Manual red cell exchange transfusion to avert sickle cell related complications.

Sickle cell-beta thalassemia is a double heterozygous state. Red cell exchange (RCE) transfusion reduces the concentration of sickle cells without increasing the hematocrit or whole-blood viscosity. It can be performed manually or by erythrocytapheresis. RCE transfusion is an effective tool for both acute and chronic complications of sickle cell disease. In patients unaffording erythrocytapheresis, even manual RCE can give favorable results. A 37-year-old male, a known case of sickle cell-beta+ thalassemia (ßsß+), presented with avascular necrosis of right femur and humeral head. He was posted for the right hip arthroplasty and shoulder hemiarthroplasty. Successful manual RCE transfusions were done. The hemoglobin S levels decreased postmanual RCE procedures, and the patient was operated successfully.

Buccal Mucosa Exfoliative Cell Prussian Blue Stain Co-Relates with Iron Overload in ß-Thalassemia Major Patients.

Thalassemics require regular blood transfusion therapy leading to iron overload in the body tissues, which is a major cause of morbidity and mortality in these patients. We hereby attempted to measure this iron overload by means of exfoliative cytology, a non-invasive and inexpensive technique. The aims and objectives of our study were: 1. To detect iron overload by oral exfoliative cytology using Perl's Prussian blue stain in ß-thalassemia major patients. 2. To correlate staining positivity with serum ferritin levels. Smears were obtained from buccal mucosa of 50 ß-thalassemia major patients (who had taken more than 12 transfusions) and 25 healthy subjects of the same age group as controls. Smears were stained with Perl's Prussian blue. Blood samples were taken from the study group for estimation of serum ferritin levels. Grading criteria were defined for assessing the Prussian blue positivity. Perl's positivity was observed in 49 out of 50 of thalassemic patients (98%). 1 patient had Grade 0, 7 patients had Grade I, 5 had Grade II, 12 had Grade III, 14 had Grade IV while 11 patients had Grade V positivity. Spearman Rank's Correlation Co-efficient was 0.38, signifying a weak positive correlation between positivity of buccal smears for Perl's Prussian blue staining and respective serum ferritin levels. Perl's Prussian blue staining of exfoliated cells from buccal mucosa can be used to assess iron overload in ß-thalassemia major patients, as a screening as well as diagnostic tool. With the grading system we can give a semi-quantitative assessment of the same.