Sickle Cell Disease in Central India: A Potentially Severe Syndrome.

OBJECTIVES: To explore clinical, hematological and molecular features of homozygous sickle cell (SS) disease in central India. METHODS: Focusing on the pediatric age group attending a clinic at the Akola Government Medical College, Akola, Maharashtra State, India, a cross-sectional assessment of 91 patients with sickle cell disease was performed during one week in March 2015. RESULTS: Of the 91 patients, there were 49 with SS disease, 36 with sickle cell-beta thalassemia, and 6 with sickle cell-HbD Punjab. Alpha globin gene deletions occurred in only 8/49 (16 %) SS disease but fetal hemoglobin (HbF) levels were markedly elevated with mean and median of 24.4 %; all except 3 SS disease patients had the Xmn1(+/+) polymorphism consistent with the Asian haplotype. Among the 36 patients with sickle cell-beta thalassemia, 25 (69 %) had the severe beta(+) mutation, IVS1-5 G > C, and seven other molecular mutations, all beta(o) occurred in the other 11 patients. Many patients had a relatively severe clinical course. Comparison of SS disease and sickle cell-beta thalassemia showed no differences in the prevalence of dactylitis, bone pain crisis, acute chest syndrome, hemoglobin level, reticulocyte counts or hydroxyurea usage but patients with sickle cell-beta thalassemia had significantly more blood transfusions, and greater frequencies of splenomegaly and hepatomegaly. CONCLUSIONS: Many patients in central India have relatively severe manifestations. This may result from lower frequencies of alpha thalassemia and more frequent severe sickle cell-beta(+) thalassemia. There is a need for assessment of the indications and policies for blood transfusion and for hydroxyurea.

Fine needle aspiration cytology of vulvar actinomycosis masquerading clinically as malignancy.

Actinomycosis is a chronic infection caused by Actinomyces israelii, usually seen in immunocompromised patients or in the background of tissue injury. Vulvar actinomycosis presenting as a fixed swelling in an elderly individual can mimic malignancy and pose a diagnostic dilemma. We report here a case of vulvar actinomycosis diagnosed by fine needle aspiration (FNA). A 60-year-old postmenopausal female presented with a gradually increasing 15 cm × 10 cm vulvar swelling involving the right mons and right labia majora. The swelling was nodular, fixed, and nonulcerated, with multiple healed and few active discharging sinus tracts draining serosanguineous fluid and yellowish sulfur granules. FNA was diagnostic, as it revealed colonies of Actinomyces surrounded by polymorphs. Histopathology of excised specimen confirmed the cytological findings. FNA is an effective tool in the diagnosis of actinomycosis, although, its documentation is rare. Difficulties in the management can be avoided by early diagnosis using the FNA technique.