An unusual association between splenectomy and Kikuchi's disease

Kikuchi's disease is a clinico-pathologic entity of unknown etiology characterized by subacute inflammatory process of lymph nodes. It affects mostly women around the age of 30 years. It is usually a self limiting illness characterized by pyrexia, neutropenia, and cervical lymphadenopathy. We report a case of Kikuchi's disease in a patient with past history of splenectomy. A 35-year-old otherwise healthy female patient presented with 15 days history of fever, night sweats, and right cervical lymphadenopathy. She was on no medication and had no contact with animals or patients with tuberculosis. Her past history revealed splenectomy for thrombocytopenia 14 years before presentation. Lymphoma was suspected and she was referred for a cervical lymph node biopsy. The final histopathology diagnosis revealed subacute necrotizing lymphadenitis consistent with Kikuchi's disease. This is the first case of Kikuchi's disease presenting in a post splenectomy patient.     

Systemic lupus erythematosus in children: a case report and review

A 12-year-old Papua New Guinean female presented initially with nonspecific clinical symptoms, fever of unknown origin and anaemia. She subsequently developed multisystem disease involving the respiratory, gastrointestinal, central nervous, musculoskeletal and cutaneous systems. She was diagnosed to have systemic lupus erythromatosus (SLE) and started on treatment. Unfortunately the patient defaulted from follow-up after treatment, which covered seven months only, to present with acute respiratory distress from which she died within 24 hours. A relevant literature review with the clinical features of systemic lupus erythematosus in children is described.     

Parkinsonian syndrome as a complication of systemic lupus erythematosus. Report of a case and review of the literature

A 34-year-old woman has had systemic lupus erythematosus since 11 years, with multiple treatments and incomplete result. When examined in our department, she presented with a recent parkinsonian syndrome including bradykinesia, adiadochokinesia, cogwheel rigidity and postural tremor of hands. Deep tendon reflexes were increased. Brain MRI showed abnormal signal intensity in the periventricular white matter, internal capsule, brain stem, middle cerebellar peduncles and cerebellar hemispheres. She was treated with prednisone and mycophenolate for the lupus, and amantadine and benzhexol for the parkinsonian syndrome which resolved within ten months, while the lupus improved. There are 30 cases of Parkinson's disease as a complication of lupus reported in the literature. The pathogenesis may be cerebral vasculitis with ischemia, and antibodies against neurons, mainly dopaminergic. Twenty cases improved, eight of them with the treatment of the lupus alone.     

Systemic lupus erythematosus and primary cerebral lymphoma

A woman with a 21 year old history of systemic lupus erythematosus (SLE) is presented. During a period of low connective tissue disease activity she developed sudden difficulty in walking and after much investigation was found to have a primary lymphoma of the brain. She died 6 months after this diagnosis from meningeal spread, despite radiotherapy to the tumour. The association between SLE and lymphoma is discussed.     

Kikuchi's disease associated with hemophagocytosis

We report the case of a 40 year-old Nepali man admitted to the hospital with a one month history of fever associated with swelling, pain, and redness on the right side of the neck. On examination, tender lymph nodes were palpated in the right posterior cervical triangle. Bone marrow aspiration and biopsy showed hemophagocytosis. Cervical lymph node biopsy showed the typical necrotizing lymphadenitis of Kikuchi's disease. The patient was given non-steroidal anti-inflammatory drugs (Naproxen 500 mg twice daily orally). After ten days, the fever and lymphadenopathy subsided and he was consequently discharged.     

Hodgkin's lymphoma with exuberant granulomatous reaction

We report a 36-year-old woman, presented with cervical lymphadenopathy and low-grade fever. Two fine needle aspiration cytology and one excisional biopsy were performed in the referral hospital, all showed granulomatous lesions without necrosis. A tentative diagnosis of tuberculosis was made, and she started on antituberculous treatment. However, there was no clinical improvement. She presented to our institution one year after the initial diagnosis, and a new biopsy from the cervical lymph node revealed effacement of the whole node by marked non-necrotizing granulomatous reaction. However, there were scattered large cells with few classic Reed-Sternberg cells between the granulomas. Immunohistochemistry reveals strong reaction of CD15 and CD30, and negative staining for CD45RB, CD45RO, and CD20. These findings confirmed the diagnosis of Hodgkin's lymphoma with remarkable granulomatous reaction that almost masked the malignant component. She was treated with chemotherapy, and she showed an excellent response.     

Disseminated histoplasmosis in a non-immunocompromised child

We describe a 2 year-old non-immunocompromised girl with disseminated histoplasmosis who presented with a 2-month history of fever and bloody diarrhoea. On presentation, she was severely wasted and anaemic. There were gross hepatosplenomegaly and multiple lymphadenopathy. A septic screen was negative. A subsequent stool culture isolated Salmonella enteriditis. Serial Widal-Weil Felix (WWF) titres showed serological response after 2 weeks of Ceftriaxone. However, she continued to have spiking fever, bloody diarrhoea and weight loss. She developed pancytopaenia and disseminated intravascular coagulation. A bone marrow aspirate and trephine, and lymph node biopsy showed the presence of Histoplasma capsulatum, confirmed by Gomori-Methenamine Silver staining. She responded to intravenous amphotericin B followed by fluconazole (intravenous then oral) for 6 months after discharge. Human Immunodeficiency Virus screening tests were negative. Complement and immunoglobulin levels were normal. T and B enumeration tests showed gross leucopaenia with very low T cell function with defective phagocytic function. A repeat T and B cell enumeration test and phagocytic function tests done 3 months later were normal.     

系统性红斑狼疮合并可逆性后部脑病综合征1例

This case report concerns a 22-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE). She had intermittent fever, butterfly erythema, photosensitivity, oral ulcers, and multiple arthralgia in the past seven years, but she did not adhere to regular treatments. The edema of the lower extremities and face aggravated in the recent two weeks, so she was admitted to our Department of Rheumatology and Clinical Immunology. Meanwhile, we found she had severe hypertension, the maximal blood pressure was 170/120 mmHg. The patient had high SLE disease activity (the disease activity index score was as high as 23) with blood involvement, acute renal insufficiency, multiple serous effusion and rash. After one week treatments of intravenous methylprednisolone 80 mg daily and other drugs, her conditions made some extent improvement. However, she suffered sudden epileptic attacks. No positive neuro-pathological signs were found, and the blood pressure was up to 190/130 mmHg before the onset of the seizures. Her cerebrospinal fluid (CSF) pressure was 330 mmH₂O, the CSF protein level was normal value, and the white blood cell count was 0 cell/mm ³, with no signs of infection. Cranial MRI showed vasogenic edema at bilateral parietal, occipito-parietal regions, and centrum ovale. We prescribed drugs of decreasing intracranial pressure, intravenous drugs of decreasing blood pressure and midazolam for sedation, without corticosteroid impulse therapy. She recovered consciousness in the next day, without epilepsy recurrence. We eventually diagnosed it as posterior reversible encephalopathy syndrome (PRES), according to the history, laboratory results, imaging featuresand clinical outcome. PRES is a disorder of reversible subcortical vasogenic brain edema in patients with acute neurological symptoms (eg, seizures, encephalopathy, headache, and visual disturbances).PRES is mainly caused by blood pressure changes or endothelial injury, which lead to breakdown of the blood-brain barrier and subsequent brain edema. Most patients have a favourable prognosis.SLE complicated with PRES is not rare, especially in patients with disease activity, hypertension, lupus nephritis and/or renal insufficiency, and use of cytotoxic drugs, early recognition and appropriate treatment remain important. Brainstem involvement, intracranial hemorrhage, renal insufficiency and high disease activity of lupus are risk factors for poor prognosis.     

An unusual case of pyrexia of unknown origin with cervical lymphadenopathy

Kikuchi's disease is usually a self limiting illness characterised by pyrexia, neutropenia, and cervical lymphadenopathy particularly in young women of Asian descent. This often leads to an initial misdiagnosis of lymphoma. A case of a young Asian woman who presented with pyrexia of unknown origin is described.     

Acute acalculous cholecystitis complicating systemic lupus erythematosus: case report and review

A case of acalculous cholecystitis presented as an acute abdominal emergency in a 22 year old woman with severe systemic lupus erythematosus. At the time of presentation the patient was receiving high doses of prednisone and cyclophosphamide to control her underlying disease. Histological examination of the biopsy specimen from the gall bladder showed lupus vasculitis. This complication of systemic lupus erythematosus has not been reported before. Laboratory studies and changes in lupus activity may fail to predict the onset of cholecystitis.     

Recurrent multiple myeloma presenting as a breast plasmacytoma

We describe a patient with multiple myeloma, who initially responded to chemotherapy and went into remission. She presented 10 months later with a right breast lump which was confirmed by core biopsy to be a plasmacytoma. Further treatment with radiotherapy, thalidomide and later second line chemotherapy appeared unsuccessful and she showed rapid disease progression with rising paraproteins and new extramedullary plasmacytoma lesions in the forehead, supraclavicular region, nasopharynx, liver, spleen, pancreas and paraaortic lymph nodes.     

Melioidosis: Missed opportunities and opportunistic pathogens

A 31-year Indian homemaker, known to have Systemic Lupus Erythematosus (SLE) and lupus nephritis, was admitted previously in another medical care unit with fever, hemoptysis, arthralgia, and joint swelling. She had been treated with antibiotics and corticosteroids for probable diffuse alveolar hemorrhage (DAH) with clinical and radiological resolution. She was readmitted one month later for similar complaints. Her autoimmune workup revealed evidence of active lupus. Her chest imaging showed the presence of well-circumscribed macronodular lesions with halo sign, but Bronchoalveolar Lavage (BAL) cultures and serum galactomannan were negative. BAL tested positive for hemosiderin-laden macrophages. She was treated with corticosteroids, plasmapheresis, and empiric antibiotics with partial clinical response. One week later, her fever recurred, and she developed new-onset myositis. Bactec blood cultures grew Burkholderia pseudomallei. She received treatment for 3 months with good clinical and radiological resolution. In hindsight, a CT-guided biopsy of the lung lesion may have provided an earlier diagnosis of melioidosis.     

系统性红斑狼疮合并皮肤脂膜炎多发溃疡1例

SUMMARY A 36-year-old woman had an 8-year history of systemic lupus erythematosus (SLE) and was being treated with 10 mg/d of prednisone.She presented with a 6-month history of intermittent fever and m...     

狼疮肾炎患者的妊娠问题

系统性红斑狼疮（systemic lupus erythematosus,SLE）是一种自身免疫性疾病,累及肾脏称为狼疮肾炎（lupus nephritis,LN）。SLE好发于中青年女性。妊娠对于女性SLE患者来说是尤其重要的一个问题。妊娠可能导致SLE患者病情恶化;SLE患者的妊娠结局可能较差。以前认为SLE是妊娠的禁忌证,现大量观察发现,SLE女性患者选择适当的时机妊娠,并在妊娠过程中严密监测病情,可以安全妊娠并分娩出健康存活胎儿。因此,现就SLE患者的妊娠问题作一综述。     

Lichen amyloidosis in an unusual location

We report lichen amyloidosis occurring on the upper lip and nasolabial folds of a 61-year-old woman from Singapore. She had a past history of systemic lupus erythematosus, which was in remission for three years. There had been no lesions of lupus erythematosus in this area. Clinically, the lesions were skin-coloured, firm papules and our differential diagnoses included trichoepithelioma, papular sarcoid or lupus miliaris disseminatus faciei. Skin biopsy from one of the lesions showed amyloid deposits in the dermis which were Congo red stain positive. These deposits also showed apple green birefringence. Immunohistochemical staining of the amyloid deposits stained positive for cytokeratins (CK) 5 and 6, and negative for CK 14. The kappa and lambda stains were equivocal. Further investigations, including multiple myeloma screen and rectal biopsy, ruled out systemic amyloidosis. There was no other evidence of cutaneous amyloidosis on her limbs or trunk. She refused treatment for her lesions. This case highlights the commonly-seen form of primary localised cutaneous amyloidosis in an unusual location.     

Pharmacological therapy of lupus nephritis

Kidney involvement is common in systemic lupus erythematosus, occurring in up to 60% of affected adults during the course of their disease. Diffuse proliferative lupus nephritis (World Health Organization class IV), the most ominous variant, has traditionally been treated with cyclophosphamide and glucocorticoids. With cyclophosphamide, women of childbearing potential must weigh the risks of sustained amenorrhea, infertility, increased susceptibility to infection, bone marrow suppression, hemorrhagic cystitis, and malignancy against the benefits of better disease control compared with glucocorticoids alone. Because of the host of adverse effects associated with cyclophosphamide, alternative approaches to the treatment of lupus nephritis are desirable. A 31-year-old woman developed class IV lupus nephritis in the postpartum period. Seeking to preserve fertility and avoid other known toxicities of cyclophosphamide, she chose to undergo therapy with mycophenolate mofetil. In the treatment of severe lupus nephritis, mycophenolate mofetil has emerged as an alternative to cyclophosphamide, offering a major advance in the therapy of lupus nephritis.     

Systemic lupus erythematosus presenting as parotitis and secondary Sjogren's syndrome

A 38-year-old woman presented with right-sided parotid swelling, dry mouth and dry eyes of one year duration. Her Schirmer's test and sialometry were positive and histopathology showed lymphoplasmacytic infiltration. She also had concomitant normochromic , normocytic anaemia and mild haematuria. Her anti-nuclear antibody titre was also positive, 1:640, with a speckled pattern on immunofluorescence. We discuss the atypical presentation of systemic lupus erythematosus, particularly parotitis and secondary Sjogren's syndrome.     

Sub-clinical systemic lupus erythematosus presenting with acute myocarditis.

A 46 year old woman presented with fever and normochromic anaemia followed rapidly by severe myocardial failure, unresponsive to maximum inotropic support and broad spectrum antibiotics. There were no classical clinical stigmata of systemic lupus erythematosus (SLE) but a possible immunological cause was looked for, and on the basis of her immuno-serology a diagnosis of SLE-like disease was made. She responded rapidly to high dose steroids. The importance of considering the possibility of SLE or ''lupus overlap'' in an acutely ill ''undiagnosed'' patient is emphasized. The relevance of instigating appropriate immuno-serological tests in the course of such an illness is discussed.     

Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant

The lupus anticoagulant was found in the plasma of 31 of 60 patients with systemic lupus erythematosus and other connective tissue disorders (mixed connective tissue disease, systemic vasculitis, polyarteritis nodosa, primary sicca syndrome, discoid lupus, Behcet's syndrome, and systemic sclerosis). Strong associations were found with biological false positive seroreaction for syphilis and thrombocytopenia. The most striking association, however, was with the high prevalence of thrombosis. This tendency to thrombosis was independent of disease activity of systemic lupus erythematosus. The lupus anticoagulant appears to be a useful marker for a subset of patients with systemic lupus erythematosus at risk for the development of thromboembolic complications.     

早发性卵巢功能不全患者赠卵IVF获得妊娠在孕期合并系统性红斑狼疮:1例报告并文献复习

为探讨早发性卵巢功能不全(POI)与免疫因素的关系,对我院生殖中心接诊的1名POI患者通过赠卵成功妊娠后发生系统性红斑狼疮(SLE)的病例进行报告,并结合相关文献,回顾POI病因,讨论SLE与妊娠的相互影响以及POI患者妊娠后合并SLE的临床特点及产科处理,以期为不明原因/免疫因素POI患者孕前处理及妊娠后产科随访提供参考.该病例中,患者确诊卵巢早衰4年,2003年我中心行赠卵IVF成功妊娠.孕11周以颜面部浮肿为首发症状,经免疫学检查、肾脏穿刺活检后确诊为SLE、狼疮性肾炎.综合相关专科意见以及患者意愿,予口服强的松控制狼疮活动,加用阿司匹林、低分子肝素改善胎盘血流,密切监测孕妇各项指标及胎儿生长发育.期间孕妇病情反复,孕晚期出现肝功能损害、胎盘功能下降,B超监测不除外胎儿发育迟缓可能,经全院讨论,择期剖宫产分娩一早产男婴.产后继续专科治疗,随访14年,期间患者复查免疫指标逐渐好转,无狼疮相关表现,考虑SLE临床治愈.其子经新生儿科诊治,其生长发育与同龄人无异.通过病例分析和文献回顾,我们认为免疫因素是POI重要病因,并建议不明原因POI患者孕前完善免疫学检查以保障母胎生命.