ABSTRACT
Rosai Dorfman disease is a benign lymphoproliferative disorder. Isolated extranodal involvement is rarely encountered. The causation has not been attributed to any particular factor and the clinical progress of the disease is varied. Treatment options range from observation to medical therapy to surgical excision. Long term follow up is advocated. We report an elderly female with complaints of nasal obstruction who underwent ESS and was diagnosed with this disease on account of histopathology. Follow up period has been uneventful. The case report illustrated here is aimed at highlighting high index of suspicion as well as creating awareness regarding diagnosis and management of a rarely seen pathology.
ABSTRACT
Humoral immune responses can produce autoantibodies against self-cellular proteins and nucleic acids without the presence of autoimmune diseases. Numerous kinds of autoantibodies are detected in liver diseases such as viral hepatitis, alcoholic liver disease (ALD), and nonalcoholic fatty liver disease (NAFLD), where their production could be secondary to hepatocellular inflammation and necrosis. Hence, the presence of an autoantibody does not necessarily indicate the presence of autoimmune disease; nor does it predict its severity and potential response to therapy. In literature, the spectrum and methods of diagnosis of liver disease in lupus are well described. However, chronic liver disease can manifest with signs similar to those in lupus, and it is important to recognize that autoantibodies in patients with chronic liver disease can be seen without the presence of autoimmune rheumatic disease. In this report, we discuss a very interesting case of a middle-aged female with a history of ALD presenting with calciphylaxis, thrombocytopenia, hypocomplementemia, and positive serologies, but without any clinical evidence of autoimmune rheumatic disease.
ABSTRACT
Undifferentiated embryonal sarcoma of the liver is a rare entity. It is a malignant primitive mesenchymal tumor seen in the pediatric age group often between 6 to 10 years of age. It involves the right lobe of the liver commonly and is usually asymptomatic. Acute presentation in these cases is secondary to its rupture/ wall dehiscence. Alfa fetoprotein, a tumor marker elevated in most of the hepatic malignant tumors is however normal in undifferentiated embryonal sarcoma. Imaging wise it is a large encapsulated multiseptated lesion. It shows a "paradoxical appearance" with a predominantly solid appearance on ultrasonography and cystic appearance on CT/MRI. This is a peculiar feature that can help in the early diagnosis of this entity. Besides, normal serum alfa fetoprotein levels favor its diagnosis. Hereby we present a case of a 5-year-old female child, presented with complaints of acute onset abdominal pain and distension which on imaging investigation showed a liver mass with typical paradoxical appearance on ultrasonography, CT and MRI as described.
ABSTRACT
Persistent left superior vena cava is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cor triatriatum. The presence of PLSVC can render access to the right side of heart challenging via the left subclavian approach, which is a common site of access utilized when placing pacemakers and Swan-Ganz catheters. Incidental notation of a dilated coronary sinus on echocardiography should raise the suspicion of PLSVC. The diagnosis should be confirmed by saline contrast echocardiography.
