Implications of Population Screening for Thalassemias and Hemoglobinopathies in Rural Areas of West Bengal, India: Report of a 10-Year Study of 287,258 Cases.

Thalassemia and hemoglobinopathies are the most common cause of high morbidity and mortality in India. Detection of carriers and premarital counseling play an important role in preventing the birth of a thalassemic child. The present study aimed to detect large numbers of asymptomatic carriers in rural areas of West Bengal, India. The present cross-sectional study was conducted over a period of 10 years. Thalassemia awareness programs and detection camps were organized at the community level. After signed written consent was obtained, the collected blood samples were subjected to a complete blood count (CBC) in an automated blood cell counter and then analyzed by high performance liquid chromatography (HPLC); in difficult cases, samples were sent to the reference laboratory for molecular characterization. Out of 287,258 samples collected, 32,921 (11.46%) cases revealed abnormal hemoglobins (Hbs); of these, 31,782 (11.06%) carried heterozygous states (carriers/traits), and the remainder were either homozygous or compound heterozygous for different hemoglobinopathies. Two common variants were revealed in the study, namely ß-thalassemia (ß-thal) (7.23%) and Hb E [ß26(B8)Glu→Lys, HBB: c.79G>A] (2.77%) traits. Among homozygous or compound heterozygous states, Hb E/ß-thal (0.14%) and ß-thal major (ß-TM) (0.12%) were predominant. In rural areas of West Bengal, the most common Hb variants detected were ß-thal and Hb E traits. In view of the high prevalence of hemoglobinopathies in this region, routine premarital screening and genetic counseling should be emphasized and encouraged to prevent the birth of a thalassemic child, and thus curtailing the burden on families and the health economy.

Corrigendum to "An Unusual Cause of Bleeding in a Patient with Chronic Myeloid Leukemia Chronic Phase".

[This corrects the article DOI: 10.1155/2019/5674193.].

An Unusual Cause of Bleeding in a Patient with Chronic Myeloid Leukemia Chronic Phase.

Chronic myelogenous leukemia (CML) is a clonal myeloproliferative neoplasm (MPN) characterized by dysregulated and uncontrolled proliferation of mature and maturing granulocytes with normal differentiation. A genetic hallmark of CML is the presence of the fusion gene product BCR-ABL. Bleeding diathesis in CML patients is rare (<10%) and primarily caused by acquired platelet dysfunction. We report a rare case of an adult CML chronic phase patient who presented with spontaneous muscle hematoma due to acquired Glanzmann's thrombasthenia (GT). On laboratory workup, a GT was confirmed along with the diagnosis of CML in chronic phase. The muscle hematoma was completely resolved following imatinib therapy. The present case demonstrates that bleeding is a complication of MPNs and highlights the importance of both acquired GT diagnosis to determine the cause of bleeding in CML and of prompt treatment with imatinib to reverse this condition.

Etiology of Pancytopenia: An Observation from a Referral Medical Institution of Eastern Region of India.

BACKGROUND: Pancytopenia is a relatively common hematological condition, the etiological factors of which vary widely in different geographic location. Determining the specific etiology is of immense importance for appropriate management. AIMS AND OBJECTIVES: The present study was undertaken to delineate etiological factors leading to pancytopenia in a Tertiary Care Hospital of West Bengal from Eastern Region of India. MATERIALS AND METHODS: A prospective study was conducted for a period of 2 years in which 248 patients were included. After obtaining a relevant clinical history, physical examination was done followed by complete blood count including peripheral blood smears examination, relevant biochemical, and radiological investigations. Afterward, bone marrow aspiration and biopsy were performed and microscopically examined. RESULTS: Among 248 patients studied, 156 (62.9%) were males and 92 (37.09%) were females. The mean age of the patients was 33 years. Aplastic anemia was the most common cause of pancytopenia that was observed in 83 cases (33.47%) followed by megaloblastic anemia in 52 cases (20.97%), leishmaniasis in 34 patients (13.71%), hypersplenism also in 34 patients (13.71%), and tuberculosis and other connective tissue disorders in 18 cases (7.26%). The occurrence of aplastic anemia was statistically significant in pediatric (≤15 years) age group. CONCLUSION: Aplastic anemia was found to be the most common cause of pancytopenia in this study, which is in contrast to studies conducted from other regions of India. Delineation of etiologies of pancytopenia in various regions can help in defining diagnostic and therapeutic strategies, which is expected to contribute toward the better management of such patients.