Arsenic intoxication presenting as a myelodysplastic syndrome: a case report

A case of arsenic intoxication presenting as a myelodysplastic syndrome is reported. A 41-year-old woman with a 6-month history of gastrointestinal and neurological symptoms was noted to be pancytopenic at presentation. A bone marrow aspirate revealed dysmyelopoietic changes involving all three marrow cell lines. Subsequent analysis of urine for heavy metals demonstrated very high levels of arsenic. Treatment with British anti-Lewisite (BAL) resulted in the resolution of gastrointestinal symptoms and hematological abnormalities although the neurological complications progressed. This case emphasizes that heavy metal intoxication should be considered in the differential diagnosis of any individual presenting with the hematological features of myelodysplasia especially when accompanied by clinical features considered atypical for primary or secondary myelodysplasia.     

Intravascular lymphomatosis (malignant angioendotheliomatosis) presenting as pulmonary hypertension

Intravascular lymphomatosis is a rare lymphoma characterized by proliferation of malignant cells within the lumen of small blood vessels. We describe a case of intravascular lymphomatosis resulting in pulmonary hypertension, hypoxemia, and dyspnea. This lymphoma occasionally responds to combination chemotherapy, suggesting that pulmonary hypertension secondary to intravascular lymphomatosis may be reversible. Intravascular lymphomatosis should be considered in the differential diagnosis of pulmonary hypertension.     

Lyme carditis presenting as acute coronary syndrome: a case report

A case of a 26-year-old man with Lyme carditis (LC) mimicking acute coronary syndrome is presented. Considering clinical presentation, electrocardiographic findings and markedly elevated levels of cardiac biomarkers, emergency coronary angiography was performed and revealed normal coronaries. Ventricular arrhythmias of Lown grade IVb during catheterization were recorded. Echocardiography showed mild global left ventricular dysfunction with ejection fraction of 50%. The diagnosis of LC was confirmed by ELISA and Western blot serologic testing. After 21 days of continuous antibiotic therapy with ceftriaxone (2.0 g/d) the patient recovered completely. We also present the current state of knowledge on the cardiovascular aspects of Lyme borreliosis.     

一例表现为 Av ellis 综合征的延髓梗死

Av ellis 综合征于1891年由德国喉科医师Av ellis率先报道,其受累病灶主要位于疑核上部和脊髓丘脑侧束,临床症状主要表现为吞咽困难,病灶侧软腭、喉和会厌麻痹,声音嘶哑,言语不清,病灶对侧肢体分离性感觉障碍,以及病灶对侧面部痛温觉减退等［1-3］。 Av ellis 综合征的相关病例报道主要集中于延髓梗死患者,与动脉粥样硬化和血栓形成导致椎基底动脉和延髓动脉管腔狭窄或闭塞引起的急性脑缺血密切相关［4］,但国外也有报道头部外伤、鼻翼神经营养性溃疡、Borrelia疏螺旋体感染、全身性血管炎、溃疡性结肠炎等疾病引起该综合征的病例［5-8］。疑核上部受损可引起严重的咽喉麻痹,其严重程度可能与延髓外侧缺血性病变范围有关［9］。当病灶位于疑核上部时,Avellis综合征的吞咽困难、声音嘶哑等临床表现更为明显;脊髓丘脑侧束受损时,可出现对侧手臂、躯干和腿浅感觉减退;腹侧三叉神经丘脑束（包括腹侧三叉神经核上行纤维）受损则出现病灶对侧面部浅感觉减退［10］。由于腹侧三叉神经核上行纤维临近延髓外侧的脊髓丘脑侧束,若同时损伤上述2个部位则会出现相应叠加的临床表现［11］。 Avellis 综合征的发病率相对较低,在国内鲜有报道,现报道1例。     

首诊睡眠呼吸暂停综合征的Chiari畸形1例

Chiari畸形(Chiari malformation,CM)是一种先天性小脑扁桃体延髓联合畸形,表现为小脑扁桃体楔形延长,伸入枕骨大孔而达颈椎管内,后组颅神经、上部颈神经根受牵拉下移而产生相应症状,主要表现为:(1)延髓及上颈段症状;(2)后组神经损害症状;(3)小脑症状;(4)高颅压症状.CM尚有其他临床表现,如小脑扁桃体疝出压迫延髓所致的睡眠呼吸暂停.     

Sarcoidosis presenting with Raynaud's syndrome: a case report

We report a case of association between sarcoidosis and Raynaud's syndrome. A 39 year old female presented fatigue, Raynaud's syndrome, IgG and erithrosedimentation rate (ESR) increase, polyarthralgy in which disseminated micronodular infiltration in the chest X-ray and histological demonstration of non-caseating epitheloid microgranulomas led to a diagnosis of concomitant sarcoidosis. Clues to the diagnosis of sarcoidosis coexisting with autoimmune disease are discussed.     

(18)FDG-PET-scan in staging of primary malignant melanoma of the oesophagus: a case report

Primary malignant melanoma of the oesophagus is a rare disease, only 262 cases being reported up to June 2005. In general, the prognosis is dismal because of its tendency to present as an advanced neoplasm with aggressive biological behaviour. (18)FDG-PET-scan is a useful tool for evaluation of metastatic disease and locoregional lymph node metastasis. We present herein a case of a young adult with primary malignant melanoma of the oesophagus, followed by a literature review on the subject.     

Primary antiphospholipid syndrome presenting as hyponatremic hypertensive syndrome and acute adrenal insufficiency following it: a rare case

Hyponatremic hypertensive syndrome (HHS) is rare and is characterized with hypertension together with hyponatremia, hypokalemia and low plasma osmolarity and considered to be based on renal ischemia. It can rarely develop due to antiphospholipid syndrome (APS). We reported a case of 43-year-old man with unilateral renal artery stenosis due to primary APS. He presented with a hypertensive hyponatremic episode. First, he was treated with antihypertensive drugs. Further, radiological imaging showed enlargement of bilaterally adrenal glands that mimic adrenal masses. Unilateral nephrectomy and adrenalectomy were performed. Pathological examination revealed necrosis in the adrenal tissue and thrombosis in its artery. After operation, acute adrenal insufficiency developed in the patient because of hemorrhagic infarction of bilaterally adrenal glands. He improved after intravenous hydrocortisone treatment. His therapy was maintained with oral prednisolone and fludrocortisone. This patient with HHS, due to primary APS, is the first case in literature and acute adrenal insufficiency is a rare complication of APS.     

Arrhythmogenic right ventricular dysplasia presenting as acute coronary syndrome: a case report.

Arrhythmogenic right ventricular dysplasia (ARVD) is underdiagnosed cardiomyopathy which commonly presents in young adults with ventricular tachycardia or sudden death. We report a case of ARVD presenting with features of acute coronary syndrome. The suspicion of ARVD came only when echocardiogram revealed abnormal shape and wall motion of right ventricle, which was later confirmed by right ventricular angiogram. The diagnosis of ARVD was discussed and the literature reviewed.     

Ectopic pancreatic tissue presenting as an umbilcal mass in a newborn: a case report

The case of an ectopic pancreatic mass at the umbilicus in an 8-day-old male neonate is reported, the youngest patient with this condition ever reported in an English-language journal. The patient was healthy except for a protruding mass with intermittent mucous discharge at the base of the umbilical stump. Surgical intervention was performed under the impression of the umbilical mass. Pathology diagnosed an ectopic pancreas with acute hemorrhage. To the best of our knowledge, only one case of ectopic pancreas presenting as an umbilical mass with intermittent mucous discharge has previously been reported.     

Portal hypertension as the initial manifestation of POEMS syndrome: a case report

Background

Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. In differential diagnosis of portal hypertension, POEMS syndrome should be considered especially when other symptoms such as numbness, organomegaly, endocrine alteration and skin changes also present, as it is highlighted by our case.

Case presentation

We report a 46-year-old Chinese male, a teacher, presenting with portal hypertension. Electromyography revealed peripheral neuropathy. Immunofixation showed monoclonal immunoglobulin A lambda protein. The diagnosis of POEMS syndrome was established. After treatment of lenalidomide combined with dexamethasone over 2 years, the patient achieved a considerable improvement.

Conclusion

This case highlights the manifestation of portal hypertension in POEMS syndrome. Lenalidomide with or without dexamethasone is effective for portal hypertension due to POEMS syndrome, though esophageal and gastric varices seems not reversible so easily.

     

Antiphospholipid syndrome associated with malignant mesothelioma presenting with superior vena cava thrombosis: a case report.

A 59-year-old woman who had dyspnea and neck swelling for 10 days was admitted to the hospital. Malignant peritoneal mesothelioma was diagnosed previously. According to the clinical findings, and laboratory and pathologic examination, the patient was found to have disseminated venous thrombosis and antiphospholipid syndrome, which is treatment-resistant autoimmune paraneoplastic syndrome.     

Poland syndrome (a case report)

Poland syndrome is characterized with unilateral absence of pectoralis major muscle. Its incidence is one in 30000 live births. A 20 years old case with Poland syndrome is presented together with its clinical and laboratory features in this study. The case had anomaly of shortness of right hand fingers and syndactily between second and third fingers in addition to absence of right pectoralis muscle group. There was not another associated anomaly except aforementioned ones. Strength loss in abduction and adduction of right shoulder was detected with Cybex dynamometer. Furthermore decrease in predicted maximal inspiratory and expiratory pressures was detected.     

Intrahepatic cholangiocarcinoma presenting as the Budd-Chiari syndrome: a case report and literature review.

Intrahepatic cholangiocarcinoma, an increasingly recognized primary tumour of the liver, is associated with a very poor prognosis. A patient with this tumour who presented with Budd-Chiari syndrome (the first to the authors' knowledge in Western literature and only the third patient overall) secondary to extensive thrombosis in his inferior vena cava extending from the right atrium down to his iliac vessels is described. Neither curative nor palliative intervention was deemed to be an option in this patient, who deteriorated rapidly while on anti-coagulants. Postmortem examination confirmed the radiological findings, and histological analysis revealed characteristic appearances of this tumour within the biliary tree and invasion into the inferior vena cava. Furthermore, biliary dysplasia, which can be a precursor to this cancer, was also noted within some of the bile ducts.     

Crohn's disease presenting with erythema nodosum as an early sign: a case report

Erythema nodosum is the most common form of septal panniculitis and the most frequent skin manifestation associated with inflammatory bowel disease, affecting up to 15% of Crohn's disease patients. Since the development of erythema nodosum is closely related with a variety of disorders and condition, it can serve as an important early sign of systemic disease. Here, we present the occurrence of erythema nodosum as an early sign of Cronh's disease in a 16-year-old woman.