, Bradley D McDowell, Bruce H Fireman, Elizabeth A Chrischilles
In patients with hypogammaglobulinemia secondary to chronic lymphocytic leukemia (CLL) or multiple myeloma (MM), intravenous immune globulin (IVIg) may be administered to reduce the risk of infection. Since 2013, IVIg products have carried a boxed safety warning about the risk of thromboembolic events (TEEs), with TEEs reported in 0.5% to 15% of patients treated with IVIg. In this retrospective cohort study of older patients with CLL or MM identified from the Surveillance, Epidemiology, and End Results-Medicare Linked Database, we assessed rates of clinically serious TEEs in 2724 new users of IVIg and a propensity-matched comparison group of 8035 nonusers...
January 14, 2016: Blood
Clare Sun, Xin Tian, Yuh Shan Lee, Sreenivasulu Gunti, Andrew Lipsky, Sarah E M Herman, Dalia Salem, Maryalice Stetler-Stevenson, Constance Yuan, Lela Kardava, Susan Moir, Irina Maric, Janet Valdez, Susan Soto, Gerald E Marti, Mohammed Z Farooqui, Abner L Notkins, Adrian Wiestner, Georg Aue
Chronic lymphocytic leukemia (CLL) is characterized by immune dysregulation, often including hypogammaglobulinemia, which contributes to a high rate of infections and morbidity. Ibrutinib, a covalent inhibitor of Bruton tyrosine kinase (BTK), inhibits B-cell receptor signaling and is an effective, US Food and Drug Administration (FDA)-approved treatment of CLL. Inactivating germline mutations in BTK cause a severe B-cell defect and agammaglobulinemia. Therefore, we assessed the impact of ibrutinib on immunoglobulin levels, normal B cells, and infection rate in patients with CLL treated with single-agent ibrutinib on a phase 2 investigator-initiated trial...
November 5, 2015: Blood
Neil Gupta, Basile Njei
While it commonly occurs in the pediatric population, syncytial giant cell hepatitis is rare in adults, which is diagnosed histologically by the presence of multinucleated cells in the liver. The literature has recorded only approximately one hundred cases in adults during the past two decades in whom malignancy is rarely associated with giant cell hepatitis. Our patient is a 66-year-old woman who was diagnosed with chronic lymphocytic leukemia (CLL) and subsequently developed syncytial giant cell hepatitis...
November 2015: Journal of Digestive Diseases
Sameer A Parikh, Jose F Leis, Kari G Chaffee, Timothy G Call, Curtis A Hanson, Wei Ding, Asher A Chanan-Khan, Deborah Bowen, Michael Conte, Susan Schwager, Susan L Slager, Daniel L Van Dyke, Diane F Jelinek, Neil E Kay, Tait D Shanafelt
BACKGROUND: Although hypogammaglobulinemia is a well recognized complication in patients with chronic lymphocytic leukemia (CLL), its prevalence at the time of CLL diagnosis, and association with novel prognostic markers and clinical outcome is not well understood. METHODS: All patients at the Mayo Clinic between January 1999 and July 2013 who had newly diagnosed CLL and had a baseline assessment of serum immunoglobulin G (IgG) were included. The relation between hypogammaglobulinemia at diagnosis and the novel prognostic parameters time to first treatment (TFT) and overall survival (OS) were evaluated...
September 1, 2015: Cancer
Teresa Jurado-Camino, Raúl Córdoba, Laura Esteban-Burgos, Enrique Hernández-Jiménez, Victor Toledano, Jose-Angel Hernandez-Rivas, Elena Ruiz-Sainz, Teresa Cobo, María Siliceo, Rebeca Perez de Diego, Cristobal Belda, Carolina Cubillos-Zapata, Eduardo López-Collazo
Infections are a significant cause of morbidity and mortality in patients with chronic lymphocytic leukemia (CLL). The pathogenesis of infections is multifactorial and includes hypogammaglobulinemia, conventional therapy with alkylating drugs, and recently, purine analogs and mAb-associated T cells. Patients without these risk factors also suffer from infections, although the mechanism remains unknown. In a cohort of 70 patients with CLL, we demonstrated that their monocytes were locked into a refractory state and were unable to mount a classic inflammatory response to pathogens...
January 15, 2015: Journal of Immunology: Official Journal of the American Association of Immunologists
Amelia Maria Gaman, Ana-Maria Buga, Mihnea-Alexandru Gaman, Aurel Popa-Wagner
Chronic lymphocytic leukemia (CLL) is characterized by a predominant humoral immune deficiency predisposing the patients to infections. Oxidative stress leads to an increased immunoglobulin k light chain production in B cells and contributes to the antibodies' deficiency and hypogammaglobulinemia. Aim of the Study. To evaluate the global oxidative status in patients with CLL and to determine whether the administration of antioxidants decreases complications due to infections. Patients and Method. We studied 84 patients with CLL stratified by Binet staging...
2014: Oxidative Medicine and Cellular Longevity
Asif Najmuddin, Sarah Hadique, John Parker
Strongyloidiasis is a major global health challenge that is often underestimated in many countries. In immuno-compromised hosts, an autoinfection can go unchecked with large numbers of invasive Strongyloides larvae disseminating widely and causing hyperinfection, with fatal consequences. This review will highlight a case of gram negative bacteremia complicated by meningitis and Adult Respiratory Distress Syndrome (ARDS) as a fatal outcome of Strongyloides hyperinfection, commonly known to occur in the setting of immunosuppression...
January 2012: West Virginia Medical Journal
D Rizzo, J Chauzeix, F Trimoreau, J B Woillard, F Genevieve, A Bouvier, J Labrousse, C Poli, E Guerin, N Dmytruk, L Remenieras, J Feuillard, N Gachard
We examined the significance of IgM peaks in chronic lymphocytic leukemia (CLL), including its association with newly reported MYD88, BIRC3, NOTCH1 and SF3B1 mutations. A total of 27, 25, 41 and 57 patients with monoclonal IgM or IgG peaks (IgM and IgG groups), hypogammaglobulinemia (Hypo-γ group) and normal immunoglobulin serum levels (normal-γ group) were, respectively, included. IgM peaks were mainly associated with Binet stage C and the del(17p). Biased usage of IGHV3-48 was shared by both IgM and IgG groups...
February 2015: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Pralay K Sarkar, Paras Malhotra, P S Sriram
Chronic lymphocytic leukemia (CLL) is associated with a state of immunosuppression characterized by hypogammaglobulinemia as well as B and T lymphocyte dysfunction. Though opportunistic infections are common in CLL patients, particularly after treatment, reports of infections by endemic dimorphic fungi are very few. Here we report a case of pulmonary blastomycosis in a CLL patient who initially presented with an indolent pulmonary mass lesion. The pulmonary lesions progressed rapidly over a two-week period...
2014: Case Reports in Medicine
Fatima Dhalla, Mary Lucas, Anna Schuh, Malini Bhole, Rashmi Jain, Smita Y Patel, Siraj Misbah, Helen Chapel
Hypogammaglobulinemia is a common finding in chronic lymphocytic leukemia (CLL). Its incidence increases with disease duration and stage such that it is present in up to 85 % of patients at some point in their disease course. It is therefore important to monitor patients for the development of an antibody deficiency. However, not all patients with antibody deficiency secondary to CLL are symptomatic with bacterial infections. In addition patients are susceptible to viral, fungal and opportunistic infections as a result of iatrogenic immunosuppression and through a variety of disease-related mechanisms, which affect cellular immunity and phagocytes...
April 2014: Journal of Clinical Immunology
Lev Shvidel, Tamar Tadmor, Andrei Braester, Osnat Bairey, Naomi Rahimi-Levene, Yair Herishanu, Abraham Klepfish, Rosa Ruchlemer, Alain Berrebi, Aaron Polliack
OBJECTIVES: Hypogammaglobulinemia, commonly encountered in chronic lymphocytic leukemia (CLL), is one of the main causes of morbidity and mortality; however, its prognostic significance in patients diagnosed in early stages of disease remains uncertain. The aim of this study was to evaluate the predictive power of hypogammaglobulinemia at Bonet stage A. METHODS: Using the database of the Israeli CLL Study Group, we analyzed the relationship between low serum levels of IgG, IgA, and IgM; the presence of paraproteinemia, as well as other well-recognized prognostic markers in CLL; and time to first treatment (TTT) and overall survival...
July 2014: European Journal of Haematology
Katrina Vanura, Franz Rieder, Marie-Theres Kastner, Julia Biebl, Michael Sandhofer, Trang Le, Robert Strassl, Elisabeth Puchhammer-Stöckl, Thomas Perkmann, Christoph F Steininger, Kostas Stamatopoulos, Wolfgang Graninger, Ulrich Jäger, Christoph Steininger
Chronic lymphocytic leukemia (CLL) is characterized by progressive hypogammaglobulinemia predisposing affected patients to a variety of infectious diseases but paradoxically not to cytomegalovirus (CMV) disease. Moreover, we found reactivity of a panel of CLL recombinant antibodies (CLL-rAbs) encoded by a germ-line allele with a single CMV protein, pUL32, despite differing antibody binding motifs. To put these findings into perspective, we studied prospectively relative frequency of viremia, kinetics of total and virus-specific IgG over time, and UL32 genetic variation in a cohort of therapy-naive patients (n=200)...
2013: PloS One
Georg Günther, Bettina Dreger
Secondary hypogammaglobulinemia is one of the factors responsible for the increased susceptibility to infection in patients with chronic lymphocytic leukemia (CLL). This study assessed the therapeutic results, concomitant medication and tolerance of administering 5% intravenous immunoglobulin, secondary immunodeficiency and recurrent serious bacterial infections. A single center, post-marketing, observational clinical study was performed on 10 patients with a variety of hematological malignancies (CLL, follicular non-Hodgkin lymphoma, IgM-secreting immunocytoma, IgA plasmacytoma and myelodysplastic syndrome/non-Hodgkin lymphoma) who had been infused with IVIG from June 1994 to May 2009...
July 2013: Microbiology and Immunology
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