Screening for B- and T-cell defects in Egyptian infants and children with suspected primary immunodeficiency.

BACKGROUND: Primary immunodeficiency diseases are underdiagnosed in developing countries. The aim of this study was to identify primary B- and T-cell immune defects in Egyptian infants and children with clinical criteria indicating primary immunodeficiency disease. MATERIAL/METHODS: We enrolled 100 consecutive infants and children clinically suspected to have primary immunodeficiency disease. Subjects were evaluated with respect to immunodeficiency-related score, complete blood count, erythrocyte sedimentation rate, serum immunoglobulin (Ig) A and tetanus IgG antibody estimation, and Candida and tuberculin intradermal testing. Subjects showing IgA deficiency underwent serum total IgG, IgM, and IgE measurement, and lymphopenic patients underwent lymphocyte subset counting by flow cytometry. RESULTS: Thirty-five subjects showed laboratory evidence of T- and/or B-cell immunodeficiency and showed significantly higher immunodeficiency-related scores, a greater frequency of hospitalization, and were more likely to have no bacillus Calmette-Guérin scar and a negative Candida skin test. Laboratory evaluation revealed evidence of predominant B-cell defects in 19 subjects, T-cell defects in 8, and combined immunodeficiency in 8. An immunodeficiency-related score of > or =6 was associated with 71% of the immune defects, and a score > or =8 was predictive of significant immune derangement. The most helpful screening tests for B-cell defects were those for serum IgA and antitetanus IgG, whereas those for T-cell immunodeficiency were peripheral blood lymphocyte count and Candida sensitivity skin test. CONCLUSIONS: Our screening procedures allowed for identification of most of the subjects requiring further evaluation of primary immunodeficiency disease in this study. Widescale screening of neonates and older children for primary immunodeficiency disease is indicated.

Prognostic value of lipoprotein lipase expression among egyptian B-chronic lymphocytic leukemia patients.

BACKGROUND: B-cell chronic lymphocytic leukemia (B-CLL) is a heterogeneous disease with a highly variable clinical course. Some patients may survive for years without need for therapy while others, although they had early treatment, the outcome is unsatisfactory. The motive to find more reliable prognostic factors apart from stage, age, tumor volume and immunoglobulin heavy chain mutations is of clinical interest. MATERIAL AND METHODS: The study was carried out on 25 CLL patients attending Hematology Clinic at Ain Shams University Hospitals. Peripheral blood sample was taken from each patient for surface CD38 and cytoplasmic zeta-chain-associated protein tyrosine kinase (ZAP-70) by flow cytometry and lipoprotein lipase (LPL) expression by real time PCR. RESULTS: We demonstrated statistically significant association between high level of LPL expression and significantly high LDH level, poor cytogenetic risk, ZAP- 70 expression and response to therapy (p=0.01, 0.02, 0.04 and 0.001 respectively). CONCLUSIONS: LPL expression can serve as a new surrogate prognostic factor for CLL patients and can be used to detect patients who need early treatment. KEYWORDS: Lipoprotein lipase - ZAP-70 - Chronic lymphocytic leukemia.