Chediak-Higashi syndrome

i.e. (Disorders of phagocytosis): (159-Ananthanarayan 8th)CHEDIAK - HIGASHI SYNDROME: This is a genetic disorder characterised by decreased pigmentation of skin, eyes and hair, photophobia, nystagmus and giant peroxidase positive inclusions in the cytoplasm of leucocytes. The inclusion may be the result of autophagocytic activity. The leucocytes possess diminishedphagocytic activity. Patients suffer from frequent and severe pyogenic infectionsClassification of Primary immunodeficiency syndromeA. Disorders of specific immunityI. Humoral immuno deficiencies (B-cell defects)a. X-linked agammaglobulinemiab. Transient hypogammaglobulinemia of infantsc. Common variable immunodeficiencies (late onset hypogamma globulinemia)d. Selective immunoglobulin deficiencies (IgA, IgM or IgG subclasses)e. Immunodeficienceis with hyper-IgMf. Transcobalamin II deficiencyII. Cellular immunodeficiencies (T-cell defects)a. Thymic hypopolasia (Digeorge syndrome)b. Chronic mucocutaneous candidiasisc. Purine nucleoside phosphorylase (PNP) deficiencyIII. Combined immuno deficiency (B and T cell defects)a. Cellular immuno deficiency with abnormal immunoglobulin synthesis (Nezel of Syndrome)b. Ataxia - telangiectasiac. Wiskott - Aldrich syndromed. Immuno deficiency with thymomae. Immuno deficiency with short -limbed dwarfismf. Episodic lymphopenia with lymphocytotoxing. Severe combined immuno deficiencies1. 'Swiss type' agammaglobulinemia2. Reticular dysgenesis of de Vail3. Adenosine deaminase (ADA) deficiency**B. Disorders of complementa. Complement component deficienciesb. Complement inhibitor deficienciesC. Disorders of phagocytosisa. Chronic granulomatous diseaseb. Myeloperoxidase deficiencyc. Chediak - Higashi syndromed. Leucocyte G6PD deficiencye. Job's syndromef. Tuftsin deficiencyg. Lazy leucocyte syndromeh. Hyper - IgE syndromei. Actin - binding protein deficiencyj. Shwachman's diseaseSecondary Immuno deficiencies - arise when infections (HIV), drugs (immuno suppressive agents, corticosteroids), nutritional inadequacies (Kwashiorkor), metabolic disorders (protein losing enteropathies) and malignancies (CLL, Multiple myeloma, Hodgkin's disease) leads to defects in specific and nonspecific immunity.