Hemoglobin E Hemoglobinopathy in an Adult from Assam with Unusual Presentation: A Diagnostic Dilemma.

Hemoglobin E (HbE) is estimated to affect at least one million people around the world. Carrier frequency of hemoglobin E/ß-thalassemia (HbE/ß-thalassemia) is highest in Southeast Asia, reaching as high as 60% in parts of Thailand, Laos, and Cambodia. In the Indian subcontinent, highest frequency is observed in The Northeast regions, but relatively rare in rest of the country. Increasing migration of population from highly affected areas is resulting in rising prevalence in The South and other parts of India. HbE/ß-thalassemia is characterized by marked clinical diversity, phenotypic instability, and age-related changes in adaptation to anemia. This paper reports a case of HbE disease in an adult immigrant from Assam and documents the difficulties encountered in the definitive subtyping of HbE hemoglobinopathy. Distinguishing between homozygous HbE disease and HbE/ß-thalassemia is a challenge to hematopathologist as both are clinically and hematologically similar.

Double gallbladder with different disease entities: A case report.

We report a rare case of gallbladder duplication in a young male patient with acute pyocoele in one vesicle and acute cholecystitis with cystadenoma in the other; another unusual feature was the absent or obliterated cystic duct in the proximal vesicle and non-communication with the second vesicle or the biliary system. Ultrasound examination had suggested a septate gallbladder; the diagnosis of dual gallbladder was made per-operatively during separation of the distal moiety which was presumed to be an adherent duodenum initially. Intraoperative cholecystogram confirmed the diagnosis and both gallbladders were removed successfully laparoscopically.A high degree of awareness, detailed preoperative investigations when anomalies are suspected and intraoperative cholangiography are necessary for accurate detailing of the biliary tree to avoid inadvertent damage to the biliary ductal system and overlooking of second or third gallbladder during surgery.

True giant intra-abdominal esophageal cyst.

Isolated intra-abdominal duplication cysts of foregut origin are extremely rare and are discovered incidentally. We report a 70-year-old lady with a giant, truly intra-abdominal esophageal duplication cyst. She was symptom-free one year after excision of the cyst.