Immunologic deficiency syndromes

987 views
846 views

Published on

Published in: Health & Medicine
0 Comments
2 Likes
Statistics
Notes
  • Be the first to comment

No Downloads
Views
Total views
987
On SlideShare
0
From Embeds
0
Number of Embeds
9
Actions
Shares
0
Downloads
71
Comments
0
Likes
2
Embeds 0
No embeds

No notes for slide

Immunologic deficiency syndromes

  1. 1. IMMUNOLOGIC DEFICIENCY SYNDROMES
  2. 2. PRIMARY IMMUNODEFICIENCY <ul><li>mainly B-cell defect : X-linked agammaglobulinemia of Bruton, transient hypogammaglobulinemia of infancy, selective IgA deficiency, common variable immunodeficiency (CVID) </li></ul><ul><li>mainly T-cell defect : DiGeorge syndrom (thymic hypoplasia), hyper-IgM syndrome </li></ul><ul><li>B- and T-cell defect : severe combined immunodeficiency (SCID), Wiskott-Aldrich syndrome (immunodeficiency with thrombocytopenia and eczema </li></ul><ul><li>defect in phagocyte function : chronic granulomatous disease, leukocyte adhesion deficiency, myeloperoxidase deficiency </li></ul><ul><li>primary complement deficiencies </li></ul>
  3. 3. SECONDARY IMMUNODEFICIENCY <ul><li>due to impaired synthesis and function: protein and energy deficiency in malnutrition, cachexia in disseminated cancer, anorexia, alcoholism, prevalent monoclonal Ig in some lymphoproliferative diseases, suppression of cell mediated immunity due to acute viral infection (CMV, EBV, measles, etc.), bacterial and protozoal infection (leprosy, leishmaniasis), iatrogenic (immunosuppressive and cytostatic drugs, radiotherapy, splenectomy), diabetes mellitus and other metabolic diseases, sarcoidosis, chronic stress, certain age groups (old, newborn, immature infants) </li></ul><ul><li>increased catabolism or loss: nephrotic syndrome and renal failure, inflammatory intestinal diseases </li></ul>
  4. 4. HIV - AIDS
  5. 5. HIV - AIDS <ul><li>The global percentage of adults living with HIV has leveled off since 2000. </li></ul><ul><li>In 2007: 2.7 million new HIV infections, 2 million deaths. </li></ul><ul><li>The rate of new HIV infections: fallen in several countries, but globally these favourable trends are at least partially offset by increases in new infections in other countries. </li></ul><ul><li>In 14 of 17 African countries with adequate survey data, the percentage of young pregnant women (ages 15–24) living with HIV has declined since 2000-2001. </li></ul>
  6. 6. HIV - AIDS <ul><li>Sub-Saharan Africa 67% of all people living with HIV and for 75% of AIDS deaths in 2007. </li></ul><ul><li>Problems in other regions - Indonesia, the Russian Federation, various high-income countries. </li></ul><ul><li>Globally, the percentage of women stable (at 50%), increasing in several countries. </li></ul><ul><li>In regions outside sub-Saharan Africa, HIV disproportionately affects injecting drug users, men who have sex with men, and sex workers. </li></ul>
  7. 7. HIV - AIDS <ul><li>Globally estimated: 33 million people living with </li></ul><ul><li>HIV in 2007. </li></ul><ul><li>The annual number of new HIV infections </li></ul><ul><li>declined from 3.0 million in 2001 to 2.7 million </li></ul><ul><li>in 2007. </li></ul><ul><li>Overall, 2.0 million people died due to AIDS in 2007, compared with an estimated 1.7 million in 2001 </li></ul>
  8. 8. HIV - AIDS <ul><li>Young people aged 15–24: 45% of new HIV infections worldwide. </li></ul><ul><li>370 000 children younger than 15 years became infected with HIV in 2007. </li></ul><ul><li>Globally, the number of children younger than 15 years living with HIV increased from 1.6 million in 2001 to 2.0 million in 2007. </li></ul><ul><li>Almost 90% live in sub-Saharan Africa </li></ul>
  9. 9. HIV - AIDS <ul><li>More than 90% of children living with HIV acquired the virus during pregnancy, birth or breastfeeding—forms of HIV transmission that can be prevented. </li></ul>
  10. 10. Southern Africa <ul><li>35% of HIV infections </li></ul><ul><li>38% of AIDS deaths in 2007 </li></ul><ul><li>sub-Saharan Africa :67% of all people living with HIV. </li></ul>
  11. 11. Sub-Saharan Africa <ul><li>In countries with high HIV prevalence, life expectancy at birth has dramatically fallen. </li></ul><ul><li>Southern Africa: average life expectancy at birth ↓ to levels in the 1950s; now below 50 years for the whole subregion, </li></ul><ul><li>below 40 years in Zimbabwe </li></ul>
  12. 12. Asia <ul><li>5.0 million living with HIV in 2007, </li></ul><ul><li>380 000 newly infected that year. </li></ul><ul><li>380 000 died from AIDS-related illnesses. National HIV infection levels are highest in South-East Asia </li></ul>
  13. 13. Eastern Europe and Central Asia <ul><li>The number of people living with HIV rose to 1.5 million in 2007; </li></ul><ul><li>almost 90% of those in Russian Federation (69%) or Ukraine (29%). </li></ul><ul><li>110 000 infected with HIV in 2007 </li></ul><ul><li>58 000 died of AIDS </li></ul>
  14. 14. Latin America <ul><li>New HIV infections in 2007: 140 000 </li></ul><ul><li>1.7 million living with HIV </li></ul><ul><li>63 000 died of AIDS last year. </li></ul>
  15. 15. North America, Western and Central Europe <ul><li>2.0 million (USA: 1.2 million) people living with HIV in 2007. </li></ul><ul><li>81 000 newly infected with HIV in 2007. </li></ul><ul><li>31 000 died of AIDS last year. </li></ul>
  16. 16. Middle East and North Africa <ul><li>limited HIV information </li></ul><ul><li>approximately 380 000 living with HIV in 2007, </li></ul><ul><li>40 000 newly infected </li></ul>
  17. 17. HIV ISSUES <ul><li>Blood safety </li></ul><ul><li>HIV treatment: antiretroviral therapy </li></ul><ul><li>Prevention of mother-to-child transmission </li></ul><ul><li>Co-management of tuberculosis and </li></ul><ul><li>HIV treatment </li></ul><ul><li>HIV testing in the general and most-at-risk population </li></ul>
  18. 18. HIV ISSUES <ul><li>Most-at-risk populations: Prevention </li></ul><ul><li>programmes </li></ul><ul><li>HIV education </li></ul>
  19. 22. HIV infection of cells <ul><li>T-lymphocytes </li></ul><ul><li>macrophages/monocytes (viral reservoir, replication and transport) </li></ul><ul><li>mucosal and follicular dendritic cells </li></ul><ul><li>cells in CNS (microglia) </li></ul>
  20. 23. Immune dysfunctions in AIDS <ul><ul><li>Lymphopenia (selective loss of CD4+ T-cells) </li></ul></ul><ul><ul><li>Decreased T-cell function in vivo (loss of memory T-cells, susceptibility to opportunistic infections and neoplasms, decreased delayed-type hypersensitivity) </li></ul></ul><ul><ul><li>Altered T-cell function in vitro </li></ul></ul><ul><ul><li>Polyclonal B-cell activation (hypergammaglobulinemia, CIC, inability of new antibody response) </li></ul></ul><ul><ul><li>Altered monocyte or macrophage functions (decreased chemotaxis, phagocytosis, antigen presentation; increased spontaneous secretion of TNF, IL-1 etc. </li></ul></ul>
  21. 24. Phases of HIV infection <ul><li>Acute retroviral syndrome (3-6 wks after infection, in 40-90%, self-limited in 2-4 wks) </li></ul><ul><li>Chronic phase (clinical latency, persistent generalized lymphadenopathy – PGL) </li></ul><ul><li>Progression to AIDS (AIDS-related complex – ARC, AIDS indicator conditions: constitutional, neurologic, opportunistic infection, neoplasm </li></ul>
  22. 25. Persistent generalized lymphadenopathy
  23. 26. HIV neurologic disease <ul><li>Acute aseptic meningitis </li></ul><ul><li>subacute and chronic: HIV meningoencephalitis – AIDS-dementia complex, vacuolar myelopathy, myopathy and peripheral neuropathy </li></ul>
  24. 27. HIV encephalopathy – brain atrophy
  25. 28. HIV encephalopathy
  26. 29. HIV encephalitis
  27. 30. p24 immunohistochemistry
  28. 31. Opportunistic infections and neoplasms <ul><li>Protozoal and helmintic (cryptosporidiosis, toxoplasmosis, giardiosis, etc.) </li></ul><ul><li>Fungal (Pneumocystis, candidiasis, cryptococcosis, coccidiomycosis, histoplasmosis) </li></ul><ul><li>Bacterial (mycobacteriosis – atypical, TB, salmonellosis, nocardiosis) </li></ul><ul><li>Viral (CMV, Herpes simpex, Varicella-zoster, progressive multifocal leukoencephalopathy – JC polyoma virus) </li></ul><ul><li>Neoplasms ( Kaposi sarcoma – HHV 8, B-cell non-Hodgkin lymphomas, primary brain lymphomas – EBV, aggressive cervical and anal carcinomas – HPV) </li></ul>
  29. 32. CNS infections
  30. 33. Toxoplasma encephalitis
  31. 34. Toxoplasma encephalitis
  32. 35. Toxoplasma encephalitis
  33. 36. Cryptococcal meningitis
  34. 37. Cryptococcal meningitis
  35. 38. Cryptococcal meningitis
  36. 39. Cryptococcal meningitis
  37. 40. PML
  38. 41. PML
  39. 42. PML
  40. 43. LUNG INFECTIONS
  41. 44. Pneumocystis pneumonia
  42. 45. Pneumocystis pneumonia
  43. 46. TBC
  44. 47. Fungal colony
  45. 48. Fungal pneumonia
  46. 49. Histoplasmosis
  47. 50. Interstitial pneumonia
  48. 51. GIT INFECTIONS
  49. 52. Erosive gastritis
  50. 53. Haemorrhagic colitis
  51. 54. CMV colitis
  52. 55. CMV colitis
  53. 56. Coccidiomycosis
  54. 57. Coccidiomycosis
  55. 58. Protozoan colitis (amoebiasis)
  56. 59. Protozoan colitis (amoebiasis)
  57. 60. Mycobacterium avium complex (MAC) enteritis
  58. 61. MAC enteritis
  59. 62. MAC enteritis
  60. 63. Bacterial thrombus
  61. 64. SKIN INFECTIONS
  62. 65. Oral hairy leukoplakia
  63. 66. HSV
  64. 67. HIV-associated neoplasia <ul><li>HHV-8: Kaposi‘s sarcoma </li></ul><ul><li>EBV: non-Hodgkin‘s malignant lymphoma, primary brain ML </li></ul><ul><li>HPV: agressive anal, cervical squamous cell carcinoma </li></ul>
  65. 68. Kaposi‘s sarcoma
  66. 69. Kaposi‘s sarcoma
  67. 70. Kaposi‘s sarcoma
  68. 71. Kaposi‘s sarcoma
  69. 72. Kaposi‘s sarcoma
  70. 73. Kaposi‘s sarcoma + CMV colitis
  71. 74. Primary brain malignant lymphoma
  72. 75. Primary brain malignant lymphoma
  73. 76. HPV – koilocytosis - LSIL
  74. 77. HPV - immunohistochemistry
  75. 78. Invasive squamous cell carcinoma
  76. 80. Cervical squamous cell carcinoma

×