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Objective: To investigate the clinicopathological features of adult orbital xanthogranulomatous disease (AOXGD) with lacrimal gland reactive lymphoid hyperplasia. Methods: Retrospective case series study. The clinical and pathological data of AOXGD cases diagnosed and treated in Tianjin Eye Hospital from January 2002 to December 2021 was reviewed, and the clinical characteristics, radiologic findings and pathological characteristics of periocular and lacrimal gland lesions of 5 cases were retrospectively analyzed. The expression of IgG4 and IgG protein in periocular and lacrimal gland lesions was detected by immunohistochemical staining, and the role of IgG4 in AOXGD was preliminarily studied. Results: There were four females and one male with an average age of 53.8 years (39 to 77 years). Among the five AOXGD cases, there were three cases of adult-onset xanthogranuloma, one case of adult-onset asthma and periocular xanthogranuloma, and one case of necrobiotic xanthogranuloma. All cases involved both eyes. The swelling of eyelids was observed in five cases, and the yellow or pale yellow eyelid skin was found in two cases. Imaging examinations showed the tumor mainly involved the eyelids, subcutaneous tissues, anterior orbit and lacrimal gland. A large number of foam cells and typical Touton giant cells were found in the periorbital lesions, accompanied by different degrees of fibrosis. The fibrinoid necrosis was detected in one case of necrobiotic xanthogranuloma. The lacrimal gland lesions showed different types of reactive lymphoid hyperplasia, including IgG4-related disease in two cases, follicular lymphoid hyperplasia in two cases and focal lymphoid hyperplasia in one case. IgG4 levels of periorbital and lacrimal gland lesions were elevated in four cases. Asthma and elevated serum IgG4 were found in one case of adult-onset periocular xanthogranuloma. Three patients underwent surgical resection and adjuvant hormone or immunosuppressive therapy, and two patients underwent simple surgical resection. The patients were followed up for 1.5 to 10.0 years, one patient was lost, and four patients had no recurrence. Conclusions: AOXGD with lacrimal gland reactive lymphoid hyperplasia is a group of rare diseases. The periorbital lesions of that are characterized by proliferation of foamy histiocytes and Touton giant cells, and the lacrimal gland lesions of that manifest as IgG4-related disease in some cases.

Pulmonary nodular lymphoid hyperplasia, also known as pseudolymphoma, is an uncommon reactive lymphoproliferative disorder of unknown etiology that can be found in Sjögren's syndrome patients. Here, we present a case of a previously healthy woman in which the incidental finding of a lung mass compatible with nodular lymphoid hyperplasia led to the subsequent diagnosis of Sjögren's syndrome. We also performed a literature review for the association between both entities and described the main clinical aspects of the reported cases. Although its rarity, we consider that pulmonary nodular lymphoid hyperplasia should be considered in the differential diagnosis of lung nodules or masses among Sjögren's syndrome patients., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pulmonary lymphoid lesions span from benign to other forms of malignant diseases. Among these pulmonary lymphoid lesions, Nodular Lymphoid Hyperplasia (NLH) has an excellent prognosis. The surgical excision of NLH seems to be the only treatment, even if in some cases a spontaneous regression has been reported. Interestingly, these lesions may have similar clinical and radiographic presentations, making a histopathological examination vital for their differentiation. In this report we describe four cases of pediatric patients with a previous history of classical Hodgkin Lymphoma (HL) with documented or suspected NLH.

Rationale: Reactive lymphoid hyperplasia (RLH) of the liver is an uncommon benign lesion. It is usually difficult to differentiate from hepatocellular carcinoma (HCC), peripheral-type cholangiocellular carcinoma (CCC), combined HCC-CCC, and liver metastases. On account of its rarity and controversial issues, we report a case report and review the literature to discuss its clinical features, treatments, radiological, and immunohistochemical characteristics., Patient Concerns: A 54-year-old woman had a history of primary biliary cirrhosis and chronic cholecystitis. She complained of finding a lesion in the right liver during her last medical check-up by abdominal B type ultrasound. The Contrast-enhanced computed tomography revealed a circular and low-density lesion in the right posterior lobe of the liver, approximately 22.0 × 18.7 mm in size. On magnetic resonance imaging, the lesion showed low-signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Laboratory test results were almost normal., Diagnosis: After the postoperative pathological and immunohistochemical examination, the patient was finally diagnosed as having RLH., Interventions: The patient received right posterior lobe hepatectomy and cholecystectomy., Outcomes: The patient was discharged 11 days after surgery. No evidence of recurrence was noted 1 year after the surgery., Lessons: Although RLH of the liver is rare, it is necessary to be considered in a liver lesion, especially in female patients. This case report may advance the understanding of RLH of the liver and reduce the number of mistakenly diagnosed patients.

Lymphocytic myocarditis is an inflammatory disease of the heart that may present in a myriad of fashions ranging from mild febrile illness to florid myocarditis and cardiogenic shock. Given its nonspecific clinical presentation, the diagnosis of lymphocytic myocarditis is often challenging. The authors describe four cases of lymphocytic myocarditis in young women who presented with cardiogenic shock. Two patients survived and two died. This presentation has not been seen previously in Hawai'i and the public awareness of this condition is critical. Early diagnosis and the prompt initiation of biventricular mechanical circulatory support appear to have been critical in improving patient survival., Competing Interests: The authors declare that there is no conflict of interests regarding the publication of this paper.

A 49-year-old woman who was asymptomatic was found to have a small liver tumor on abdominal ultrasonography (US) at her annual health checkup. US revealed a hypoechoic, solid, mass measuring 17-mm in size in segment 6. The tumor markers associated with liver malignancy were negative. An infectious disease screen was negative for hepatitis B surface antigen, but positive for antibody to hepatitis B core antigen. Imaging studies using computed tomography (CT), magnetic resonance imaging (MRI), and CT angiography suggested a malignant liver tumor, such as hepatocellular carcinoma. Partial hepatic resection of the posterior segment was performed. The pathological diagnosis was pseudolymphoma of the liver.

DLBCL is the most common lymphoma subtype occurring in older populations as well as in younger HIV infected patients. The current treatment options for DLBCL are effective for most patients yet the relapse rate is high. While many biomarkers for DLBCL exist, they are not in clinical use due to low sensitivity and specificity. In addition, these biomarkers have not been studied in the HIV context. Therefore, the identification of new biomarkers for HIV negative and HIV positive DLBCL, may lead to a better understanding of the disease pathology and better therapeutic design. Protein biomarkers for DLBCL were determined using MALDI imaging mass spectrometry (IMS) and characterised using LC-MS. The expression of one of the biomarkers, heat shock protein (Hsp) 70, was confirmed on a separate cohort of samples using immunohistochemistry. The biomarkers identified in the study consisted of four protein clusters including glycolytic enzymes, ribosomal proteins, histones and collagen. These proteins could differentiate between control and tumour tissue, and the DLBCL immunohistochemical subtypes in both cohorts. The majority (41/52) of samples in the confirmation cohort were negative for Hsp70 expression. The HIV positive DLBCL cases had a higher percentage of cases expressing Hsp70 than their HIV negative counterparts. The non-GC subtype also frequently overexpressed Hsp70, confirming MALDI IMS data. The expression of Hsp70 did not correlate with survival in both the HIV negative and HIV positive cohort. This study identified potential biomarkers for HIV negative and HIV positive DLBCL from FFPE tissue sections. These may be used as diagnostic and prognostic markers complementary to current clinical management programmes for DLBCL.

Background: Information on the course and outcome of borrelial lymphocytoma (BL) is limited., Methods: The study comprised 144 adult patients (75 female, 69 male; median age, 49 years) who had BL diagnosed at a single center between 1986 and 2014 and were followed up for 1 year., Results: BL was located on the breast in 106 patients (73.6%), on the ear lobe in 27 (18.8%), and elsewhere in 11 (7.6%). The median duration of BL before diagnosis was 27 days (interquartile range [IQR], 9-68 days). Concomitant erythema migrans was registered in 104 of 144 patients (72.2%); other objective manifestations of Lyme borreliosis (LB) were present in 11 (7.6%). Immunoglobulin M and/or G borrelial serum antibodies were present in 72 patients (50%). Borreliae were isolated from BL lesions in 14 of 42 patients (33.3%) who had not received antibiotics before skin biopsy. Of 13 typed Borrelia strains, 11 were B. afzelii, 1 was B. garinii, and 1 was B. bissettii The median duration of BL after starting antibiotic treatment was 21 days ([IQR], 10-30 days); the average duration was longer in patients who were older, had longer BL duration before treatment, or had signs of disseminated LB. Treatment failure occurred in 14 of 144 patients (9.7%). Patients with signs or symptoms of disseminated LB before treatment had nearly 4 times higher odds of treatment failure (95% confidence interval, 1.22-13.07) than those without such symptoms. All patients with treatment failure had uneventful outcome after retreatment., Conclusions: BL is a rare manifestation of early localized LB. Fourteen-day antibiotic treatment, as used for erythema migrans, is effective., (© The Author 2016. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.)

Purpose: The purpose of this study was to determine the prevalence of lymphoid hyperplasia in the lower gastrointestinal tract and its role in patients undergoing colonoscopic examinations, particularly focusing on any allergic predisposition., Methods: A database search performed at the Department of Gastroenterology at Onomichi Municipal Hospital identified seven patients with lymphoid hyperplasia in the large intestine (i.e., cecum, colon, and/or rectum). Data regarding the endoscopic, biological, and pathological examinations performed and the allergic histories for each patient were retrospectively reviewed from the clinical records., Results: Median age of the patients (four males, three females) was 50 years. Lymphoid hyperplasia was seen in the cecum (n = 5), ascending colon (n = 2), and transverse colon (n = 1). Six patients (85.7%) had one of the allergic airway diseases: allergic rhinoconjunctivitis for pollen (n = 3), bronchial asthma (n = 1), infantile asthma (n = 1), or allergic bronchitis (n = 1). Drug allergy (n = 3) and urticaria (n = 2) were also found. All seven patients had one or more allergic diseases; however, none had a history of food allergy. Blood tests for allergens revealed that six patients (85.7%) had positive reactions to inherent allergens, whereas only one patient had a positive reaction to food allergens., Conclusions: Our results indicate that lymphoid hyperplasia in the large intestine may be associated with allergic airway diseases rather than with food allergies; thus, its presence may be useful to detect patients with underlying airway hyperreactivity.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare entity that typically occurs on the extremities of young females. Although linear arrangement of cutaneous lesions has been rarely reported, accompanying nail dystrophy has not been linked with this condition to our knowledge. We describe a case of linearly-oriented infiltrative papules and nodules on the index finger of a young female with associated onychodystrophy. Histology demonstrated a heavy lymphocytic infiltrate with plasma cells and proliferation of blood vessels consistent with APACHE. Our case is unique given the linear array of cutaneous lesions and associated nail dystrophy.

Background: Mycosis fungoides (MF) and pseudo-MF (or MF simulant) can be associated with B-cell malignancies, but distinction between a true neoplasm and a reactive process may be difficult., Objectives: To report seven patients with B-cell malignancy and folliculotropic MF or pseudo-MF and emphasize on criteria allowing distinction between the two conditions., Methods: We retrospectively and prospectively included seven patients with B-cell malignancy who presented skin lesions histologically consisting in a folliculotropic T-cell infiltrate and reviewed the literature on the topic., Results: Four men and three women had a chronic lymphocytic leukaemia (n = 6) or a MALT-type lymphoma (n = 1). Five patients had localized papules, and two had patches and plaques. Histological examination showed in all cases a diffuse dermal T-cell infiltrate with folliculotropic involvement and follicular mucinosis associated with clusters of the B-cell lymphoma, without significant expression of follicular helper T-cell markers. T-cell rearrangement studies showed a polyclonal pattern in the patients with papules and a monoclonal pattern in the cases of patches and plaques. Papular lesions had an indolent evolution, whereas patches and plaques persisted or worsened into transformed MF., Conclusion: Folliculotropic T-cell infiltrates associated with B-cell malignancies can be either a true folliculotropic MF or a pseudo-MF. The distinction between both conditions cannot rely only on the histopathological aspect, but needs both a clinical pathological correlation and the search for a dominant T-cell clone. Whether the neoplastic T and B cells derive from a common ancestor or the T-cell proliferation is promoted by the underlying B-cell lymphoma remains unsolved, but interaction between B and T cell in the skin does not appear to be dependent on a TFH differentiation of the T-cell infiltrate., (© 2014 European Academy of Dermatology and Venereology.)

Primary central nervous system lymphoproliferative disorders comprise a heterogenous group of intracranial disease, predominantly of the high-grade non-Hodgkin's lymphoma type. We report a 56-year-old woman who developed new-onset grand mal seizures and was found to have two small uniformly enhancing dural-based lesions, which were radiologically concerning for meningiomas. Biopsy demonstrated findings consistent with benign, reactive lymphoid tissue. The patient's seizures resolved post-operatively. To our knowledge, this is the first reported patient with intracranial pseudolymphoma presenting as grand mal seizures. This case highlights this rare differential consideration in a patient with symptomatic dural-based lesion., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Objectives: We performed an 8-year retrospective study to evaluate the presentation, clinical findings and nasopharyngeal biopsy results of adult nasopharyngeal pathologies., Methodology: This study included 1647 patients (801 males and 846 females) admitted to outpatient clinics. All patients underwent a nasopharyngeal biopsy for a nasopharyngeal mass. In addition, a blind biopsy was taken if there was suspicion of nasopharyngeal carcinoma, even in the absence of a mass lesion. The pathological diagnoses were analysed on the basis of the age, sex and clinical presentation of the patients., Results: Patient age ranged between 18 and 85 years; the mean was 36 years. Patient age differed significantly between those with benign and malignant disease (p=0.000); the risk of malignancy increased with age. Benign disease was found in 97.4% of the patients. Reactive lymphoid hyperplasia was the most common condition; it was found in 92.71% of benign cases. Undifferentiated nasopharyngeal cancer was the most common malignant disease, being found in 82.95% of all nasopharyngeal malignancies and in 4.43% of all nasopharyngeal disease. The most common symptom was nasal obstruction. The other main symptoms were hearing loss and neck mass. Neck mass was associated with malignancy., Conclusions: Benign disease of the nasopharynx is more common than malignant pathology in patients with a nasopharyngeal mass. Although adenoidal tissue undergoes regression in the adolescent period, this tissue may present as the chief cause of nasal obstruction in adults. Age and symptoms may predict malignant disease.

A 69-year-old man underwent endoscopic mucosal resection of a solitary polyp located in the cecum. After the procedure, a fecal mass and appendiceal orifice appeared under the cut surface. A diagnosis of reactive lymphoid hyperplasia was made based on the results of an immunostaining analysis, which revealed a segregated population of T cells and B cells in multiple lymphoid follicles. The aggregation of adipocytes forming a lipomatous area and granulation tissue was also observed. We speculate that the compaction of the fecal mass in the appendix triggered mucosal inflammation, resulting in the formation of the polyp, which concealed both the feces and appendiceal orifice.

Gastrointestinal symptoms are common in patients with common variable immunodeficiency disorders (CVID) and less frequent in X-linked agammaglobulinemia (XLA) although the exact prevalence is not well established. In this study, endoscopic screening was performed in 30 patients with CVID and four patients with XLA. Endoscopic and/or histological abnormalities were detected in 25 of 30 patients with CVID (83 %), regardless of symptoms, and in nine of these patients the results prompted medical treatment. Helicobacter pylori-associated gastritis, adenomatous polyps, and lymphoid hyperplasia were most frequently encountered; no malignancies were detected. Adenomatous polyps were found in two of the four patients with XLA at a relative young age. In conclusion, gastrointestinal pathology is frequent in patients with CVID regardless of symptoms. Patients with XLA seem to be at risk for colorectal adenomas at a young age., (© Georg Thieme Verlag KG Stuttgart · New York.)

The term pseudolymphoma refers to a heterogeneous group of benign reactive T-cell or B-cell lymphoproliferative processes of diverse causes that simulate lymphoma clinically and histologically but usually undergo spontaneous remission. Its pathogenesis is still unclear. The prognosis is good although some evidence suggests that pseudolymphoma may progress to lymphoma. Pseudolymphoma of the urinary tract is extremely rare. We herein report a case of pseudolymphoma of the renal sinus in a 70-year-old man, associated with a high grade urothelial carcinoma of the bladder and to a prostatic adenocarcinoma (Gleason score 6). A brief review of the literature is included. The kidney showed a well-defined, whitish soft mass which involved the renal sinus. Microscopically, the lesion of the renal sinus consisted of a proliferation of small to medium size lymphocytes (CD20 positive and Bcl-2 negative) sometimes arranged in hyperplastic follicular structures. The diagnosis was confirmed by molecular studies which showed an oligopolyclonal IgH rearrangement. To the best of our knowledge, this is the second case of pseudolymphoma with a complete molecular characterization ever described in the renal sinus and the first one associated with multiple urogenital carcinomas.

Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. The pathogenesis of conjunctival EMZL remains unknown. We describe an unusual case of EMZL arising from reactive lymphoid hyperplasia (RLH) of the conjunctiva. A 35-year-old woman had fleshy salmon-pink conjunctival tumors in both eyes, oculus uterque (OU). Specimens from conjunctival tumors in the right eye, oculus dexter (OD), revealed a collection of small lymphoid cells in the stroma. Immunohistochemically, immunoglobulin (Ig) light chain restriction was not detected. In contrast, diffuse atypical lymphoid cell infiltration was noted in the left eye, oculus sinister (OS), and positive for CD20, a marker for B cells OS. The tumors were histologically diagnosed as RLH OD, and EMZL OS. PCR analysis detected IgH gene rearrangement in the joining region (JH) region OU. After 11 months, a re-biopsy specimen demonstrated EMZL based on compatible pathological and genetic findings OD, arising from RLH. This case suggests that even if the diagnosis of the conjunctival lymphoproliferative lesions is histologically benign, confirmation of the B-cell clonality by checking IgH gene rearrangement should be useful to predict the incidence of malignancy.

Granuloma annulare (GA) is characterized histopathologically by 3 patterns: necrobiotic granuloma, interstitial incomplete form and, rarely, sarcoidal or tuberculoid granuloma. The amount of lymphoid infiltrate in GA is usually limited. We describe 10 cases of GA with prominent "pseudolymphomatous" lymphoid infiltrates mimicking cutaneous lymphoid hyperplasia. Patients were 6 males and 4 females (mean age 49.9 years, median age 47 years, age range 25-70). Lesions were localized to a limited area of the body (n = 6), or involved the entire trunk (n = 3), or were generalized (n = 1). The correct clinical diagnosis of GA was provided only in 30% of the cases. In all cases, histopathologic features were characterized by dense, nodular, superficial, and deep infiltrates of lymphocytes. Immunohistology revealed predominance of T lymphocytes in 7 of 7 tested cases. This "pseudolymphomatous" variant of GA represents a pitfall in the histopathologic diagnosis of the disease and may be misinterpreted as other types of cutaneous lymphoproliferative disorders.

Background and Aim: Intra-abdominal lymphadenopathy poses a diagnostic and management challenge in highly endemic regions for tuberculosis. Opting for empirical anti-tuberculosis treatment raises the risk of wrong or delayed treatment. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) is the procedure of choice for tissue acquisition from peri-luminal lymph nodes. We studied the utility of EUS-FNA in evaluating intra-abdominal lymph nodes of unknown etiology, in the setting of high endemicity of tuberculosis., Methods: Consecutive patients with intra-abdominal lymph nodes of unknown etiology underwent EUS-FNA using a 22-gauge needle. Final diagnosis was made on surgical histology or on 6-months follow-up. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and diagnostic yield were calculated., Results: Sixty-six patients were included. Final diagnoses were tuberculosis, 35 (53%); metastatic adenocarcinoma, 11 (16.7%); lymphoma, three (4.5%); carcinoid, one (1.5%) and reactive nodes, 16 (24.2%). EUS-FNA provided a diagnosis in 61 patients (92.4%). Sensitivity, specificity, PPV and NPV for diagnosing tuberculosis via EUS-FNA were 97.1%, 100%, 100% and 96.9%, respectively. In 10 (15.2%) patients receiving empirical anti-tuberculosis treatment, the final diagnoses were metastatic adenocarcinoma (5), lymphoma (2), carcinoid (1) and reactive adenopathy (2)., Conclusion: Despite being in a highly endemic area, almost half of the patients studied have a non-tuberculosis etiology. EUS-FNA is a safe and accurate procedure for establishing the diagnosis of unexplained intra-abdominal lymphadenopathy., (© 2011 Journal of Gastroenterology and Hepatology Foundation and Blackwell Publishing Asia Pty Ltd.)

Actinic reticuloid (AR) or chronic actinic dermatitis is considered a sunlight-induced pseudolymphoma (PSL) on light exposed areas of the skin, which primarily affects elderly males. The disease is a severe, chronic photosensitive dermatosis, first described by Ive et al. in 1969. PSL is a group of non-cancerous lymphocytic skin disorders that simulate malignant lymphomas, but the changes usually spontaneously regress. The clinical appearance of Actinic reticuloid is variable, usually characterized by an eczematous, pruritic eruption, predominantly present on the head and neck, or other sun exposed areas, but can involve any area of the body. Thereby, crucial characteristic is photosensitivity, where at action spectrum involves UVB, UVA and visible light beyond 400 nm. The disease is considered as PSL which histologically resembles lymphoma with immunohistochemical analysis of the cutaneous infiltrate revealing presence of activated T cells, numerous histiocytes, macrophages and B cells. Moreover, the development of malignant (non-cutaneous) T cell lymphoma in the course of AR has been reported. As the disease has chronic character, it requires significant changes in the patient's lifestyle and avoidance of provoking factors such as contact allergens or sources of intense light. Thus AR should be considered in every patient who presents with persistent, unclear, erythematous skin changes on the face and neck that are related to sun exposure.

Both lingual tonsil and adenoid are the lymphoid tissue members of Waldeyer ring. Enlargement of the lingual tonsil and adenoid occurs in children with persistent obstructive sleep apnea despite previous tonsillectomy and adenoidectomy relatively frequently.In this article, we present a case with upper airway obstruction and infection findings because of excessive lingual tonsil and adenoid tissue hyperplasia and, additionally, bilateral multiple cervical lymphadenopathies. The patient had had tonsillectomy and adenoidectomy 8 years ago. Etiopathogenetic mechanisms, clinical, radiologic, histopathologic aspects, and surgical therapeutic options for obstructive sleep apnea related to excessive reactive lymphoid hyperplasia in children are discussed reviewing the literature.

Background: Reports on patients with European Lyme borreliosis in whom borreliae were isolated from the blood are rare and nearly exclusively limited to those with solitary or multiple erythema migrans. Here we report on patients with other manifestations of Lyme borreliosis in whom borreliae were isolated from their blood., Patients and Methods: This is a retrospective review of the medical files of patients diagnosed with borrelial lymphocytoma, Lyme neuroborreliosis, Lyme arthritis and acrodermatitis chronica atrophicans at the Department of Infectious Diseases of the UMC Ljubljana, Slovenia, for whom a borrelia blood culture was ordered. The clinical features of patients whose blood culture tested positive for Borrelia burgdorferi sensu lato were reviewed, and the association between the proportion of patients with a positive blood culture and various clinical manifestations was examined., Results: Borrelia burgdorferi sensu lato was isolated from the blood of 1/53 (1.9%) patients with borrelial lymphocytoma, 6/176 (3.4%) patients with Lyme neuroborreliosis, 1/13 (7.7%) patients with Lyme arthritis, and 3/200 (1.5%) patients with acrodermatitis chronica atrophicans. The time interval from the onset of symptoms attributed to Lyme borreliosis and the blood culture ranged from 1 day to >2 years (median 3.5 weeks). At the time of the blood culture, erythema migrans was present in 4/11 (36.4%) borrelia blood culture-positive patients, i.e. in the patient with borrelial lymphocytoma, the patient with Lyme arthritis and the 2/6 patients with Lyme neuroborreliosis. Only two of these 11 (18.2%) patients had fever at the time of the blood culture., Conclusions: In European patients with Lyme borreliosis, borreliae can be cultured from the blood not only early in the course of the disease but also occasionally later during disease progression.

Background: Although reactive lymphoid hyperplasia (RLH) can be found in various organs, including the gastrointestinal tract, orbit, lung, and skin, its occurrence in the liver is rare., Case Report: We report the case of a 47-year-old RLH woman who was identified with RLH of the liver during clinical follow- up of primary biliary cirrhosis. The mass, found incidentally during a medical examination, appeared on ultrasonogram as a hypoechoic mass in the 7th segment of the liver. Further analyses using composed tomography, magnetic resonance imaging, and angiography suggested malignancy, and we performed lateral segmentectomy of the liver. Histologically, the tumor was composed of lymphoid follicles with germinal centers that expressed kappa and lambda light-chain B-cell markers at equal frequency, and no IgH gene rearrangements were detected in Southern blots. Based on these results, we identified the lesion as RLH., Conclusions: We suggest that this diagnosis be taken into consideration in other cases involving a space-occupying liver mass associated with autoimmune disease.

Nodular lymphoid hyperplasia is a rare, benign, lymphoproliferative disease usually found in the gastrointestinal tract. It has never been reported in the sinuses. We present an unusual case of pulmonary nodular lymphoid hyperplasia with locally invasive sinus involvement and describe the CT, MRI and positron emission tomography findings.

This selection reviews several topics in the news: - in internal medicine, the cardiovascular and pulmonary risk factors in progressive systemic sclerosis (circulating lupus anticoagulant, smoking, NT-pro BNP marker); the relations between lupus erythematous and polymorphic light eruption, or Jessner-Kanof syndrome; the diagnostic score of autoinflammatory syndromes; and the dysmetabolism syndrome of psoriasis; - in infectious diseases, the return of epidemic typhus; the emergence of TIBOLA rickettsiosis; the development of methicillin-resistant Staphylococcus aureus strains in both the nosocomial and community settings; and finally news on herpes group viral infections.

The multimodality management of visual pathway tumors frequently involves radiation. Most commonly, photons are delivered via multiple focused beams aimed at the tumor while sparing adjacent tissues. The dose can be delivered in multiple treatments (radiation therapy) or in a single treatment (radiosurgery). Children with visual pathway gliomas should be treated with chemotherapy alone, delaying the use of radiation therapy until progression. Definitive radiation therapy of optic nerve sheath meningiomas results in stable vision in most patients. Radiation therapy or radiosurgery for pituitary tumors can result in control of both tumor growth and hormone hypersecretion. Postoperative radiation therapy or radiosurgery of craniopharyngiomas significantly improves local control rates compared with surgery alone. Radiation therapy is highly effective for eradicating orbital pseudolymphoma and lymphoma. The risk of complications from radiation treatment is dependent on the organ at risk, the cumulative dose it receives, and the dose delivered per fraction.

Objective: To investigate the correlation of Epstein-Barr virus (EBV) infection and childhood lymphoma., Methods: Paraffin-embedded specimens of lymphoma collected between 1996 and 2005, including 36 Hodgkin lymphomas (HL) and 51 non-Hodgkin lymphomas (NHL), were included in this study. Paraffin-embedded specimens of reactive hyperplasia of lymph nodes (RL) collected during the same period were used as controls. Immunohistochemical (IHC) assay was used to detect EBV-LMP1 and in situ hybridization (ISH) to detect EBV-EBERs., Results: EBV was detected in 72.2% (26/36) of the Hodgkin lymphomas, 15.7% (8/51) of the non-Hodgkin lymphomas and 33.3% (15/45) of the reactive hyperplasia of lymph nodes. There was a significant difference among Hodgkin lymphoma, non-Hodgkin lymphoma and RL (P = 0. 000)., Conclusion: Childhood Hodgkin lymphoma is closely correlated with Epstein-Barr virus infection. However, the low rate of EBV infection detected in childhood non-Hodgkin lymphoma might be due to heterogeneous distribution of pathological types in this study.

Nodular lymphoid hyperplasia (NLH) is a rare condition with unknown etiology frequently associated with common variable immunodeficiency (CVID) and selective immunoglobulin A deficiency. NLH is a common cause of gastrointestinal bleeding. The risk of malignancy has been well recognized in subjects with NLH. The present knowledge about the nodular lymphoid hyperplasia (NLH) was described.

Pulmonary nodular lymphoid hyperplasia (NLH) is a term first suggested by Kradin and Mark to describe one or more pulmonary nodules or localized lung infiltrates consisting of reactive lymphoid proliferation. To date, there have been only a few cases of pulmonary NLH reported associated with autoimmune disorders. There is no case of NLH associated with Sjögren's syndrome from Korea in the medical literature. A 56-year-old woman was referred to our hospital with cough productive of sputum and chest tightness. The Computed tomography scans of the chest revealed multiple and well-defined peribronchiolar nodular opacities. A video assisted thoracoscopic surgery (VATS) biopsy was performed and the nodular opacity in the lung parenchyma was pathologically confirmed as NLH. Through meticulous review of patient's record, we found that she had been suffering from dry eye and dry mouth. The symptoms suggested Sjögren's syndrome, which was confirmed by specific laboratory tests including the Schirmer test, anti-nuclear antibody and anti-Ro/La antibody. The patient is followed regularly and has no further progression of symptoms.

Langerhans cell histiocytosis (LCH) has been described in association with a variety of neoplasms preceding, after, or synchronous with the other tumor. In some cases, a neoplasm may arise as a complication of therapy for LCH, and in others, the association may be coincidental. Synchronous occurrence has been reported most commonly in association with malignant lymphoma in which discrete proliferations of Langerhans cells (LCs) histologically indistinguishable from LCH are seen. In most cases, these LCs are closely related to or intermingling with the primary pathology. The nature of LCs in this context remains elusive with debate as to whether they represent a true clonal neoplasm or an exaggerated reactive phenomenon. The lack of evidence for LCH progression or disease elsewhere strongly supports the latter. We have encountered 5 examples of LCH-like proliferations occurring in the context of other lymphoproliferative disorders. These include 2 cases of mycosis fungoides and 1 of cutaneous B-cell pseudolymphoma, associations that to our knowledge have not been described before. Two patients were female, and the clonality of the LC proliferation was assessed using laser capture microdissection and the human androgen receptor. The results showed that the LCs forming discrete nodules in a case of cutaneous B-cell pseudolymphoma and a case of Hodgkin's lymphoma were polyclonal. This suggests that, at least in a proportion of cases, the aggregates of LCs occasionally identified within other lymphoproliferative lesions represent a reactive proliferation rather than a potentially aggressive second neoplasm.

Cutaneous pseudolymphomas (CPL) are benign cutaneous lymphoproliferative infiltrations of various origin, including among others bacterial infections, viral infections and drugs . Helicobacter pylori has been frequently founded in the stomach of patients with MALT lymphoma. In January 2001, a 43-year-old man was referred to our department because of a 1-month history of itchy erythematous patches, plaques and flat tumors on his body. Histological examination revealed nodular infiltrations composed of lymphocytes, plasma cells and histiocytes with exocytosis of lymphocytes within the epidermis. Molecular analysis of rearrangement of T cell receptor and immunoglobulin heavy-chain genes did not reveal monoclonality. Based on these clinical, laboratory and histopathological data, a diagnosis of cutaneous T cell pseudolymphoma (CTPL) was made. The patient was anti-H. pylori antibody-positive, and was treated with anti-H. pylori combination with the result that all of the tumors had disappeared by January 2002. The patient has maintained complete response up to the last follow-up visit in December 2005., (Copyright (c) 2006 S. Karger AG, Basel.)

Purpose: To report on a case of bilateral conjunctival lymphoid hyperplasia presenting with bilateral panuveitis in a female patient., Design: Observational case report., Methods: In this case excisional biopsy indicated lymphoid tissue. Immunohistochemistry showed a follicular architecture with appropriate zoning of B and T lymphocytes., Results: Causes of panuveitis were considered and excluded based on a detailed history, physical examination, and laboratory investigations. The bilateral panuveitis resolved on topical corticosteroids and visual acuity returned to normal., Conclusions: Ocular adnexal lymphoproliferative lesions consist of a spectrum of disease entities, including reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, and lymphoma. There are no established clinical criteria to differentiate between these lesions. This case highlights the importance of a thorough ophthalmic examination in these patients, as an intra-ocular inflammatory process may accompany the conjunctival findings. Furthermore, we advocate periodic follow-up examinations because of the small potential risk of developing ocular or systemic lymphoma in these patients.

Known causes of conjunctival salmon patches include lymphoma, amyloidosis, sarcoidosis, leukaemia and benign reactive lymphoid hyperplasia. The aetiology of benign reactive lymphoid hyperplasia is thought to be a localized reactive change induced by an irritative or antigenic stimulus. The case of benign reactive lymphoid hyperplasia reported herein occurred in a myopic patient with extremely thin sclera. The authors' hypothesis is that choroidal antigens are able to perfuse through thin sclera and act as chronic irritants to the overlying conjunctiva resulting in a lymphoid response and subsequent salmon patch formation.

Background: Photodynamic therapy (PDT) using topical delta-aminolevulinic acid (ALA) has been used for nonmelanoma skin cancers, including malignant cutaneous T-cell lymphomas. Moreover, PDT has been tried for benign inflammatory or infectious skin lesions., Objective: To evaluate the effects of ALA-PDT on skin lesions of lymphadenosis benigna cutis (LABC)., Patients and Methods: Two 16-year-old females with solitary and infiltrated nodules were treated 5 times with topical ALA-PDT., Results: Both patients responded well and showed dramatic clinical and histopathological improvement without visible scars., Conclusion: The results confirm that topical ALA-PDT is an effective and safe modality for the treatment of LABC, and that such treatment may be cosmetically beneficial., (Copyright 2005 S. Karger AG, Basel.)

Recent data suggest a critical role for dendritic cells (DCs) in the generation of immunoglobulin-secreting plasma cells. In the work reported herein, we analyzed the frequency of peripheral blood plasmacytoid DCs (pDCs) and myeloid DCs (mDCs) in a cohort of 44 adults with common variable immunodeficiency (CVID) classified according to their CD27 membrane expression status on B cells. A deep alteration in the distribution of DC subsets, especially of pDCs, in the peripheral blood of CVID patients was found. Patients with a reduced number of class-switched CD27+IgD-IgM- memory B cells and patients with granulomatous disease had a dramatic decrease in pDCs (P = 0.00005 and 0.0003 vs controls, respectively) and, to a lesser extent, of mDCs (P = 0.001 and 0.01 vs controls, respectively). In contrast, patients with normal numbers of switched memory B cells had a DC distribution pattern similar to that in controls. Taken together, our results raise the possibility that innate immunity contributes to pathogenesis in CVID.