The present study was carried out to assess the sickle cell anemia and morbidity pattern among rural population in Chandrapur district of Maharashtra. The subjects were screened by solubility test and positive samples were subjected to cellulose acetate membrane electrophoresis. The study showed that 18.3 % subjects were having sickle cell anemia. The common morbidity forms were joint pain (14....

The most common cause of stroke in children with sickle cell anemia is infarction due to ischemia. In adults, however, stroke is most commonly hemorrhagic in nature. Other causes of stroke in patients with sickle cell disease are very rare. In this short communication, we describe a woman with sickle cell anemia responsive to hydroxyurea (HU) therapy who had primary stroke due to paradoxical em...

Since the first report of a young girl affected by sickle cell anemia, treated successfully by bone marrow transplantation (BMT) for acute myeloid leukemia, more than 200 patients have been transplanted worldwide for sickle cell anemia. The disease-free survival (DFS) is good (80-85% in several series), even though many children who received allografts had already significant sickle-related com...

Hematopoietic stem cell transplantation (HSCT) still remains the only definitive cure currently available for patients with thalassemia and sickle cell anemia. Results of transplant in thalassemia and in sickle cell anemia have steadily improved over the last two decades due to improvements in preventive strategies, and effective control of transplant-related complications. From 2004 through 20...

By E. SILvEsTR0NI, M.D. AND I. BIANCO, M.D. S ICKLE CELL ANEMIA is a severe chromsic disease usually confined to the Negro race l ttt occasiotsally foutsd its imidividuals of the white race mostly of Southerti Italians on Greek stock. Clitsically there is chromsic hemolytic amsemia s ’iths crises, osteo-muscular pains, bouts of severe abcbomitsal pain and ulceratiomis of the legs. Hematological...

BACKGROUND Posterior reversible encephalopathy syndrome is a neurotoxic condition that occurs as a result of the failure of posterior circulatory autoregulation in response to acute changes in blood pressure. Overperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction. Posterior reversible encephalopathy syndrome can be the presenting featur...

Oxidative stress may be contributory to the pathophysiology of the abnormalities that underlie the clinical course of sickle cell anemia. We looked for a possible genetic association between the functional polymorphism Ala-9Val in the human Mn-SOD gene and sickle cell anemia. One hundred and twenty-seven patients with sickle cell anemia and 127 healthy controls were recruited into the study. Al...

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, characterized clinically by fever, splenomegaly, cytopenia, and high ferritin. Infectious causes have been associated with secondary HLH, with viruses being the most common. We report a case of Mycobacterium avium complex-associated HLH in a sickle cell anemia patient. To the best of our knowledge, this association h...

Cardiopulmonary bypass (CPB) in patients with sickle cell anemia can trigger lethal vaso-occlusive crises, especially cases of hypoxia, hypothermia, acidosis, or low-flow states. We described a patient who had bicuspid aortic valve stenosis and aneurysmal dilatation the ascending aorta complicated infective endocarditis. The history stroke. During routine workup, Hodgkin’s Lymphoma was diagnosed...

Compound heterozygosity for βS/βD results in a severe hemolytic anemia and a clinical syndrome similar to that of sickle cell disease. Here, we report a case of HbSD Punjab disease. A 10 year old female child residing at Nagpur, Maharashtra presented with severe hemolytic anemia, hepatosplenomegaly and occasional pains in bones and abdomen. Initially, she was thought to be a case of sickle cell...