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Objective: To evaluate adverse reactions of the Bacillus Calmette-Guérin (BCG) Statens Serum Institut (SSI) (Danish strain 1331) used as intervention in a randomized clinical trial., Design: A randomized clinical multicenter trial, The Danish Calmette Study, randomizing newborns to BCG or no intervention. Follow-up until 13 months of age., Setting: Pediatric and maternity wards at three Danish university hospitals., Participants: All women planning to give birth at the three study sites (n=16,521) during the recruitment period were invited to participate in the study. Four thousand one hundred and eighty four families consented to participate and 4262 children, gestational age 32 weeks and above, were randomized: 2129 to BCG vaccine and 2133 to no vaccine. None of the participants withdrew because of adverse reactions., Main Outcome and Measure: Trial-registered adverse reactions after BCG vaccination at birth. Follow-up at 3 and 13 months by telephone interviews and clinical examinations., Results: Among the 2118 BCG-vaccinated children we registered no cases of severe unexpected adverse reaction related to BCG vaccination and no cases of disseminated BCG disease. Two cases of regional lymphadenitis were hospitalized and thus classified as serious adverse reactions related to BCG. The most severe adverse reactions were 10 cases of suppurative lymphadenitis. This was nearly a fivefold increase compared to what was expected based on the summary of product characteristics of the vaccine. All cases were treated conservatively and recovered. Six of 10 (60%) families of children experiencing suppurative lymphadenitis compared to 117/2071 (6%) of those with no lymphadenitis indicated that the vaccine had more adverse effects than expected (p-value <0.001)., Conclusions and Relevance: BCG vaccination was associated with only mild morbidity and no mortality. A higher incidence of suppurative lymphadenitis than expected was observed. All children were treated conservatively without sequelae or complications., Trial Registration: Trial registration number NCT01694108 at www.clinicaltrials.gov., (Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.)

PFAPA syndrome represents the most common cause of recurrent fever in children in European populations, and it is characterized by recurrent episodes of high fever, pharyngitis, cervical adenitis, and aphthous stomatitis. Many possible causative factors have been explored so far, including infectious agents, immunologic mechanisms and genetic predisposition, but the exact etiology remains unclear. Recent findings demonstrate a dysregulation of different components of innate immunity during PFAPA flares, such as monocytes, neutrophils, complement, and pro-inflammatory cytokines, especially IL-1β, suggesting an inflammasome-mediated innate immune system activation and supporting the hypothesis of an autoinflammatory disease. Moreover, in contrast with previous considerations, the strong familial clustering suggests a potential genetic origin rather than a sporadic disease. In addition, the presence of variants in inflammasome-related genes, mostly in NLRP3 and MEFV, suggests a possible role of inflammasome-composing genes in PFAPA pathogenesis. However, none of these variants seem to be relevant, alone, to its etiology, indicating a high genetic heterogeneity as well as an oligogenic or polygenic genetic background.

Background: When administered to an immune-compromised patient, BCG (Bacille Calmette-Guérin) can cause disseminated and life-threatening infections., Objective: To describe the imaging findings in children with primary immunodeficiency and BCG-related infections., Materials and Methods: We reviewed the imaging findings of children with primary immunodeficiency treated at a children's hospital during 2012-2014 with localized or disseminated BCG infection. Imaging modalities included US, CT and radiography., Results: Nine children with primary immunodeficiency had clinical signs of post-vaccination BCGitis; seven of these children showed disseminated disease and two showed only regional lesions with characteristic ipsilateral lymphadenopathy. Overall, lymphadenopathy was the most prevalent feature (n = 8) and characteristically appeared as a ring-enhancing hypodense (CT) or hypoechoic (US) lesion. Visceral involvement with multiple abscesses appeared in the spleen (n = 2), liver (n = 1) and bones (n = 1). All lesions regressed following appropriate anti-tuberculosis treatment., Conclusion: BCG infection needs to be considered in children with typical findings and with suspected primary immunodeficiency.

Unlabelled: As of June 2014, among six patients who had severe fever with thrombocytopenia syndrome (SFTS) at our hospital, an 83-year-old man died despite receiving appropriate critical care. An autopsy revealed extensive ischemic damage of the intra-abdominal organs, including the liver, spleen, stomach and gut, due to severe celiac atherosclerotic stenosis and superior mesenteric arterial thrombosis. Many SFTS virus nucleoprotein antigen-immunoreactive cells were detected in a paraaortic node, where necrotizing lymphadenitis was seen, and in the spleen. Fewer such cells were seen in the liver, bone marrow and adrenals., Conclusion: Atherosclerosis, in addition to hemophagocytic lymphohistiocytosis syndrome, can be lethal in elderly SFTS patients.

A 25-year-old woman diagnosed 1 year earlier with Primary Sjogren's syndrome was admitted to a nearby hospital with fever of unknown origin. Examination revealed anasarca, systemic lymphadenopathy, hepatosplenomegaly and high C-reactive protein level. The patient's symptoms were initially suspected to be caused by severe bacterial infection with Sjogren's syndrome flare. She was given antibiotics and prednisolone (PSL) at 50 mg/day. However, the patient developed anemia and thrombocytopenia and was transferred to our hospital for further care. Histological examination of the right inguinal lymph node showed neutrophilic infiltration. Bone marrow aspiration revealed a normocellular marrow with increased megakaryocytes and mild reticulin fiber hyperplasia. After initiation of minocycline hydrochloride, the patient's symptoms improved. However, as PSL was tapered, her symptoms worsened. The patient's clinical symptoms and laboratory data improved again with initiation of intravenous steroid pulse therapy and cyclosporine. TAFRO syndrome is characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis and Organomegaly. Although histological findings of the lymph node in this case differed from previous reports, the patient's other symptoms and clinical course were similar to TAFRO syndrome. TAFRO syndrome can occur with several diseases, including infection, rheumatic disease and malignancies. We report a case in which infection might have triggered TAFRO syndrome.

Background: Bacille Calmette-Guérin (BCG) vaccine is one of the most widely used vaccines globally. Management of local BCG complications (injection site reactions and suppurative or non-suppurative lymphadenitis) varies between clinicians, and the optimal approach remains uncertain., Aim: To determine the clinical features, management and outcome of BCG complications at two large acute hospitals in London, United Kingdom., Methods: All children presenting with complications of BCG vaccination between January 2008 and December 2013 were included in this observational study. Medical and electronic laboratory records were reviewed to determine clinical features, treatment and outcome., Results: Sixty children presented with adverse reactions. Two-thirds (65%) presented with BCG lymphadenitis, one-third (30%) presented with injection site complications and two children (3%) presented with both injection site reaction and lymphadenitis; only one child (2%) had disseminated BCG disease. The majority (88%) of children with injection site reactions were managed conservatively; overall, 95% showed complete resolution within 6 months. Among children with lymphadenitis, 46% were managed conservatively, whilst 54% had anti-tuberculous therapy and/or a procedure (aspiration mostly, or surgery); complete resolution was seen in 59% of cases., Conclusions: Injection site reactions and non-suppurative lymphadenitis were generally managed conservatively, with good outcomes. There was more variation in management and outcome of suppurative lymphadenitis and the optimal approach remains uncertain., (Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.)

The hyper-immunoglobulin M (HIGM) syndrome is a heterogeneous group of genetic disorders characterized by recurrent infections, decreased serum levels of immunoglobulin G (IgG) and IgA, and normal/increased serum levels of IgM. Herein, we describe three Turkish siblings with HIGM syndrome who had a homozygous missense mutation (c.70C>T, p.Arg24Trp) in the activation-induced cytidine deaminase gene which results in autosomal recessive HIGM syndrome. Two of the siblings, sibling 1 and sibling 3, presented with cervical deep abscess and cervical tuberculosis lymphadenitis, respectively.

A 9-year-old HIV-infected child previously treated with inadequate doses of antitubercular drugs based on weight was admitted 5 months after initial tuberculosis (TB) diagnosis with acute hemiplegia and inguinal lymphadenopathies in a rural hospital in Tanzania. He was diagnosed with TB meningitis and lymphadenitis using Xpert Mycobacterium tuberculosis/rifampicin (MTB/RIF) assay. Rifampicin resistance was detected in the lymph node aspirate but not in the cerebrospinal fluid. His TB therapy was optimised based on available medications and antiretroviral treatment was initiated 6 weeks later. Despite these efforts, the clinical evolution was poor and the child died 12 weeks after admission., (2015 BMJ Publishing Group Ltd.)

Human infection with Histoplasma capsulatum and Cryptococcus runs the gamut from asymptomatic illness to disseminated disease. Though both are the most prevalent systemic mycoses in HIV-infected patients, simultaneous infection by both the pathogens rarely occurs. We document the first case from Asian subcontinent with concurrent infection with disseminated cryptococcosis and histoplasmosis infections in a HIV-infected Indian patient diagnosed by morphological examination of fine-needle aspiration cytology samples obtained from the enlarged lymph nodes on light microscopy and were later confirmed by culture studies. A prompt, accurate and timely diagnosis of the disseminated form of dual mycosis (or either of the mycosis as well) is of utmost importance which has obvious impact on early initiation of treatment. Fine-needle aspiration cytology is a rapid, cost-effective and reliable method to identify infection with Cryptococcus and Histoplasma and is comparable with the essential culture studies.

Concerted efforts to eliminate lymphatic filariasis worldwide have registered success; multiple rounds of mass drug administration have led to the interruption of transmission in many previously endemic areas. However, the management of patients with established clinical disease (e.g., lymphoedema, hydrocoele and acute dermatolymphangioadenitis) has not been addressed sufficiently. Two recent studies from Malawi underscore the need for accurate epidemiological and clinical data, and comprehensive morbidity assessments across various domains of daily life. Addressing these issues will guide the implementation of programmes to improve access to treatment and disability prevention for affected individuals in Malawi and beyond., (© The Author 2015. Published by Oxford University Press on behalf of Royal Society of Tropical Medicine and Hygiene. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive procedure for the diagnosis of mediastinal lymph nodes and masses. Its complications are rare and include hemorrhage, pneumothorax and infections such as mediastinitis. We report the case of a 51-year-old patient who presented with a localized subcarinal adenitis after EBUS-TBNA. Germs colonizing the oropharynx may have been dragged along by the echobronchoscope, inoculating the punctured mediastinal lymph node., (© 2015 S. Karger AG, Basel.)

Adult T-cell leukemia/lymphoma (ATL) sometimes causes opportunistic infections. A 53-year-old woman with systemic lymphadenopathies was diagnosed with ATL by inguinal lymph node biopsies and underwent oral chemotherapy. Two months later, high grade fever, lower abdominal pain and lymphadenopathy recurred. Computed tomography revealed the presence of lymphadenopathy with marked gas formation in the pelvic lesion. Blood cultures were suggestive of septic lymphadenitis by Bacteroides fragilis (BF). This represents the first demonstration of giant lymphadenitis with gas formation caused by BF in a patient with ATL. Notably, septic lymphadenitis is pivotal in the differential diagnosis of systemic lymphadenopathy in ATL.

Background: Bacillus Calmette Guerin (BCG) is the only available vaccine for tuberculosis (TB). Low grade complications in healthy recipients and disseminated vaccine associated complications among immuno-suppressed individuals were noticed globally after administration. Recently a series of clinically suspected BCG associated suppurative and non-suppurative lymphadenitis cases were reported from different regions of Saudi Arabia. However a molecular confirmative analysis was lacking to prove these claims., Methodology: During 2009-2010, 42 Mycobacterium bovis BCG suspected clinical isolates from children diagnosed with suppurative lymphadenitis from different provinces of the country were collected and subjected to 24 loci based MIRU-VNTR typing, spoligotyping and first line anti-TB drugs susceptibility testing., Principal Findings: Of the total 42 cases, 41 (97.6%) were Saudi nationals and particularly male (64.3%). Majority of the cases were aged below 6 months (83.3%) with a median of age 4 months. All the enrolled subjects showed left axillary mass which suppurated in a median of 4 months after vaccination. Among the study subjects, 1 (2.4%) case was reactive to HIV antigen and 2 (4.8%) case had severe combined immunodeficiency. Genotyping results showed that, 41 (97.6%) isolates were identical to the vaccine strain Danish 1331 and one to Tokyo 172-1. Phylogenetic analysis revealed all the Danish 1331 isolates in a single cluster., Conclusion: Elevated proportion of suppurative lymphadenitis caused by M. bovis BCG reported in the country recently is majorly related to the vaccine strain Danish 1331. However lack of nationwide data on real magnitude of BCG related adverse events warrants population centric, long term future studies.

We describe a fatal case of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome with human herpesvirus-6B (HHV-6B)-associated lymphadenitis and virus-associated hemophagocytic syndrome triggered by an over-the-counter medication to treat respiratory and influenza-like symptoms. Histologically, the structure of the lymph node was disrupted with infiltration of large lymphocytes carrying intranuclear acidophilic inclusion bodies. Immunohistochemistry and real-time PCR analysis revealed that these large lymphocytes were positive for HHV-6B. Numerous HHV-6 particles were detected in the inclusion body of the lymphocytes by electron microscopy. Interestingly, immunohistochemistry revealed that HHV-6B-infected cells in the lymph node were CD3(+), CD4(+), CD25(+), and FoxP3(+) T cells, indicating a phenotypic resemblance to regulatory T-cells. This case provides direct evidence of HHV-6 infection in CD25(+)/FoxP3(+) T cells in a case of acute lymphadenitis of DRESS syndrome, suggesting a significant role of HHV-6 infection of regulatory T-cells in the pathogenesis of DRESS syndrome., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: Little is known about the immunological milieu of the skin-draining lymph nodes (LNs) in mycosis fungoides (MF)., Objectives: We studied dendritic cell (DC) subsets in the dermatopathic lymphadenitis of MF patients., Methods: We immunohistochemically examined DC subsets and their distribution in 16 LN samples from 14 patients with MF (N1 LN, eight patients; N2, four; and N3, four), and we compared them with non-metastatic sentinel LNs from eight patients with melanoma., Results: The number of S-100 protein+ DCs was markedly increased in the LNs from the MF patients and the major component was DC-LAMP+ mature DCs in the outer and paracortex areas, where DC-SIGN+ immature DCs were relatively decreased in proportion. In contrast, DC-SIGN+ cells were relatively increased in proportion compared to DC-LAMP+ cells in the medulla. Although no significant difference was observed in the proportions of CD1a+ or Langerin+ DCs among the N1, N2, and N3 nodes, CD163+ M2-type macrophages were increased in number in the N2 and N3 nodes., Conclusions: Our observations indicate that mature DCs accumulate in the outer and paracortex areas in dermatopathic lymphadenitis and M2-type macrophages might increase in number during disease progression.

Patients presenting with cervical lymphadenitis are a complex and common occurrence in a general pediatric practice. Although Staphylococcus aureus (often methicillin-resistant S. aureus [MRSA]) and Streptococcus pyogenes predominate as causative pathogens, the next most common pathogens, Bartonella, atypical mycobacterium, and mononucleosis, also must be considered early on. The best way to diagnose and manage these cases initially is to proceed methodically, with detailed history and physical examination, initial streptococcal mono rapid tests, serial office visits, and complete blood counts. In nonviral cases, an empiric oral antibiotic trial is usually prescribed as early as possible to cover for MRSA or Bartonella. Very tender or reddened lymph nodes larger than 5 cm that are unresponsive and worsening may likely require inpatient parenteral antibiotics and sometimes surgical removal. The practitioner must also realize that submandibular and supraclavicular node locations are highly suggestive of atypical mycombacterium and cancer, respectively., (Copyright 2014, SLACK Incorporated.)

Background: Tuberculosis (TB) is a cause of 1.2-1.5 million deaths worldwide, including deaths from TB among HIV positive people. Determining the extent of immune cells belonging to cell mediated immunity and haematological parameters is critical to maximize the potential benefit of anti-tubercular treatment and case management., Materials and Methods: Comparative cross sectional study was conducted to determine the white blood cell (WBC) count, CD4, CD8, haemoglobin (Hgb), red blood cell (RBC) count, mean corpuscular haemoglobin (MCHC), mean corpuscular volume (MCV) between newly diagnosed TB patients and apparently healthy controls (HCs)., Results: From consecutively enrolled 108 TB patients, pulmonary TB (PTB) accounted for 48(44.4%), TB lymphadenitis accounted for 48(44.4%), and disseminated/miliary TB accounted for 12(11.1%). Analysis of variance revealed that mean ± SD of CD4 count of male TB patients (650 ± 224cells/µl) was significantly lower than male control group (883 ± 256 cells/µl) (p= 0.001). In a similar manner, the mean CD4 count of female TB patients (793 ± 332cells/µl) was lower than female control group (975 ± 300 cells/µl) (p=0.001). There was no statistically significant difference in CD8 counts between cases and controls for both genders. Forty (37.0%) TB patients had developed anaemia of whom 22(55%) were among PTB, 13(32.5%) from tuberculous lymphadenitis and 5(20%) from disseminated TB. Morphologically, from all anaemia among TB patients, normocytic normochromic anaemia accounted for 15(37.5%) followed by normocytic hypochromic anaemia 13(30.4%)., Conclusion: CD4 lymphopenia was significant among TB patients. Granulocyte count was increased. Mild anaemia was found major haematological abnormality among newly diagnosed TB patients.

Background: Immune reconstitution syndrome (IRS) is a relatively common complication in HIV-infected adults starting combination antiretroviral therapy (cART). Data on IRS in HIV-infected children remain limited and are largely restricted to resource-limited settings. This study investigated the incidence, spectrum and outcome of IRS in a pediatric cohort in the United Kingdom., Methods: Retrospective analysis of clinical events during the first 12 months after initiation of cART in 135 treatment-naïve, HIV-infected children in the United Kingdom over a 5-year period. Demographic and laboratory data were provided by the Collaborative HIV Paediatric Study., Results: The median age at cART initiation was 6.6 years (interquartile range: 2.3-10.2). The median CD4 lymphocyte percentage (CD4%) at baseline was 15% (median CD4 lymphocyte count: 390 cells/μL). Eight patients (5.9%) developed IRS (incidence: 5.7/100 person years). The IRS events comprised: Bacillus Calmette-Guerin-related complications (local ulceration/lymphadenitis; n = 4), pulmonary tuberculosis (n = 1), Mycobacterium avium intracellulare infection (n = 1), combined tuberculosis/Mycobacterium avium intracellulare infection (n = 1) and cutaneous herpes simplex (n = 1). The mortality was significantly higher in children with IRS than in those without (P < 0.0001). The only statistically significant risk factor for IRS identified was increment in CD4 count at 12 months after starting cART (P = 0.03)., Conclusions: The incidence of IRS was significantly lower than previously reported from resource-limited settings, likely reflecting less profound immunodeficiency at cART initiation and fewer coexisting opportunistic infections in our cohort. However, IRS events were associated with considerable morbidity and mortality. Therefore, preventive strategies that can reduce the risk of IRS in children need to be identified.

Lymphadenitis and abscess formation are the most common side effects of vaccination with Bacille Calmette Guerin (BCG). The lower the child's age at the time of vaccination, the higher the incidence of BCG lymphadenitis tends to be. Although various therapeutic approaches are in use for the treatment of BCG lymphadenitis, there is no consensus on which of them is optimal. This study aimed to determine whether oral cephalexin treatment hastens recovery from BCG lymphadenitis. The study involved 40 children (24 boys and 16 girls) with BCG lymphadenitis who were referred to Qazvin Children's Hospital, Qazvin University of Medical Sciences between December 2008 and the end of September 2009. The patients were randomly assigned to two groups of 20 patients each (12 boys and 8 girls in each group): group A patients did not receive any treatment and served as controls, and group B patients were treated with 50 mg/kg/day cephalexin syrup, administered in four doses, for 10 days. In all patients, clinical examination was normal, except for lymphadenitis. In all patients, BCG vaccination had been performed at birth, and polymerase chain reaction tests were positive for tuberculous bacilli. The recovery period and requirement of fine needle aspiration did not significantly differ between the two groups (P 0.05). This study showed that treatment with cephalexin does not hasten recovery from BCG lymphadenitis.

Purpose: Distant migration associated with sinus lifting procedures has not been investigated. In the present study, a case of distant migration of graft material was observed, and the potential mechanisms of migration were analyzed using material analysis and in vivo experiments., Materials and Methods: The migrated graft material was biphasic calcium phosphate-based alloplastic material (BCP), and its physical properties were compared with those of xenogenic material (Bio-Oss). The comparisons of the physical properties were performed using scanning electronic microscopic, x-ray diffraction, and Fourier-transform infrared absorbance spectra analysis. The comparative graft migration study was performed using the subcutaneous pocket model in rats (n = 10). The clinical case was analyzed by histologic section and energy dispersive x-ray (EDX) microanalysis., Results: The observed diffraction patterns from the Bio-Oss revealed characteristic diffractions for the hydroxyapatite phase, and those from the BCP revealed additional diffractions that could be assigned to the tricalcium phosphate phase. In the animal model, the graft migration distances observed in the BCP group were significantly greater than those observed in the Bio-Oss group (P = .012). In the clinical case, the lymphatic vessels of the submandibular gland contained foreign materials that were morphologically similar to those of the maxillary sinus. EDX microanalysis revealed that the particles in the lymphatic vessels exhibited calcium concentrations that were approximately 200 times greater than those in the adjacent glandular tissue., Conclusions: In the present study, BCP-based sinus grafts had migrated into the submandibular glandular area by way of the lymphatic chain in the presented clinical case., (Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective and Importance: Bartonella henselae infections are among the most common causes of fever and lymphadenopathies, but can lead to severe complications in immunocompromised hosts; early recognition of these infections is of paramount importance in immunocompromised patients., Clinical Presentation: Here we report the case of a renal transplant recipient who presented with fever, lymphadenopathies, and a splenic abscess secondary to Bartonella henselae infection, successfully treated with doxycycline., Discussion and Conclusions: We discuss the various clinical presentations of Bartonella henselae infections in immunocompromised patients and the available diagnostic tools for this potentially severe complication.

Background: Tuberculosis is a global emergency predominantly affecting developing countries. HIV has been the single most important reason for acquisition of tuberculosis for many patients. Conversely, tuberculosis can result in rapid progression of HIV disease. Ethiopia is a country affected seriously by HIV and tuberculosis. The main aim of this study is assessment of the types of tuberculosis and the extent of HIV infection among tuberculosis patients visiting private health institutions in Amhara region of Ethiopia., Methods: The study used a cross sectional method with data collected using well structured pretested questionnaires containing socio-demographic and clinical variables including HIV serostatus. The setting is tuberculosis treatment sites situated at 15 private health institutions in Amhara region., Results: A total of 1153 TB patients were included. The proportions of smear positive pulmonary TB, smear negative pulmonary TB, isolated extrapulmonary TB and disseminated TB cases were found to be 29.6%, 22.2%, 43.9% and 2.9%, respectively. TB lymphadenitis accounted for about 61% of the extrapulmonary cases followed by TB pleurisy (10.6%). Seventy percent of the patients had undergone HIV test, and 20% of them were HIV positive. Marital status, patient residence and type of TB are the major determinants of co-infection., Conclusion: The occurrence of pulmonary tuberculosis is relatively low. Tuberculosis/HIV co-infection is also lower than other reports.

Introduction: One of the preventive methods of pulmonary tuberculosis is bacillus Calmette-Guérin (BCG) vaccination and one of the common complications of vaccine is lymphadenitis. We aimed to evaluate clinical course of BCG lymphadenitis in children of Kerman Province, Iran., Method: 314 children with BCG lymphadenitis were enrolled in study. Patients were followed up monthly regarding clinical improvement and complications., Results: BCG lymphadenitis was improved in many patients during follow up; during 6 months 23.7% of patients had spontaneous regression. Only the primary size of lymphadenitis had significant effect on clinical course of lymphadenitis and developing complication (p = 0.0540)., Conclusion: Many patients improved without any intervention. The primary size of lymphadenitis may affect clinical course of lymphadenitis and dispose person to abscess and fistula. To confirm this result and evaluate efficiency of preventive surgery instead of observation further study is recommended.

Background: TIBOLA (tick-borne lymphadenopathy) is a rickettsiosis caused chiefly by R. slovaca, transmitted by a Dermacentor tick. We report five cases., Patients and Methods: Three patients were diagnosed at the initial inflammatory stage (facial oedema, necrotic eschar, lymphadenopathy, fever) and two at the stage of sequelae (alopecia and fatigue). Microbiological evidence was present in only one case., Discussion: TIBOLA is a form of rickettsiosis that is currently spreading in Europe. Clinical diagnosis is often made late because of the mild symptoms and the lack of knowledge among clinicians concerning the condition. Microbiological tests (serology, PCR, culture of eschar or serum samples) are negative in one third of cases. The reference treatment consists of antibiotics effective against intracellular bacteria, cyclines and macrolides., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

A 74-year-old woman was referred to our hospital for an evaluation of unidentified pneumonia. She gradually developed a high-grade fever with a growing infiltrative shadow on chest CT and an enlarging bilateral cervical mass. She was diagnosed with a pulmonary Mycobacterium fortuitum (M. fortuitum) infection with cervical lymphadenitis based on the results of an open biopsy of the cervical lymph node. While the patient's clinical condition resolved almost completely after treatment with multiple antibiotics, neutralizing autoantibodies to interferon-gamma (IFN-γ) were identified in her serum. The progression of disseminated M. fortuitum infection in immunocompetent patients may be affected by the presence of autoantibodies to IFN-γ.

This clinicopathological, immunohistochemical, electron microscopic, and serological study of 382 cases (148 male, 234 female) of necrotizing lymphadenitis (NEL) in Japan confirms NEL as a self-limited disease with characteristic clinical features: high fever (38-40 °C), painful cervical lymphadenopathy (88.3 %), and leukopenia (under 4,000/mm(3)) without seasonal occurrence. Patient age varied from 5 to 80 years, but 62.8 % was younger than 30 years. There were five recurrent cases and four familial cases. In several cases, elevated serum aminotransaminase and antinuclear antibodies were found. Early in the disease, peripheral blood CD8+ cells were more abundant than CD4+ cells, but CD8+ cells decreased gradually with clinical progression, leading to an increasing ratio of CD4+/CD8+ cells during clinical course. Morphological features of involved lymph nodes are numerous CD8+ large immunoblasts, smaller CD4+ lymphocytes, plasmacytoid dendritic cells, histiocytes, and macrophages, the latter with phagocytized CD4+ apoptotic lymphocytes. Granulocytes are generally absent. These characteristics suggest that NEL is a reactive disease characterized by diploid disrupted CD4+ cells and CD8+ cells transforming to blastic cells. The etiology of the disease remains unknown, although viral infection is suggested, and its pathogenesis might include autoimmunity. Clinical characteristics and cytological and histological findings on lymph node biopsies can improve NEL diagnosis.

Periodic fevers are acquired or inherited disorders of innate immunity, which were first described in the 1940s. The patients are typically young at onset and have regularly recurring fevers for a few days to a few weeks with systemic inflammatory symptoms that are interrupted by symptom-free periods. There is a variety of clinical manifestations including gastrointestinal complaints, myalgias, arthralgias, and rash. A differential diagnosis in these patients may include recurrent infections, other inflammatory disorders, and neoplastic disease. This clinical review focuses on a sample of autoinflammatory disorders including familial Mediterranean fever, tumor necrosis factor receptor 1-associated periodic syndrome, hyperimmunoglobulinemia D syndrome, the cryopyrin-associated periodic syndrome, and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. We review the basics, pertinent clinical and laboratory features, and management of each entity.

The diagnosis of syphilis remains challenging. The absence of classical features of the disease, such as the rash of secondary syphilis or genital lesion, may pose diagnostic difficulties. In this article, we report a case of secondary syphilis in which the clinical syndrome and pattern of fluorodeoxyglucose uptake mimicked malignant lymphoma. This case highlights the importance of thorough history taking including sexual contact. Clinicians should be alert for syphilis-underlying unexplained lymphadenopathy, even in the absence of typical rash or genital lesion.

Introduction: A foreign body reaction due to silicone where it is infiltrated or at the places to which it can migrate is known as siliconoma. The use of silicone in breast augmentation procedures can provoke this reaction at the neck level in cases of leakage from mammary implants., Methods: We reviewed the cases of patients with increased size neck lymph nodes who had previously undergone plastic surgery of the breast with highly cohesive silicone gel implants., Results: In a 10-year period, we identified 12 cases with silicone-infiltrated neck lymphadenopathies, histologically confirmed by fine needle aspiration. They represented 3.5% of patients attended for neck lymph node study. We removed those detected by physical examination and CT in 5 cases, due to pathological characteristics of the node or a previous malignant history. In 2 of these nodes recurred, and node size also increased in 2 of the other 7 non-operated cases. After implant removal, silicone leakage was observed in only 7 cases., Conclusions: Cohesive gel silicone used for mammary implants can generate increased neck lymphadenopathies as a secondary effect due to systemic reactions against the silicone when it migrates in cases of implant failure. Surgical options for involved nodes usually do not offer good long-term results., (Copyright © 2012 Elsevier España, S.L. All rights reserved.)

This article reports on a 62-year-old man, who presented with cervical mass and rather nonspecific symptoms. The medical history and clinical workup initially favored a malignant disease such as a carcinoma of unknown primary as the underlying cause. Eventually, the patient was diagnosed with a granulomatous lymphadenitis caused by Francisella tularensis subsp holarctica. Tularemia is a rare disease in Western Europe and can present in multiple ways encompassing almost asymptomatic infections and fatal disease. A rapid diagnosis is often hampered by nonspecific symptoms and the generally low prevalence and incidence of this disease in endemic countries. This case report also provides a comprehensive review of the literature on cervical tularemia and discusses the differential diagnoses.

Patients presented with the supraclavicular lymphadenopathy in the medicine department have a strong suspicion of serious illness like tuberculosis, sarcoidosis, toxoplasmosis and malignancy of lymphnode, blood, lung, upper GIT, breast, ovary, testes, and other sites of body. This prospective type of observational study carried out in the indoor and out patient department of medicine of Mymensingh Medical College Hospital over a period of 6 month from April 2011 to September 2011 to diagnose the causes of supraclavicular lymphadenopathy. Patient of either sex, 18 years or above presented with supraclavicular lymphadenopathy were included. Biopsy or FNAC were done. The study showed that mean age of the patient of supraclavicular lymphadenopathy that finally diagnosed as malignant was 49.7 years and that of non malignant was 33.7 years. Male patient have suffered more (60%) from malignant disease than that of female patient (40%). Discrete, hard, non tender either fixed or non fixed supraclavicular lymphadenopathy was found malignant (18 of 18 cases, 100%) and discrete, firm, tender lymphnode were found non malignant (5 of 5 cases, 100%). Increased frequency (11 of 28, 39.3%) of granulomatous inflammation from the tuberculoid lymphadenitis were found among the patient undergone supraclavicular lymphnode biopsy. FNAC result was also of simillar type and finally it was found that frequency of tuberculosis (20 of 53, 37.7%) was highest and bronchial carcima was the second most frequent diagnosis (14 of 53, 26.4%). This study showed that supraclavicular lymphadenopathy is associated mostly with serious disease like tuberculosis and malignancy.

We describe a case of a 2-week-old male infant who presented with a rapidly enlarging inguinal mass after having received both the bacille Calmette-Guérin (BCG) and hepatitis B vaccines at birth. The clinical picture raised suspicion of a neoplasm, and an excision biopsy was performed. It showed complete effacement of the lymph node architecture by a diffuse proliferation of monomorphic, mitotically active, and medium-sized T-cell blasts with strong expression of CD99. Coalescent necrotizing granulomas were also seen. The lymph node culture was negative for BCG. Upon expert review and additional molecular diagnostics, the initial pathological diagnosis of lymphoblastic T-cell lymphoma was changed to ectopic BCG lymphadenitis and hyperimmune post-vaccinal reaction. The atypical T-cell proliferation was most likely a result of the adjuvant effects of the co-administered vaccines. Post-vaccinal reactions usually involve the injection site or result in localized lymph node enlargements in the areas draining the inoculation site. This case highlights the importance of the clinical context for accurate interpretation of the pathological findings. In the setting of post-vaccinal lymphadenopathy, a biopsy is rarely needed but, when performed, should be interpreted with great caution.