Hairy cell leukemia-variant--a case report.

Hairy-cell leukemia-variant (HCL-V) is a rare B-cell disorder which accountsfor 10% of HCL cases. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. The circulating cells have a morphology intermediate between prolymphocytes and hairy cells. The immunophenotype shows a mature B-cell phenotype with expression of B-cell antigens CD11c and CD103 but unlike typical hairy cell the cells are negative for CD25. The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL. We present a case of HCL-V in a 66-year-old male. The bone marrow findings, immunophenotypic profile and electron microscopic features are described. The patient underwent splenectomy which also revealed infiltration by leukemia. Patients are resistant to alkylating agents and alpha-interferon (á-IFN). Splenectomy may be beneficial for long-lasting partial responses in some of the patients and is a good palliative treatment.

Clinical score card for diagnosis of group A streptococcal sore throat.

OBJECTIVE: Group A beta hemolytic streptococcus (GAS) sore throat primarily occurs among children in 5-15 years age group, and if not treated appropriately causes rheumatic fever/rheumatic heart disease (RF/RHD). Present study was aimed at validation of a clinical scoring system for diagnosis of GAS. METHODS: Five hundred and thirty six children in 5-15 years age group were enrolled by systematic random selection of households from a peri-urban slum of Chandigarh. They were visited fortnightly at their home for one year to record signs and symptoms of cough and cold. Throat swabs were collected in 918 episodes, of which 123 (13.4%) were GAS culture positive. RESULT: Significant association of GAS was found with pain in the throat, enlarged tonsils, pharyngeal erythema and tender cervical lymphadenopathy. According to the percentage positivity of GAS culture, weighted scores were assigned to age of the child, season of occurrence, fever, size of tonsil, pharyngeal erythema and exudate, lymphadenopathy and pain in throat. Combinations of various symptoms and signs gave sensitivity of 86-89% and specificity of 83-89% whereas clinical score of 15 or more had 91% sensitivity and 98% specificity for diagnosis of GAS pharyngitis. CONCLUSION: As the level of clinical acumen and prevalence of GAS may differ in different primary care settings of the country, the proposed scoring system should be validated and adapted to suit local conditions before establishing it in the primary prophylaxis strategy to prevention of RF/RHD.