scholarly journals Common Variable Immunodeficiency (CVID): A Case Report

2017 ◽  
Vol 2 (1) ◽  
pp. 26
Author(s):  
Maryam Montazeri ◽  
Mohammad Reza Ebadpour ◽  
Farideh Kouchak ◽  
Naser Esmaeili

Common variable immune deficiency disease is the most prevalent acquired immune deficiency in human being after selective immunoglobulin A deficiency. It causes reduction of immunoglobulin levels and specific antibodies production and enhancement of recurrent and chronic infections risk, especially respiratory infections. CVID patients faces increased risk of granulomatous disease, autoimmune and phenomenon and malignancy. The disease involves males and females equally. Some studies showed that early diagnosis of CVID disease and regular treatment of patients with IVIG may have an efficient role in decreasing pneumonia and frequency of hospitalization due to infections and its complications. In this study we report a 16 years old girl with CVID, without clinical history of determined infection with recurrent sinusitis.

Author(s):  
Annamaria Biczok ◽  
Philipp Karschnia ◽  
Raffaela Vitalini ◽  
Markus Lenski ◽  
Tobias Greve ◽  
...  

Abstract Background Prognostic markers for meningioma recurrence are needed to guide patient management. Apart from rare hereditary syndromes, the impact of a previous unrelated tumor disease on meningioma recurrence has not been described before. Methods We retrospectively searched our database for patients with meningioma WHO grade I and complete resection provided between 2002 and 2016. Demographical, clinical, pathological, and outcome data were recorded. The following covariates were included in the statistical model: age, sex, clinical history of unrelated tumor disease, and localization (skull base vs. convexity). Particular interest was paid to the patients’ past medical history. The study endpoint was date of tumor recurrence on imaging. Prognostic factors were obtained from multivariate proportional hazards models. Results Out of 976 meningioma patients diagnosed with a meningioma WHO grade I, 416 patients fulfilled our inclusion criteria. We encountered 305 women and 111 men with a median age of 57 years (range: 21–89 years). Forty-six patients suffered from a tumor other than meningioma, and no TERT mutation was detected in these patients. There were no differences between patients with and without a positive oncological history in terms of age, tumor localization, or mitotic cell count. Clinical history of prior tumors other than meningioma showed the strongest association with meningioma recurrence (p = 0.004, HR = 3.113, CI = 1.431–6.771) both on uni- and multivariate analysis. Conclusion Past medical history of tumors other than meningioma might be associated with an increased risk of meningioma recurrence. A detailed pre-surgical history might help to identify patients at risk for early recurrence.


Blood ◽  
2012 ◽  
Vol 119 (7) ◽  
pp. 1650-1657 ◽  
Author(s):  
Elena S. Resnick ◽  
Erin L. Moshier ◽  
James H. Godbold ◽  
Charlotte Cunningham-Rundles

Abstract The demographics, immunologic parameters, medical complications, and mortality statistics from 473 subjects with common variable immune deficiency followed over 4 decades in New York were analyzed. Median immunoglobulin levels were IgG, 246 mg/dL; IgA, 8 mg/dL; and IgM, 21 mg/dL; 22.6% had an IgG less than 100 mg/dL. Males were diagnosed earlier (median age, 30 years) than females (median age, 33.5 years; P = .004). Ninety-four percent of patients had a history of infections; 68% also had noninfectious complications: hematologic or organ-specific autoimmunity, 28.6%; chronic lung disease, 28.5%; bronchiectasis, 11.2%; gastrointestinal inflammatory disease, 15.4%; malabsorption, 5.9%; granulomatous disease, 9.7%; liver diseases and hepatitis, 9.1%; lymphoma, 8.2%; or other cancers, 7.0%. Females had higher baseline serum IgM (P = .009) and were more likely to develop lymphoma (P = .04); 19.6% of patients died, a significantly shorter survival than age- and sex-matched population controls (P < .0001). Reduced survival was associated with age at diagnosis, lower baseline IgG, higher IgM, and fewer peripheral B cells. The risk of death was 11 times higher for patients with noninfectious complications (hazard ratio = 10.95; P < .0001). Mortality was associated with lymphoma, any form of hepatitis, functional or structural lung impairment, and gastrointestinal disease with or without malabsorption, but not with bronchiectasis, autoimmunity, other cancers, granulomatous disease, or previous splenectomy.


CytoJournal ◽  
2010 ◽  
Vol 7 ◽  
pp. 7 ◽  
Author(s):  
Liron Pantanowitz* ◽  
Michael Kuperman ◽  
Robert A. Goulart

Human immunodeficiency virus (HIV)-infected patients are at an increased risk for developing opportunistic infections, reactive conditions and neoplasms. As a result, a broad range of conditions are frequently included in the differential diagnosis of HIV-related lesions. The clinical history of HIV infection may, however, be misleading in some cases. Illustrative cases are presented in which knowledge of a patient's HIV status proved to be misleading and increased the degree of complexity of the cytologic evaluation. Case 1 involved the fine needle aspiration (FNA) of a painful 3 cm unilateral neck mass in a 38-year-old female with generalized lymphadenopathy. Her aspirate revealed a spindle cell proliferation devoid of mycobacteria that was immunoreactive for S-100 and macrophage markers (KP-1, PGM1). Multiple noncontributory repeat procedures were performed until a final excision revealed a schwannoma. Case 2 was a CT-guided FNA of a positron emission tomography positive lung mass in a 53-year-old man. The acellular aspirate in this case contained structures resembling fungal spore forms that were negative for mucicarmine and GMS stains, as well as cryptococcal antigen immunocytochemistry. A Von Kossa stain confirmed that these pseudo-fungal structures were calcified debris. Follow up revealed multiple calcified lung and hilar node based granulomata. Case 3 involved the cytologic evaluation of pleural fluid from a 47-year-old man with Kaposi sarcoma and recurrent chylous pleural effusions. Large atypical cells identified in his effusion were concerning for primary effusion lymphoma. Subsequent pleural biopsy revealed extramedullary hematopoiesis, documenting these atypical cells as megakaryocytes. These cases demonstrate that knowledge of a patient's HIV status can be misleading in the evaluation of cytology specimens, with potential for misdiagnosis and/or multiple procedures. To avoid this pitfall in the setting of HIV infection, common entities unrelated to HIV infection and artifacts should always be included in the differential diagnosis.


2017 ◽  
Vol 63 (1) ◽  
Author(s):  
Michał Larysz ◽  
Rafał Becht ◽  
Michał Falco ◽  
Przemysław Nawrocki ◽  
Ireneusz Kojder

ABSTRACTA case of glioblastoma multiforme in female patient suffering from common variable immune deficiency syndrome is presented. The patient with history of previously treated due to breast carcinoma and ovarian cancer. The patient underwent craniotomy twice followed by radiotherapy and chemotherapy with temozolomide and finally died after 11 months.


2021 ◽  
pp. 1-8
Author(s):  
Xingji Lian ◽  
Li Fan ◽  
Xin Ning ◽  
Cong Wang ◽  
Yi Lin ◽  
...  

<b><i>Background:</i></b> Gestation complications have a recurrence risk and could predispose to each other in the next pregnancy. We aimed to evaluate the relationship between a history of adverse pregnancy and maternal-fetal outcomes in subsequent pregnancy in patients with Immunoglobulin A nephropathy (IgAN). <b><i>Methods:</i></b> A retrospective cohort study from a Chinese single center was conducted. Pregnant women with biopsy-proven primary IgAN and aged ≥18 years were enrolled and divided into the 2 groups by a history of adverse pregnancy. The primary outcome was adverse pregnancy outcome, which included maternal-fetal outcomes. Logistical regression model was used to evaluate the association of a history of adverse pregnancy with subsequent adverse maternal and fetal outcomes. <b><i>Results:</i></b> Ninety-one women with 100 pregnancies were included, of which 54 (54%) pregnancies had a history of adverse pregnancy. IgAN patients with adverse pregnancy history had more composite maternal outcomes (70.4% vs. 45.7%, <i>p</i> = 0.012), while there was no difference in the composite adverse fetal outcomes between the 2 groups (55.6% vs. 45.7%). IgAN patients with a history of adverse pregnancy were associated with an increased risk of subsequent adverse maternal outcomes (adjusted odds ratio [OR], 2.64; 95% CI, 1.07–6.47). Similar results were shown in those with baseline serum albumin &#x3c;3.5 g/dL, 24 h proteinuria ≥1 g/day, and a history of hypertension. There was no association between a history of adverse pregnancy and subsequent adverse fetal outcomes in IgAN patients (adjusted OR, 1.56; 95% CI, 0.63–3.87). <b><i>Conclusion:</i></b> A history of adverse pregnancy was associated with an increased risk of subsequent adverse maternal outcomes, but not for adverse fetal outcomes in IgAN patients.


Blood ◽  
1994 ◽  
Vol 83 (4) ◽  
pp. 1067-1078 ◽  
Author(s):  
VL Ng ◽  
MH Hurt ◽  
CL Fein ◽  
F Khayam-Bashi ◽  
J Marsh ◽  
...  

Two B-cell lines, 2F7 and 10C9, were established by single cell cloning from biopsies obtained from two acquired immune deficiency syndrome patients with Burkitt's lymphoma. Representation of the original tumors was verified by demonstration of (1) identical biallelic rearrangement of Ig genes for 2F7 and (2) shared idiotype for 10C9. Both cell lines displayed cell-surface Ig and secreted Ig (IgM lambda for 2F7, IgM kappa for 10C9). IgMs from both cell lines immunoprecipitated actin; in addition, 2F7 IgM lambda immunoprecipitated recombinant human immunodeficiency virus type 1 (HIV-1) gp 160. 2F7 IgM lambda did not react with other autoantigens (double-stranded and single-stranded DNA, actin, bovine serum albumin, IgG), whereas 10C9 IgM kappa reacted with human IgG. The 2F7 IgM heavy-chain variable region (VH) showed a 95% nucleotide homology with a previously sequenced VHIII germline gene, hv3019b9, whereas the 10C9 IgM VH showed a 95% homology with a previously sequenced VHIV germline gene, VH4.21. Use of minimally modified VH genes and demonstration of reactivity with chronically present antigens (ie, actin, HIV-1 gp 160, or human IgG) suggests that B cells in HIV-1-infected individuals proliferating in response to chronic antigenic stimulation may be at increased risk for lymphomagenesis.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Robert S McKelvie ◽  
Michel Komajda ◽  
Barry M Massie ◽  
John J McMurray ◽  
Michael R Zile ◽  
...  

Background: Diabetes mellitus (DM), present in about a quarter of heart failure (HF) patients with reduced ejection fraction (HF-REF), is associated with increased risk of fatal and non-fatal cardiovascular (CV) events. Less is known about the prevalence and impact of DM in HF patients with preserved ejection fraction (HF-PEF). The prevalence and effect of DM on clinical outcomes were examined in patients enrolled in the Irbesartan in Heart Failure with Preserved Systolic Function Trial (I-PRESERVE). Methods: The I-PRESERVE trial randomized 4128 HF-PEF patients (EF≥45%) to receive irbesartan or placebo. The primary outcome of time to all-cause mortality or CV hospitalization (myocardial infarction [MI], stroke, worsening HF, atrial or ventricular arrhythmia or unstable angina) was compared between patients with and without DM over one year of follow-up. A combined HF endpoint (HF mortality and hospitalization) was also evaluated. Comparison of the outcomes between patients with and without DM was expressed as a hazard ratio (HR). The independent predictive role of DM was examined in a multivariable model (which included symptoms, signs, clinical history, CV examination, biochemical, and hematological findings). Results: In I-PRESERVE 27% had a history of DM at baseline. DM patients more often had a body mass index ≥30 (51% vs 38%), history of stroke (12% vs 9%), history of MI (28% vs 22%), estimated glomerular filtration rate <60 ml/min/1.73m 2 (34% vs 29%), and pulmonary congestion on chest x-ray (46% vs 38%). In patients with DM, 17% and 11% had primary and HF events, respectively within 1 year; for patients without DM, 11% and 6% had primary and HF events. In a multivariate analysis DM remained a significant predictor of primary events (HR 1.48; 95% CI 1.22, 1.79) or HF events (HR 1.67; 95% CI 1.32, 2.12). Conclusions: The prevalence of DM in HF-PEF is similar to that reported in HF-REF. HF-PEF patients with DM have a significantly worse outcome than those without DM and this increased risk is independent of other factors associated with a worse prognosis.


Blood ◽  
2010 ◽  
Vol 116 (1) ◽  
pp. 7-15 ◽  
Author(s):  
Charlotte Cunningham-Rundles

Abstract Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum immunoglobulin G, A, and/or M with loss of antibody production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune disease, and an increased incidence of cancer and lymphoma. For all these reasons, the disease phenotype is both heterogeneous and complex. Contributing to the complexity is that patient cohorts are generally small, criteria used for diagnosis vary, and the doses of replacement immune globulin differ. In addition, routines for monitoring patients over the years and protocols for the use of other biologic agents for complications have not been clarified or standardized. In the past few years, data from large patient registries have revealed that both selected laboratory markers and clinical phenotyping may aid in dissecting groups of subjects into biologically relevant categories. This review presents my approach to the diagnosis and treatment of patients with common variable immunodeficiency, with suggestions for the use of laboratory biomarkers and means of monitoring patients.


2014 ◽  
Vol 18 (2) ◽  
pp. 70-77 ◽  
Author(s):  
A. Dermata ◽  
A. Arhakis

SUMMARYNatural rubber latex is found in numerous medical and dental products. Adverse latex reactions in dental patients and practitioners have significantly increased since the introduction of universal precautions for infection control. These reactions range from contact dermatitis to potentially life-threatening hypersensitivity. Patients with a history of spina bifida, urogenital anomalies, multiple surgical procedures, allergic reactions or atopy, health care personnel and latex production workers are at increased risk of latex allergy. Diagnosis is based on a combination of clinical history and laboratory tests. Identification of latex sources and the avoidance of latex exposure are critical for protecting both dental patients and dental personnel.


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