Draz MY Egypt COMMON VARIABLE IMMUNODEFICIENCYP,PID,HYPOGAMMAGLOBULINEMIA

1. بسم الله الرحمن الرحيم

10. Lines of treatment of PIDs The four principal general categories of therapy include: 1-Bone marrow and hematopoietic stem cell transplantation : hematopoietic stem cell transplantation (HSCT) is a very effective treatment for many of PIDs . The initial successful application of stem cell transplantation in 1968 in two patients with Sever combined immuno-deficiency (SCID )gave evidence that a new donor derived immune system can reverse the poor prognosis in many PIDs . 2-Gene therapy: The first true success of human gene therapy was reported with the correction of several patients with X-linked SCID by ex vivo transduction and reinfusion of stem cells with a functional copy of the gamma chain gene by using a retroviral vector (Bonilla and Geha .,2003). Now, all gene therapy using retroviral vectors for immunodeficiency are freezed after the occurrence of T cell leukemia in two of the 10 children administered gene therapy for SCID with IL2RG gene mutation. It was found that the retroviral gene construct of the IL2RG gene inserted itself on the oncogene LMO2 that is aberrantly expressed in acute lymphocytic leukemia of childhood. Thus, insertional oncogenesis was the probable cause of the T-cell leukemia in these two cases .

20. The pattern of B lymphocytes and immunoglobulin level in various types of primary hypogammaglobulinemia (Eley , 2008). Raised IgM ,Low IgG and IgA. Normal 6-HIGM Normal Normal 5-Specific antibody Deficiency. Normal Normal 4-Isolated IgG Deficiency. Low IgG, IgA , and IgM. Absent/low 3-X-linked and autosomal recessive agammaglobulinemias Low IgG and IgA, Normal/low IgM. low 2-CVID Low IgA ,Normal IgG, IgM. Normal 1-Selective IgA. Immunoglobulins B cells Disease

33. The stages of B cells development and the defect in CVID and IgAD which occurs in late stages during activation and differentiation of B cells into plasma cells ( Kokron et a.,2004).

41. الحمد لله رب العالمين و شكرا لأساتذتي و السادة الحضور

42. الحمد لله رب العالمين و شكرا لأساتذتي و السادة الحضور