Hemoglobin E (beta26Glu --> Lys) is the most common hemoglobin (Hb) variant in Southeast Asia and the second most prevalent worldwide. However in India, it is prevalent in Bengal and the north-eastern region, but relatively rare in the rest of the country. Identification of this Hb variant is important, because the doubly heterozygous state for HbE and beta-thalassemia is characterized clinically by thalassemia major, a situation different from other compound heterozygous states for structural beta-chain variants and beta-thalassemia. Thus, the affected individual may be symptomatic and transfusion dependent at an early age. This paper reports four cases with Hb E trait, three cases with hemoglobin E disease and another four cases with Ebeta-thalassemia. Laboratory investigations are based on RBC indices and high performance liquid chromatography (HPLC). A negative correlation has been found to exist between levels of HbA(2) and RBC indices including the MCV and MCH. A similar correlation has been seen between levels of HbF with Hb, RBC count, and MCV. The main aim is to increase the awareness of this relatively rare disorder, so that it can be included in the differential diagnosis of patients presenting clinically like thalassemia intermedia or thalassemia major. This awareness may also help in prenatal diagnosis, genetic counseling and clinical management. The clinical, hematological and laboratory features of this disorder are also discussed.
Quadrigeminal lipoma is a rare tumor that has been categorized as developmental malformation rather than a hamartoma or true neoplasm, due to its origin from abnormal persistence and mal-differentiation of meninx primitiva during the development of the subarachnoid cisterns. Reported admixture of adipose tissue with heterotopic elements also supports a developmental origin. Quadrigeminal lipomas are frequently asymptomatic and detected incidentally. Though a favorable clinical course is usually expected, recurrences may occur due to partial removal of lesions in close relation to vital structure. We describe the role of intraoperative squash smear cytology as a diagnostic aid in quadrigeminal cistern lipoma and an alternative to frozen sections that are technically difficult to obtain due to presence of lobules of fibro-adipose tissue. With radiological correlation, squash cytology can be an economical method for intraoperative diagnosis, pending subsequent histopathological confirmation.
Odontogenic myxofibroma represents a rare slow-growing benign neoplasm, which usually occurs in the second and third decades of life and rarely in children or adults over 50 years of age. Myxomas in general represent from 2.3% to 17.7% of all odontogenic tumors, and myxofibromas represent a small number of all myxomas. Limited evidence is present in literature regarding the cytological diagnosis of odontogenic myxoma/myxofibroma. We hereby report the cytomorphological features of a histologically confirmed case of odontogenic myxofibroma and the pitfalls of the cytological diagnosis. A painless jaw swelling in a young boy was aspirated. Scanty mucoid material was obtained. Cytology Smears were moderately cellular and showed a population comprising predominantly of singly scattered plump to fusiform cells with bipolar cytoplasmic processes showing mild to moderate atypia embedded within dense myxoid matrix and another population of cells arranged in clusters. Case was interpreted as low grade mesenchymal tumor. Subsequent biopsy confirmed it as odontogenic myxofibroma arising in a odontogenic keratocyst. Precise interpretation of intraosseous jaw lesions FNAC may not always be possible, but an attempt should be made to broadly classify the lesion as an inflammatory lesion, cystic lesion, giant cell lesion, fibro-osseous lesion or as an odontogenic tumor. If dual population of odontogenic epithelium and mesenchymal cells embedded in myxoid matrix are identified in such aspirates, a possibility of myxoid odontogenic tumor may be suggested. Triple correlation of cytological, clinical and radiological findings can guide the surgeon for taking appropriate therapeutic decisions.
Coeliac hepatitis could be a distinct entity and the patients may present with either CD or secondary hepatic derangement. Evaluation for the presence of CD is recommended for patients presenting with AILD, unexplained transaminasaemia or anaemia. This is one of the very few studies demonstrating the continuum of liver histological changes in 'coeliac hepatitis'. Trial of a GFD may result in clinicopathological improvement of 'coeliac hepatitis'.
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