Kikuchi’s disease involving the supraclavicular lymph nodes and associated with transient eruption

A 26-year-old woman presented with high fever, marked supraclavicular lymphadenopathy, and morbilliform eruptions and was diagnosed with Kikuchis disease (KD) based on pathologic findings from biopsied lymph nodes. All her manifestations of KD improved, however, without any specific treatment. The picture of transient morbilliform eruptions typified in KD here is seldom shown in the literature. In general, KD would run a benign course of supraclavicular lymphadenopathy.     

Lymphocytic interstitial pneumonia and non-caseating epithelioid granuloma in mediastinal lymph nodes in a patient with primary Sjögren’s syndrome

A 63-year-old woman diagnosed with Sjögren’s syndrome (SS) was indicated as having interstitial pneumonia with enlarged mediastinal lymph nodes. Lung tissue specimens showed lymphocytic interstitial pneumonia (LIP) and non-caseating epithelioid granulomas were recognized in only the mediastinal lymph nodes. Monoclonality was not detected using polymerase chain reaction of IgH rearrangement. The pulmonary lesions including lymphadenopathy were remarkably reduced by admistration of prednisolone. In this case, the sarcoid-like granulomas may have been associated with LIP, rather than co-existence of SS with sarcoidosis.     

Takayasu’s arteritis misdiagnosed as mediastinal malignant lymphoma: a case report and review of the literature

Takayasu’s arteritis (TA) is a rare chronic large-vessel vasculitis. The early diagnosis is difficult, because of lack of characteristic clinical manifestations. In this paper, we reported a TA case of young female was misdiagnosed as mediastinal malignant lymphoma and mediastinoscope biopsy was performed. The biopsy result demonstrated that thickened tissue adjacent to the aortic arch pathological presentations were in accord with TA. Glucocorticoid was administrated and the condition was greatly improved after treatment. Therefore, we reported this case and review of the pertinent literature in order to help clinicians improve the understanding of TA and PET/CT manifestations of TA at early phase to realize the early diagnosis and treatment of TA, finally reducing the hazards.     

D-Penicillamine improved laparoscopic and histological findings of the liver in a patient with Wilson’s disease: 3-year follow-up after diagnosis of Coombs-negative hemolytic anemia of Wilson’s disease

We report a 13-year-old girl who presented with hepatic failure and hemolytic anemia. Laboratory findings showed a normal serum copper level (104 µg/dl), high urinary copper level (2370 µg/dl), and low serum ceruloplasmin level (14.3 µg/dl). Slit-lamp examination revealed Kayser-Fleischer rings on her cornea, and she was diagnosed with Wilson’s disease. Plasma exchange and continuous hemodiafiltration relieved the serious condition, after that laparoscopic examination was performed. Administration of D-penicillamine and restriction of dietary copper (<1 mg/day) were started, leading to a normalized serum alanine amino transferase (ALT) level. After 3 years, she again underwent laparoscopic examination, and the laparoscopic and histological findings of her liver were obviously improved. Management of the copper level can reverse severe liver fibrosis in Wilson’s disease.     

Effectiveness and safety of abatacept for the treatment of patients with primary Sjögren’s syndrome

Clinical Rheumatology - Sjögren’s syndrome is an autoimmune disease characterized by inflammation of the exocrine glands. The disease can be primary or secondary (if it is associated...     

Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation?

New options are available for the medical treatment of patients with Wilson’s disease. Penicillamine is no longer the treatment of choice, as there is a growing experience with safer and more effective alternatives. Trientine may be the best choice for initial therapy in symptomatic patients requiring chelation therapy, and it may be even more effective when used in combination with zinc, which is recommended for maintenance therapy. Further studies are needed to determine the best therapy for pregnant patients with Wilson’s disease, and whether combination therapy using trientine and zinc will be the next treatment of choice for all symptomatic patients with liver or neurologic disease.     

Collision lesions of the sella: co-existence of craniopharyngioma with gonadotroph adenoma and of Rathke’s cleft cyst with corticotroph adenoma

Collision lesions of the sellar region are relatively uncommon. Most contributions include a pituitary adenoma or a cyst/cystic tumor, particularly a Rathke cleft cyst. The association of craniopharyngioma with an adenoma is particularly rare. Among reported cases, some have included secondary prolactin cell hyperplasia due to pituitary stalk section effect. Herein, we report two collision lesions, including a gonadotroph adenoma with adamantinomatous craniopharyngioma and a corticotroph adenoma with Rathke's cleft cyst. Clinicopathologic correlation and a review of the literature are undertaken.     

Addison’s disease with pituitary hyperplasia: a case report and review of the literature

This case study describes a 33-year-old man with Addison’s disease who presented with increased plasma adrenocorticotropic hormone (ACTH), normal plasma cortisol, and absent diurnal rhythms. Magnetic resonance imaging (MRI) indicated pituitary hyperplasia. Conventional hydrocortisone replacement therapy may not inhibit high ACTH levels in the morning; however, replacing hydrocortisone with dexamethasone achieved good therapeutic results.     

Asthma combined with Hodgkin’s lymphoma: a case report and review of the literature

Both asthma and hematological malignancies are influenced by genetic and environmental factors. The previous studies have revealed lymphoma were found among atopic men and women. However, the relationship between asthma and Hodgkin’s lymphoma has not been well recognized. We present here, a case of a 53-year-old male patient suffered asthma and then Hodgkin’s lymphoma.KEYWORDS : Aasthma, Hodgkin’s lymphoma, Th2 lymphocyte     

Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature

Kikuchi-Fujimotos disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.     

Pituicytoma in a patient with Cushing’s disease: case report and review of the literature

There are scant prospective studies defining improvements in critical outcome measures with hormone replacement in hypopituitarism secondary to brain injury. We review the tests of cognition and physical function and summarize their use for subjects that are deficient in anterior hormone production during anterior pituitary hormone replacement in brain injury and propose these as the minimal tests that are feasible for a physician to perform in a clinical setting. We summarize the studies conducted to assess outcome measures after brain injury and also report preliminary findings for improvements in cognition and physical function in subjects with brain injury and GH deficiency.     

A tale of two sisters with hypertrophic cardiomyopathy and recurrent embolism: When is the optimal timing of the intervention for left atrial appendage?

Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous genetic disease that affects the left ventricle (LV) and has a varied clinical course and phenotypic expression. Here, we report a case of two sisters with HCM who developed a massive refractory left atrial appendage (LAA) thrombus and recurrent embolism. The older sister, who was at a high surgical risk due to progressive LV systolic dysfunction with an ejection fraction of 19%, underwent LAA plication in combination with implantation of an LV assist device after progression to treatment-refractory heart failure at the age of 49. The younger sister underwent surgical thrombectomy, LAA plication, and Maze surgery before deterioration of heart failure at the age of 47. She was free from embolism and atrial fibrillation for 2years after surgery. Individualized therapeutic approaches targeting the LAA at a relatively early stage are required in the subgroups of HCM patients with left atrial dysfunction.     

Treatment of refractory adult-onset still’s disease with tocilizumab: report of two cases and review of the literature

Adult-onset Still’s disease (AOSD) is empirically treated with nonsteroidal anti-inflammatory drugs, corticosteroids, conventional disease-modifying antirheumatic drugs, tumor necrosis factor–blocking agents or anakinra. The monoclonal anti-interleukin (IL)-6 antibody tocilicumab (TOC) has recently been approved for the treatment of rheumatoid arthritis and may be an attractive therapeutic option for AOSD as well. We report two AOSD patients treated with TOC and review of the current data on the use of TOC in AOSD. TOC was applied to the first patient after failure of cloroquin, methotrexate, adalimumab and etanercept. The second patient received TOC because of inefficacious methotrexate treatment. TOC was well tolerated by both the patients, and no clinically significant side effects occurred. Including these two cases, a total of seven AOSD patients have been successfully treated with TOC so far. TOC may be a promising treatment option for AOSD patients refractory to conventional disease-modifying antirheumatic drugs anakinra and tumor necrosis factor- _boxclose\tilde{\alpha }.     

Natalizumab: pharmacology,clinical efficacy and safety in the treatment of patients with Crohn’s disease

Natalizumab is a humanized monoclonal antibody against α4 integrin. In preclinical and clinical studies, natalizumab, which interferes with leukocyte trafficking in the intestinal tract, demonstrated effectiveness in inducing clinical response and maintaining remission in patients with moderate-to-severely active Crohn’s disease. However, during clinical trials, three natalizumab-treated patients (one Crohn’s disease patient and two multiple sclerosis patients) developed progressive multifocal leukoencephalopathy (PML). As a consequence of this unexpected serious adverse event, a retrospective safety evaluation was conducted; in that safety evaluation, no new cases of PML were identified. Natalizumab returned to the market in June 2006 for the treatment of relapsing multiple sclerosis. As of May 2007, an estimated 12,000 patients worldwide had received natalizumab, with no new confirmed cases of PML or opportunistic infections reported. Natalizumab is currently being investigated for use in treating patients with Crohn’s disease. If it is approved for treatment of Crohn’s patients, the clinical benefit of natalizumab should be weighed carefully against the potential risk of serious adverse events.