Spectrum of hemoglobin variants in the population of northern region of west bengal: an ethnogenetic proposition.

CONTEXT: The birth of transfusion-dependent states of hemoglobinopathies including thalassemias is preventable by population screening and genetic counseling. Magnitude is not addressed in the Northern Region of West Bengal where many ethnic variants inhabit. AIMS AND OBJECTIVES: The aim of the following study is to find out the burden of different entities of hemoglobinopathies, their correlation with ethnicity and the "at risk" groups. SUBJECTS AND METHODS: A descriptive study was conducted from the Hematology Unit of North Bengal Medical College over 1 year on the subjects underwent screening for hemoglobinopathies for detection of abnormal hemoglobin (Hb) variants by "cation-exchange high-performance liquid chromatography" principle along with other relevant tests. STATISTICAL ANALYSIS: Data was analyzed by frequency distribution and Chi-square test assuming P value as 95% of the level of significance using the SPSS version 16 (SPSS Inc., Chicago, Illinois, U.S.A). RESULT: Abnormal Hb variant was 47.5% among 1872. Hb E trait (34.4%) was most common followed by Hb E disease (25.3%) and others. Hb E disorders (92.7%) were observed mostly among Rajbangsi population while E-ß-thalassemias (40%) in the Muslims and a heterogeneous pattern noted among tribal and mongoloid. CONCLUSION: Hb E hemoglobinopathies was high among Rajbangsi and Muslims with identification of some other hemoglobinopathies involving tribal and mongoloid.

Cytopathological dilemma of anaplastic sacral chordoma with radiological and histological corroboration.

Chordoma is a relatively rare locally invasive and potentially malignant tumor of fetal notochord origin, affecting the axial skeleton. Cytopathological diagnosis of chordoma is favored by the presence of characteristic physaliphorous cells, bearing abundant foamy cytoplasm dispersed in a myxoid matrix. Anaplastic chordoma or dedifferentiated chordoma, an even rarer variant, can cause a diagnostic confusion with chondrosarcoma from the cytopathological point of view, with similar chondromyxoid matrix and atypical cells. Hence, chordoma bearing anaplastic features needs to be identified and should be distinguished from chondrosarcoma on aspiration cytopathology. We present a case of anaplastic sacral chordoma in a man 59 years of age, causing extensive destruction of sacrum and invading the paravertebral tissues as evidenced by radiology. Fine needle aspiration cytopathology revealed few large pleomorphic hyperchromatic cells, admixed with characteristic physaliphorous cells and myxoid matrix. The cytopathological diagnosis has been confirmed by histopathology and immunohistochemistry. Since anaplastic chordoma bears an unfavorable prognosis, it should be suspected on preoperative aspiration cytopathology. Clinicoradiological correlation along with histopathological and immunohistochemical confirmation is necessary subsequently.

Cytological diagnosis of adamantinoma of long bone in a 78-year-old man.

A clinicohistopathological study of a rare case of adamantinoma of long bone in a 78-year-old patient is presented. The cytological features when evaluated in conjunction with clinical and radiologic features are sufficiently diagnostic. The primary knowledge of its existence and knowledge of its cytological features are important for a correct preoperative cytological diagnosis.

Clinicohaematological analysis of aplastic anaemia among children of northern districts of West Bengal.

Though aplastic anaemia in children is an important haematological disorder, not many studies have been undertaken in India and especially in the northern districts of West Bengal. The present study was carried out at North Bengal Medical College and Hospital to find the occurrence, clinical and hematological profile of children with aplastic anaemia. All the children (less than 15 years) having relevant history and clinical features underwent a complete blood count and bone marrow aspiration study (smear and histological sections) and trephine biopsy. Total number of children diagnosed were 21 (M = 17, F = 4), having an age range of 6-14.5 years (mean 11.36). Relevant aetiological factors were noted in 10 cases (47.61%), including history of exposure to insecticides, fungicides and fertilisers in 5 cases (23.81%). The common symptoms were due to anaemia (weakness, pallor in all cases), leucopoenia [fever in 16 cases (76.19%)] and thrombocytopenia [bleeding in 10 cases (47.61%)]. Severe and moderate aplastic anaemia were noted in 7 (33.33%) and 12 (57.14%) cases respectively. The calculated occurrence of aplastic anaemia is 1.96/million population of children/year in the four northern districts of West Bengal. The bone marrow aspiration was satisfactory in all cases. Histological sections of aspirated marrow particle produced better architectural relationship among different components. Aplastic anaemia is a major hematological problem among the children of northern districts of West Bengal. Simultaneous examination of smear and histological sections of marrow particles is a satisfactory method for detection of aplastic anaemia.