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The lymphatic system has received increasing scientific and clinical attention because a wide variety of diseases are linked to lymphatic pathologies and because the lymphatic system serves as an ideal conduit for drug delivery. Lymphatic vessels exert heterogeneous roles in different organs and vascular beds, and consequently, their dysfunction leads to distinct organ-specific outcomes. Although studies in animal model systems have led to the identification of crucial lymphatic genes with potential therapeutic benefit, effective lymphatic-targeted therapeutics are currently lacking for human lymphatic pathological conditions. Here, we focus on the therapeutic roles of lymphatic vessels in diseases and summarize the promising therapeutic targets for modulating lymphangiogenesis or lymphatic function in preclinical or clinical settings. We also discuss considerations for drug delivery or targeting of lymphatic vessels for treatment of lymphatic-related diseases. The lymphatic vasculature is rapidly emerging as a critical system for targeted modulation of its function and as a vehicle for innovative drug delivery.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem predominant lymphocytic inflammatory disease, which often relapses without oral immunosuppressants. This report describes a 37-year-old male case of CLIPPERS without relapse for 1 year after early steroid treatment. He was admitted to our hospital because of sensory disturbance in the left side of his body and ataxic gait. Gadolinium-enhanced T 1 -weighted MRI revealed multiple punctate and curvilinear enhancements in the pons and right middle cerebellar peduncle. We started treatment with high-dose intravenous methylprednisolone (IVMP) therapy on the 20th day of the illness. His neurological symptoms dramatically improved. Follow-up MRI showed that the enhancing lesions disappeared. We diagnosed him with CLIPPERS based on the clinical course, radiological findings, and steroid response. He did not take any oral immunosuppressant after discharge. However, there was no clinical and radiological relapse for 1 year after the IVMP therapy. Although this case requires careful follow-up because of recurrence risk, early steroid treatment was possibly related to 1-year remission.

Background: The aim of this study was to evaluate the effects of the standardized supplement Robuvit® (oak wood extract) in defined diffuse, minimal lymphatic "retention" (DMLR)., Methods: Robuvit® has already been investigated in both primary and secondary (post-surgical, post chemo-radiotherapy) lymphatic insufficiency. This registry included subjects with diffuse, minimal lymphatic "retention" (DMLR). The registry management groups included women with mild-moderate limb swelling using standard management (SM) as controls. A second, comparable group used prevention with Robuvit® at the dosage of 3 cp/day (300 mg/day) for 4 weeks., Results: No tolerability problems or side effects were observed with the preventive supplementation. The management groups (34 women in total), including 18 women in Robuvit® and 16 in SM were comparable in age and baseline evaluations. After 4 weeks, in the Robuvit® group, edema scale values derived from ultrasound observations decreased significantly (P<0.05) at all measurement's sites, from the proximal (inguinal) level to the more distal (ankle-foot) level. No significant changes in edema were observed in control subjects. Generally, in areas with higher level of edema (distal areas at the foot and ankle), the edema decrease was larger than in more proximal, ultrasound measurement sites., Conclusions: Preventive Robuvit® supplementation appears to be safe and effective in controlling DMLR in subjects without significant or apparent clinical conditions. This preventive, concept study should be extended to a larger population for more meaningful observations.

CLAPO syndrome (Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial or generalized Overgrowth) is a nonfrequent pathology. This syndrome is characterized by the capillary malformation (CM) of the lower lip, a very important clinical sign when diagnosing CLAPO. The aim of our report is to demonstrate that rapamycin could be a reliable and safe targeted therapy in lymphatic malformations (LMs). This drug is useful in reducing the LM's size before final surgical treatment. The clinical and radiological evolution of a patient with CLAPO syndrome is reported in this article, before and after the treatment with rapamycin., (© 2019 Wiley Periodicals, Inc.)

The issues of absorption, bacterial intestinal metabolism and hepatic metabolism of diosmin are described. The main metabolites of the drug and the ways of their elimination are indicated. The article describes the main therapeutic targets and mechanisms of influence on the course of disease including effect on the venous wall tone and permeability, lymphatic drainage, inflammation and oxidative stress.

Rationale: Tuberculosis remains a serious menace to the health of people. Isolated hepatic tuberculosis is rare and pancreatic tuberculosis is extremely rare. The preoperative diagnosis of pancreatic tuberculosis remains a great challenge., Patient Concerns: A 58-year-old Asian woman was referred to our hospital for evaluation of low back pain for 4 years and abdominal pain for 1 month., Diagnoses: Computed tomography (CT) of the abdomen showed a hypodense mass in the pancreatic head and neck with abundant calcifications, a hypodense lesion in the liver without calcification, peripancreatic lymphadenopathy, calcifications in some lymph nodes. CT-guided fine needle aspiration biopsy of the hepatic lesion was carried out and the cytological examination revealed hepatic tuberculosis., Interventions: The patient was treated with antituberculous therapy for 1 year., Outcomes: Low back pain and abdominal pain disappeared 3 months after initial treatment and after 2 year of follow-up, the patient was asymptomatic., Lessons: Our data hint that calcifications in both pancreatic lesions and peripancreatic lymph nodes may suggest pancreatic tuberculosis rather than pancreatic malignancy.

Intralymphatic histiocytosis is a rare disorder associated with a variety of inflammatory conditions. We report the case of an 89-year-old woman with a history of a right knee replacement and a ruptured popliteal cyst who developed an erythematous indurated plaque over the surgical scar. Histopathology revealed fibrosis, chronic inflammation, and histiocytes within the lymphatics consistent with intralymphatic histiocytosis. The plaque flattened following intralesional injection of triamcinolone acetonide 10 mg/cc×1.6 cc once monthly for 2 consecutive months and application of a pressure bandage, with no recurrence after 4 months. This treatment may be useful for recalcitrant disease.

Intralymphatic histiocytosis is a benign condition characterized by poorly defined erythematous plaques (sometimes forming a reticular pattern) as well as the presence of nodules and vesicles. Its etiology and pathogenesis appear to be related to chronic inflammation in the affected area, prior surgery, or systemic disease, particularly rheumatoid arthritis. We report on 2 new cases, both associated with joint surgery in the affected area and osteoarticular disease (primary synovial osteochondromatosis and rheumatoid arthritis). This is a chronic disease and there is no specific treatment. Different treatment options were chosen in the 2 cases described. A spectacular response to treatment with oral pentoxifylline and topical tacrolimus was observed in 1 of the patients., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

Adult onset Still's disease (AOSD) is a chronic inflammatory disorder involving multiple systems. The symptoms mimic those of lymphomas, therefore, the diagnosis of lymphoma needs to be excluded prior to establishing the diagnosis of AOSD. Another similar condition is dermatopathic lymphadenopathy (DL). In DL, the histopathological appearance of lymph node biopsy may also mimic AOSD. The DL is associated with several systemic pathologies, such as malignant lymphomas, and rarely AOSD. We present a case of a 43-year-old male presented with 3 months history of fatigue, fever, and lymphadenopathy. Initial work-up satisfactorily met the criteria for diagnosis of AOSD. But considering the well-known association of DL with hematological malignancies, detailed pathological studies were considered, including tumor markers to rule out  the possibility of malignancy. The patient was started on steroids and showed remarkable recovery within 2 weeks. Evaluation of malignant lymphomas in a patient with DL is important, in order to diagnose AOSD and rule out hematological malignancy.

Background: Infectious mononucleosis is a clinical syndrome most commonly associated with primary Epstein-Barr virus (EBV) infection. In adults, the symptoms can often be severe and prolonged, sometimes causing serious complications. Analgesic or antipyretic drugs are normally used to relieve the symptoms. However, there is no causal treatment for the disease., Case Report: Two cases of adult patients with atopic predispositions developed nocturnal fever, general fatigue, pharyngitis and lymphadenopathy after an exacerbation of atopic symptoms or those of allergic rhinitis. Due to the positive results for EBV viral-capsid antigen (VCA) IgM and negative results for EBV nuclear antigen (EBNA) IgG, diagnoses of infectious mononucleosis induced by EBV were made in both cases. Although oral antibiotics or acetaminophen alone did not improve the deteriorating symptoms, including fever, headache and general fatigue, nonsteroidal anti-inflammatory drugs (NSAIDs), such as tiaramide or loxoprofen, completely improved the symptoms quickly after the initiation., Conclusions: In these cases, given the atopic predispositions of the patients, an enhanced immunological response was likely to be mainly responsible for the pathogenesis of the symptoms. In such cases, NSAIDs, that are known to reduce the activity of EBV, may dramatically improve the deteriorating symptoms quickly after the initiation. In the present cases, the immunosuppressive property of these drugs was considered to suppress the activity of lymphocytes and thus provide the rapid and persistent remission of the disease.

Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the central nervous system, distinguished by brainstem- and spinal cord-centered lesions with a characteristic contrast enhancement on MRI, a lymphocytic perivascular infiltrate on pathological exam, and a dramatic response to and dependence on steroids therapy. Since its initial description in 2010, different glucocorticoid-sparing agents, mostly immunosuppressant drugs, have been used to minimize the dosage, but these therapies also carry the risk of important secondary effects. We present the first reported case of CLIPPERS treated with interferon beta 1a as add-on therapy., Case Report: A previously healthy 31-year-old man presented with gait ataxia and dysarthria. MRI showed pons-centered hyperintense patchy lesions on T2-weighted images. Additional tests ruled out other possible diagnoses and symptoms reversed with intravenous methylprednisolone. Over the years the patient presented with several episodes of deterioration each year, which were partly reversed with glucocorticoid therapy, but leaving him with growing sequelae. Four years after the initial event, treatment with interferon-beta-1a was initiated, achieving reduced frequency of the relapses to 1 every 4 years, which were no longer associated to increasing disability. This allowed reducing glucocorticoids to 30 mg of Deflazacort every other day., Conclusions: Interferon beta-1a could be an alternative to corticosteroid-combined therapy in CLIPPERS and its more benign profile of secondary effects compared to immunosuppressants could make it an attractive choice.

The study of vascular anomalies is a rapidly progressing field in medicine. The development of new knowledge in the pathology and management of these disease processes are exemplified in the treatment of hemangiomas with propranolol and generalized lymphatic malformations with sirolimus. Central conducting lymphatic anomalies have traditionally been refractory to medical and surgical interventions. We report a case of a central conducting lymphatic anomaly that was responsive to sirolimus. A 14-year-old boy presented with chylothorax and chyluria with a lymphangiogram demonstrating abnormal lymphatic flow and reflux along the entire course of the central channels. Traditionally, medical management has been limited to somatostatin and low-fat diet with poor response and surgical interventions that are palliative. Sirolimus allows a new medical option that could improve management of this unresponsive population., (Copyright © 2016 by the American Academy of Pediatrics.)

To investigate clinical and radiological features of IgG4-related disease (IgG4-RD) patients with intrathoracic involvement. A prospective cohort study was performed and IgG4-RD patients were enrolled from January 2011 to March 2015 in Peking Union Medical College Hospital, in which the clinical and radiological characteristics of IgG4-RD patients with intrathoracic involvement were summarized. Out of total 248 cases with IgG4-RD, 87 cases had intrathoracic lesions, including 58 male cases and 29 female cases, with average age of 54.19 ± 13.80 years. Hilar and mediastinal lymphadenopathy were the most common manifestations of IgG4-related intrathoracic disease, accounting for 52.9% (46/87). Other imaging findings of pulmonary disease included: solid nodular (25.3%), round-shaped ground-glass opacities (9.2%), alveolar-interstitial type (20.7%), bronchovascular type (23.0%), pleural effusion (4.6%), and pleural nodules or thickening (16.1%). Only 27 patients presented with respiratory symptoms, including cough, breathless, chest pain, and asthma. Compared with patients without intrathoracic disease, IgG4-related intrathoracic disease had higher IgG4 and C-reactive protein level, and higher incidence of allergy, fever, and multi-organ involvement. Most of lung interstitial disease, mediastinal mass, and bronchial thickening were sensitive to corticosteroid and immunosuppressant therapy, while 36.3% (8/22) of solitary nodular lesions were unresponsive to treatment. Eight patients were on no treatment, with 5 cases remained stable, 2 patients improved spontaneously, and 1 patient was lost follow-up. Intrathoracic lesions are not rare in patients with IgG4-RD, involving bronchial thickening, nodules, ground glass opacity, pleural thickening/effusion, lymphadenopathy, etc. Efficacy of corticosteroid and immunosuppressant therapy were noted in most of patients with lung interstitial disease, mediastinal mass, and bronchial thickening., Competing Interests: The authors have no conflicts of interest to disclose.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. IgG4-RD tends to form tumefactive lesions. As a result, patients are often suspected of having a malignancy such as lymphoma. In this article, a patient with IgG4-RD and the deep vein thrombosis who was initially suspected of lymphoma is reported. The 63-year-old man presented with painless salivary swelling and multi-lymphadenopathy, progressively swelling and pain in the left leg. Salivary biopsy showed IgG4+ plasma cells >50 per high-power field and IgG4+/IgG+ plasma cell ratio >40 %. The serum IgG4 level was 4.28 g/L (range 0.03-2.01 mg/dL). Ultrasonography showed that the inferior vena cava was partially occluded, and thrombosis in the left iliac vein. Computed tomography scan revealed plaque-like tissue surrounding the inferior vena cava and abdominal aortic, which is typical for the diagnosis of retroperitoneal fibrosis. The patient was effectively treated with corticosteroids, interventional therapy, and anticoagulant therapy which resulted in a reduction in the swelling of the lymph nodes and left leg. Patient with IgG4-RD and deep vein thrombosis is rare and could be misdiagnosed easily as malignant disease. Accurate diagnosis is critical for disease management.

Prostatic adenocarcinoma (CaP) is the most common cancer in males and approximately 50% of patients have metastases at presentation, most commonly spreading to the bones and regional lymph nodes. CaP metastasising to inguinal lymph nodes in the absence of pelvic lymphadenopathy or other metastases is very uncommon. A 66-year-old man presented with isolated left inguinal lymphadenopathy of 3 months duration and a history of lower urinary tract symptoms for 1 year. He had prostatic cancer extending into the bladder base and seminal vesicles on contrast-enhanced CT of the pelvis, and asymptomatic vertebral secondaries on skeletal scintigraphy. Transrectal ultrasound biopsy revealed adenocarcinoma (Gleason score 3+4=7), and inguinal lymph node biopsy showed metastatic adenocarcinoma positive for prostate-specific antigen (PSA). As inguinal lymphadenopathy may also be due to other causes, biopsy is useful and meticulous clinical examination is a must in these patients., (2015 BMJ Publishing Group Ltd.)

Objectives: To present a clinicocytopathologic correlation of an atypical case of cat scratch disease (CSD) involving retroperitoneal lymph nodes, with emphasis on communication between service teams for managing lymphadenopathy of unknown origin. We consider clinical and cytologic differential diagnoses and review the literature on atypical cases of CSD, with emphasis on abdominal presentation and cytologic findings., Methods: Clinical services met with the cytology service to review clinical and pathologic features. Literature was reviewed via PubMed search (Harbor-UCLA subscriptions). Immunohistochemistry and Steiner silver stains were performed by Harbor-UCLA Department of Pathology. Enzyme-linked immunosorbent assay IgG and IgM Bartonella henselae titers were carried out by Quest Nichols Institute., Results: Fine-needle aspirate Diff-Quik and Papanicolaou smears and H&E-stained cell block showed abundant histiocytes, monocytoid B cells, and numerous neutrophils associated with necrosis corresponding to a late stage of CSD infection. Silver stain was positive for clumps of pleomorphic organisms. IgM and IgG antibody titers were elevated., Conclusions: The cytologic findings of CSD in an atypical abdominal presentation are similar to those of a classic presentation. Laboratory workup for atypical CSD should include at least two other modalities aside from cytomorphologic features. Close clinical and cytologic correlation avoided potentially unnecessary and harmful surgery and enabled timely treatment., (Copyright© by the American Society for Clinical Pathology.)

The Diffuse Infiltrative Lymphocytosis Syndrome (DILS) is a rare multisystemic syndrome described in HIV-infected patients. It is characterised by CD8(+) T-cell lymphocytosis associated with a CD8(+) T-cell infiltration of multiple organs. DILS is usually seen in uncontrolled or untreated HIV infection but can also manifest itself independently of CD4(+) T-cell counts. The syndrome may present as a Sjögren-like disease that generally associates sicca signs with bilateral parotiditis, lymphadenopathy, and extraglandular organ involvement. The latter may affect the lungs, nervous system, liver, kidneys, and digestive tract. Anomalies of the respiratory system are often identified as lymphocytic interstitial pneumonia. Facial nerve palsy, aseptic meningitis or polyneuropathy are among the more frequent neurological features. Hepatic lymphocytic infiltration, lymphocytic interstitial nephropathy and digestive tract lymphocytic infiltration account for more rarely noted complications. Sicca syndrome, organomegaly and/or organ dysfunction associated with polyclonal CD8(+) T-cell organ-infiltration are greatly suggestive of DILS in people living with HIV. Labial salivary gland biopsy is therefore helpful when the focus score is equal or greater than 1 (or Chisholm Score ≥ 3). Primary Sjögren syndrome, chronic HCV or HTLV1 infection, graft versus host disease, IgG4-related disease, and immune reconstitution inflammatory syndrome are among the differential diagnoses that need to be considered. Treatment consists in highly active anti-retroviral therapy (HAART), which is usually effective in resolving clinical signs and symptoms. Steroids, however, may also be occasionally required when organ infiltration does not respond to HAART. This review should provide an insight into this rare entity complicating the course of HIV infection., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Background: Sinus histiocytosis (Rosai-Dorfman disease) with massive lymphadenopathy is a rare nonneoplastic and nonlangerhans cell proliferation disorder of the histiocytes. Extranodal location with or without lymphadenopathy occurs in about 40 % of the cases. Intracranial location is rare in children often mimicking meningiomas. The parasphenoidal region is more frequently involved though intraxial or intraventricular locations were described as well. Rarely, the surgical treatment allows the complete excision of the lesion; however, in symptomatic cases, partial resections of the tumor allow to counteract its mass effect. Long survivals are possible, even without radiotherapy or chemotherapy, due to the frequent spontaneous benign evolution of the lesions., Case Report: A 2-year-10-month-old girl presented with high fever and vomiting. One year ago, she had a period of muscular weakness in both legs that recovered completely. MRI of the brain revealed an axial enhancing lesion with ventricular spreading mainly to the left occipital horn and bilateral frontal periventricular infiltration. After steroid therapy, all the symptoms recovered. Partial removal of the occipital intraventricular lesion was performed and the diagnosis of Rosai-Dorfman disease was established and confirmed by the reference center. At the latest follow-up (16 months), the girl is without any neurological symptoms and without any treatment.

Roles of all-trans-retinoic acid (tRA), a metabolite of vitamin A (VA), in both tolerogenic and immunogenic responses are documented. However, how tRA affects the development of systemic autoimmunity is poorly understood. Here we demonstrate that tRA have paradoxical effects on the development of autoimmune lupus in the MRL/lpr mouse model. We administered, orally, tRA or VA mixed with 10% of tRA (referred to as VARA) to female mice starting from 6 weeks of age. At this age, the mice do not exhibit overt clinical signs of lupus. However, the immunogenic environment preceding disease onset has been established as evidenced by an increase of total IgM/IgG in the plasma and expansion of lymphocytes and dendritic cells in secondary lymphoid organs. After 8 weeks of tRA, but not VARA treatment, significantly higher pathological scores in the skin, brain and lung were observed. These were accompanied by a marked increase in B-cell responses that included autoantibody production and enhanced expression of plasma cell-promoting cytokines. Paradoxically, the number of lymphocytes in the mesenteric lymph node decreased with tRA that led to significantly reduced lymphadenopathy. In addition, tRA differentially affected renal pathology, increasing leukocyte infiltration of renal tubulointerstitium while restoring the size of glomeruli in the kidney cortex. In contrast, minimal induction of inflammation with tRA in the absence of an immunogenic environment in the control mice was observed. Altogether, our results suggest that under a predisposed immunogenic environment in autoimmune lupus, tRA may decrease inflammation in some organs while generating more severe disease in others.

Since 2009, the largest reported outbreak of leishmaniasis by Leishmania infantum in Europe was reported in Fuenlabrada, Spain. In our hospital, 90 adults with localized leishmanial lymphadenopathy (LLL) or visceral leishmaniasis (VL) were treated during this outbreak; 72% were men, and the mean age was 46.2 years (range 15-95 years). A total of 17 cases (19%) were LLL, an atypical form with isolated lymphadenopathies without other symptoms. All LLL cases occurred in immunocompetent subjects, and only one subject (6%) was a native of sub-Saharan Africa. Diagnosis was performed by fine needle aspiration cytology of the lymphadenopathy. Serology was negative in 38%. LLL outcomes at 6 months were benign, even with doses of liposomal amphotericin B that were often lower (10 mg/kg) than those recommended for VL in Mediterranean areas. A total of 73 subjects (81%) presented with typical VL; 66% of this group were immunocompetent, and 50% of those who were immunocompetent were descendants of natives of sub-Saharan Africa. The rK39 test and polymerase chain reaction were the most useful tests for confirmation of the diagnosis. An initial response to treatment was observed in 99% of cases, and relapses occurred in 14% of cases. Leishmaniasis should be included in the differential diagnosis of isolated lymphadenopathies in endemic areas. LLL could be considered a more benign entity, one different than VL, and less aggressive management should be studied in future investigations., (Copyright © 2014 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

IgG4-related disease is a newly recognized entity associated with autoimmune conditions involving almost every organ system. It is characterized by elevated serum IgG4 as well as mass like tissue infiltration by IgG4-positive plasma cells. Imaging findings are nonspecific, vary depending on the site of disease, and include mass like enlargement of the salivary or lacrimal glands and enlarged lymph nodes. Radiographic findings often mimic malignancy, necessitating tissue sampling to confirm the diagnosis. Distinguishing IgG4-related disease from malignancy is important as IgG4 responds well to steroids and conservative management.

Background: Benign lymphoepithelial lesion (BLEL) is characterized by symmetric bilateral swelling of the lacrimal and salivary glands and considered a subtype of immunoglobulin G4 (IgG4)-related sclerosing disease, the etiology and pathogenesis of which has not been determined. The purpose of the present study was to analyze the clinical features of BLEL in the lacrimal gland and the relationship between the serum level of IgG4 and BLEL., Methods: Twenty consecutive patients with BLEL diagnosed in Department of Ophthalmology at Beijing Tongren Hospital, Capital Medical University between January 2012 and December 2013 were observed. The clinical features, imaging findings, laboratory tests, treatments and follow-up status of these 20 consecutive patients were analyzed., Results: In all 20 patients, the ratio of male to female was 1:4, the ages ranged from 28 to 57 years, the ratio of unilateral to bilateral eyes involvement was 1:4, and painless uncongested symmetrical swelling of the upper eyelid was the main clinical manifestation. Orbital magnetic resonance imaging (MRI) showed that all patients involved lacrimal gland, which were obviously enlarged with equal signals in T1W images and T2W images and obvious enhancement on contrast MRI. Extraocular muscles were involved in 5 patients, salivary gland in 8 patients, and frontal nerve in 3 patients. Serum IgG4 concentration was elevated in 18 patients. The treatment strategy mainly included surgery and steroid administration. Three patients were lost to follow-up, 17 patients reached complete response, and no recurrence was observed., Conclusions: Eyelid swelling is the typical symptom of BLEL. Most of the patients involved bilateral lacrimal glands. High serum IgG4 level and abundant IgG4-positive plasma cell infiltration are the important features, which can be found in most of BLEL patients. Surgery combined with glucocorticoids is an efficient treatment strategy.

Background: Kimura's disease (KD) is a slowly progressing rare, benign inflammatory disorder of the soft tissues. It typically presents as subcutaneous tumor-like nodules, located most frequently in the head and neck region. KD is often accompanied by increased peripheral eosinophilia and elevated levels of serum immunoglobulin (Ig) E. There is renal involvement in approximately 12-16% of KD cases. We report the case of a 23-year-old Chinese man who was found to have KD associated with nephrotic syndrome., Case Presentation: A 23-year-old Chinese man presented with edema in both legs and a mass in ulnar side of his right upper arm on August 8(th) 2013. Before admission to our hospital, an ultrasound examination revealed swollen lymph nodes in the medial aspect of his right upper arm, proximal to the elbow. The patient was admitted on August 19(th) 2013 as a result of edema, severe proteinuria, and low serum albumin levels. He had a white blood cell count of 7.7 × 10(9) cells/L, 48.5% eosinophils, 4+ albuminuria, 24-hour urinary protein excretion 9.3 g, serum protein 50.3 g/L; serum albumin 16 g/L and IgE 1,510 IU/ml. A biopsy of the epitrochlear nodes revealed eosinophilic hyperplastic lymphogranulomatous tissue. A renal biopsy indicated focal segmental glomerulosclerosis (FSGS) (cellular variant) with no infiltration of eosinophil in renal interstitium. The results of immune-staining on the renal biopsy were negative for IgG, IgA, IgM, C3 and C1q. The electron microscopic analysis showed podocyte effacement. His final diagnosis was Kimura's disease associated with nephrotic syndrome. He received methylprednisolone therapy as well as symptomatic treatment, and was discharged with key indicators in normal range on September 17(th) 2013. During the year following, he had methylprednisolone at a maintenance dose of 8 mg/day, and no relapses occurred up to now., Conclusion: Methylprednisolone therapy is effective in KD associated with nephrotic syndrome, and long-term administration of methylprednisolone at maintenance dose may be a way to prevent relapses of KD.

The differential diagnosis in pediatric lymphadenopathy includes bacterial, viral, fungal, and idiopathic causes. A systematic approach to patient evaluation is necessary because the differential diagnosis, presentation, and work up must consider infectious, immunologic, neoplastic, and idiopathic disorders. A thorough history and examination are vital to determining the diagnosis and ruling out a malignant process., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Löfgren's syndrome is an acute clinical form of sarcoidosis that is first described by Sven Löfgren as bilateral hilar lymphadenopathy together with Erythema Nodosum (EN) and accompanying arthritis/arthralgia. This syndrome has some differences in terms of treatment, prognosis and recurrence compared to sarcoidosis. In this report, we describe a 70 years old lady who admitted with multiple erythematous lesions and arthralgia of both lower extremities and she was diagnosed as Löfgren's syndrome via mediastinoscopy.

Introduction: Sinus histiocytosis with massive lymphadenopathy is a rare disease. Its location is ubiquitous, but the most frequent is cervical lymph nodes., Observation: We report 2 cases of sinus histiocytosis with massive lymphadenopathy. The patients were respectively 9 and 30 years of age. They both presented with bilateral cervical polylymphadenopathy with a cervical compression syndrome in the first patient. The diagnosis was made histologically by demonstrating histiocyte emperipolesis, in the first case, and by immuno-histochemistry in the second. The treatment was corticosteroids, resulting in an almost total involution., Discussion: Sinus histiocytosis with massive lymphadenopathy is a clinical and a specific pathology, but its etiopathogenesis remains to be elucidated. The cervical localization is common. The definitive diagnosis is histological. There is currently no codified treatment. The outcome is usually favorable, apart from mechanical and systemic complications that are important prognostic factors., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

A 46-year-old woman presented with right upper abdominal pain and fever. At imaging, enlarged peripancreatic and hilar lymph nodes, as well as hypodense liver lesions, were detected, suggestive of malignant disease. At endoscopy, the mass adjacent to the duodenum was seen as a protruding lesion through the duodenal wall. A biopsy of this lesion, taken through the duodenal wall, showed a histiocytic granulomatous inflammation with necrosis. Serology for Bartonella henselae IgM was highly elevated a few weeks after presentation, consistent with the diagnosis of cat scratch disease. Clinical symptoms subsided spontaneously and, after treatment with azithromycin, the lymphatic masses, liver lesions and duodenal ulceration disappeared completely., (2014 BMJ Publishing Group Ltd.)

Introduction: Tularemia is a zoonotic disease caused by Francisella tularensis. The oculoglandular form is one of the rarest forms. In this study, evaluated tularemia patients, focusing on the ocular form and the efficacy of early antibiotic therapy., Methodology: During a tularemia outbreak, the epidemiological and clinical findings, laboratory assays, and drugs used for the treatment of 48 patients were recorded prospectively. The diagnosis of tularemia was confirmed with microagglutination test (MAT) as well as clinical findings., Results: The mean age of the subject was 48.6 years; 23 (47.9%) of them were female. Thirty-six (81.25%) patients had clinical presentation compatible with oropharyngeal tularemia, seven (14.58%) with oculoglandular tularemia, and two (4.1%) with ulceroglandular tularemia. The most common symptoms were fever (91.6%) and sore throat (81.2%), and the most common findings were lymphadenopathy (91.6%) and tonsillopharyngitis (81.2%). In the oculoglandular form, fever, lymphadenopathy, periorbital edema, conjunctival injection, and chemosis were found. The most distinctive ophthalmic feature was follicular conjunctivitis and conjunctival epithelial defects. Forty-five cases had positive serological results with MAT. All the patients were treated with antibiotics considered effective against F. tularensis, and topical antimicrobial treatment was given to the patients with oculoglandular tularemia. Twenty-six (54.16%) patients, who were admitted within three weeks of the onset of symptoms, recovered without sequel., Conclusions: During tularemia outbreaks, ocular involvement should be considered carefully. The early administration of appropriate treatment will be more effective in resolving the infection and preventing complications. Along with systemic antibiotic therapy, topical treatment will help recovery.

A role for CD20 antibodies in treating prostate cancer has not yet been established. We report a case of advanced prostate cancer presenting with generalized lymphadenopathy that expressed CCR7 and CD20. CCR7 expression in prostate cancer has been previously reported only once; the expression of CD20 has not been reported before. Rituximab therapy was initiated in this case and resulted in a significant biochemical response. This unique metastatic and biochemical pattern may signify a distinct subtype of prostate cancer that may be amenable to treatment with anti-CD20 antibodies.

Background and Objective: Löfgren's syndrome is characterized by hiliar adenopathies, erythema nodosum and arthritis. It is a benign variant of sarcoidosis, common in the Mediterranean area. To describe the clinical characteristics, treatment and outcome of a series of patients diagnosed with Löfgren's syndrome., Patients and Methods: Retrospective design (1984-2013)., Setting: Two university hospitals with a reference population of 1,015,000 inhabitants., Results: Eighty patients were diagnosed: 29 men and 51 women (mean age 42.3 years). Forty eight patients (60%) presented with the classical triad: hiliar adenopathies, erythema nodosum and arthritis; 18 (22%) with hiliar adenopathy and arthritis; 13 (16%) hiliar adenopathies and erythema nodosum. All showed abnormalities in the chest study. According to the radiological pattern, patients were classified in stage i-ii. Biopsy was performed in 39 patients and was diagnostic in 28. Treatment was based on non-steroidal anti-inflammatory drugs (54 patients, 67%) and corticosteroids (33 patients, 41%). Fourteen patients (17%) suffered a recurrence of the disease., Conclusions: Löfgren's syndrome is a benign form of sarcoidosis with a well defined clinical pattern. Biopsy is usually not required. Recurrence is scarce. The disease has a good prognosis., (Copyright © 2013 Elsevier España, S.L. All rights reserved.)

Bartonella spp. are responsible for emerging and re-emerging diseases around the world. The majority of human infections are caused by Bartonella henselae, Bartonella quintana and Bartonella bacilliformis, although other Bartonella spp. have also been associated with clinical manifestations in humans. The severity of Bartonella infection correlates with the patient's immune status. Clinical manifestations can range from benign and self-limited to severe and life-threatening disease. Clinical conditions associated with Bartonella spp. include local lymphadenopathy, bacteraemia, endocarditis, and tissue colonisation resulting in bacillary angiomatosis and peliosis hepatis. Without treatment, Bartonella infection can cause high mortality. To date, no single treatment is effective for all Bartonella-associated diseases. In the absence of systematic reviews, treatment decisions for Bartonella infections are based on case reports that test a limited number of patients. Antibiotics do not significantly affect the cure rate in patients with Bartonella lymphadenopathy. Patients with Bartonella spp. bacteraemia should be treated with gentamicin and doxycycline, but chloramphenicol has been proposed for the treatment of B. bacilliformis bacteraemia. Gentamicin in combination with doxycycline is considered the best treatment regimen for endocarditis, and erythromycin is the first-line antibiotic therapy for the treatment of angioproliferative lesions. Rifampicin or streptomycin can be used to treat verruga peruana. In this review, we present recent data and recommendations related to the treatment of Bartonella infections based on the pathogenicity of Bartonella spp., (Copyright © 2014 Elsevier B.V. and the International Society of Chemotherapy. All rights reserved.)

Background: TAFRO syndrome is a unique clinicopathologic variant of multicentric Castleman's disease that has recently been identified in Japan. It is characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, reticulin Fibrosis of the bone marrow, Renal dysfunction and Organomegaly (TAFRO). Previous reports have shown that affected patients usually respond to immunosuppressive therapy, but the disease sometimes has a fatal course. TAFRO syndrome occurs in the middle-aged and elderly and there are no prior reports of the disease in adolescents. Here we report the first adolescent case, successfully treated with anti-IL-6 receptor antibody (tocilizumab, TCZ) and monitored with serial cytokine profiles., Case Presentation: A 15-year-old Japanese boy was referred to us with fever of unknown origin. Whole body computed tomography demonstrated systemic lymphadenopathy, organomegaly and anasarca. Laboratory tests showed elevated C-reactive protein and hypoproteinemia. Bone marrow biopsy revealed a hyperplastic marrow with megakaryocytic hyperplasia and mild reticulin fibrosis. Despite methylprednisolone pulse therapy, the disease progressed markedly to respiratory distress, acute renal failure, anemia and thrombocytopenia. Serum and plasma levels of cytokines, including IL-6, vascular endothelial growth factor, neopterin and soluble tumor necrosis factor receptors I and II, were markedly elevated. Repeated weekly TCZ administration dramatically improved the patient's symptoms and laboratory tests showed decreasing cytokine levels., Conclusion: To our knowledge, this is the first report of TAFRO syndrome in a young patient, suggesting that this disease can occur even in adolescence. The patient was successfully treated with TCZ. During our patient's clinical course, monitoring cytokine profiles was useful to assess the disease activity of TAFRO syndrome.

A 28-year-old man presented with loss of appetite, night sweats, eczema, and axillary and inguinal lymph node swelling. The tentative diagnosis of malignant lymphoma was made. To confirm the diagnosis, extirpation of a lymph node and a skin biopsy were performed. Systemic treatment with methylprednisolone resulted in an improvement of eczema and lymph node swelling. Because of the histological findings and clinical course, we diagnosed dermatopathic lymphadenopathy, also known as Pautrier-Woringer syndrome.