Immunodeficiencies. Immunotherapy. Immunoprophylaxis. Immunoreabilitation

1. Immunodeficiencies. Immunotherapy.
Іmmunoprophylaxis.
Іmmunoreabilitation.

5. Sponsored
Medical Lecture Notes – All Subjects
USMLE Exam (America) – Practice

10. • The most common ID diseases
• Wide range of defects: complete absence
of B cells or only absence of Ab
• Patients suffer from mainly encapsulated
bacterial infections
I. B cell deficiency

12. X-linked agammaglobulinemia
(Bruton’s agammaglobulinemia)
 Common in male infants
 Immune defect: absence of mature B cells in
lymphoid tissue and blood, no tonsils, small
lymph nodes, low or no serum Ig

13. • Specific abnormality: blockage in maturation of
pre-B cells to B cells (cytoplasm Tirosine kinase)
• Infants protected up to 6 months, then manifest
recurrent bacterial
infections
• Treatment: life-long periodic gamma-globulin
injections

14. Common variable hypo-gamma-globulinemia
 Affect both sexes, adults (late onset, 15-35 yrs),
familial incidence but no definite genetic basis
 Immune defect: low serum Ig, low plasma cell
level, abnormal function of B cells but normal
quantity of B cells in blood
 Specific abnormality: defective differentiation of
B cells into plasma cells
 Treatment: periodic gamma-globulin injections

15. Selective deficiencySelective deficiency of IgАof IgА
1)1)transient; 2) persistent; 3) asymptomatictransient; 2) persistent; 3) asymptomatic
Clinical features:Clinical features: frequent respiratory infections,frequent respiratory infections,
purulents bacterial processes on a skin, mucouspurulents bacterial processes on a skin, mucous
membranes of mouth, eyes, atopic syndrome:membranes of mouth, eyes, atopic syndrome:
asthmatic bronchitis, bronchial asthma, diffuseasthmatic bronchitis, bronchial asthma, diffuse
neurodermatitis, food allergyneurodermatitis, food allergy..

16. II. T cell deficiency
• defects in cell-mediated immunity
lead to defects in humoral-
mediated immunity
• Defects in CMI lead to
opportunistic infections (viruses,
fungi, parasites, intracellular bact.)

17. The syndrome is a spectrum of
anomalies that include thymic
hypoplasia, resulting in T-cell
deficiency, hypoparathyroidism,
facial dysmorphism, and
cardiac defects. Primary
hypoparathyroidism manifests as hypocalcimic tetany.
CONGENITAL THYMUS APLASIA (DIGEORGE SYNDROME)
Characteristic genetic violations: wide bridge
of the nose, magnify distance between pupils,
antimongoloid slant, low planted ears,
deformation of auricles, underdeveloped lower
jaw, cleft palate.

18. III. Combined deficiencies
• defects in cell-mediated and
humoral-mediated immunities

19. Severe Combined
Immunodeficiency (SCID)
• Immune defect: low count of T & B cells
• Specific abnormality: defect in
lymphoid stem cell differentiation or
defect in developmental pathways in
thymus & bone marrow
• Most severe form, associated with
recurrent fatal infections early in life.
 Treatment: bone marrow transplantation (or die
within 2 yrs), gene therapy.

20. Immunodeficiency with Ataxia-Telangiectasia
(Louis-Bar syndrome)
is characterized by a clinical triad
of symptoms:
- progressive immunodeficiency,
- cerebellar ataxia,
- oculocutaneous telangiectasia.
This progressive immunodeficiency
leads to severe bouts of sinusitis and
lung infection (so-called sinopulmonary
syndrome). Therapy is symptomatic.

21. IV- Complement deficiency
Defect in:
C1-C9 components can result in defective
opsonisation of bacteria, recurrent infections
by Neisseria spp., immune complex diseases
(e.g. SLE-like)

22. 1) deficiencyof early components of
classical pathway
2) deficiency of late components of
complement (С5, С6, С8, С9)
3) deficiency of complement inhibitor
Classification of complement deficiencycomplement deficiency

23. V- Defects in phagocytes
Defects associated with myeloid
cells (monocytes/neutrophils)

24. 1. Leukocyte adhesion deficiency
• Immune defect: adherence defect in
leukocyte
• Specific abnormality: Defective adhesion
molecules consequences:
Failure of inflammatory cells to migrate
Defective Phagocytosis of opsonised
bacteria
Defective T cell help in B cell activation
Defective CTL killing

25. 2. Chronic granulomatous disease (CGD)
Immune defect: defective killing of intracellular
bacteria by phagocytes
Specific abnormality: Phagocytes fail to produce
Oxygen intermediates (O2
-
, H2O2)

26. Associated with recurrent bacterial
& fungal infections, disseminated
granulomas, children die of
septicemia by 7 years of age
Treatment: Antibiotics,
Bone marrow transplantation

28. Acquired Immunodeficiency Disease
(AIDS)
 Causative agent: HIV
 Target cells: CD4+ T cells,
monocytes, macrophages,
dendritic cells, etc.
 Importance of gp120
 Is divided 4 types: 1, 2, 3 and 4
 present at all tissues: sperm, saliva,
sweat, earwax, excrement

29. Prevalence of HIV (2001)

30. Prevalence of HIV in Africa (2003)

31. показник понад 90
показник від 41 до 90
показник до 40
середній показник 155,17
Prevalence of HIV in Ukraine (2004)

32. Prevalence of HIV (2005)

33. HIV-infection

34. The life cycle of HIV
1
2
3
4
5
6
7

36. Channel of infection:
- sexual
- parenteral
- vertical
Risk of infection:
- at a contact with blood and its
preparations – 100%;
- transplacental infection – 15-20%;
- at a breast feeding – 30-40%.

37. Disease has several stages:
1. Asymptomatic for years (latency)
2. AIDS with fever, weight loss, major opportunistic infections,
malignancies, when number of CD4+ cells is low.
The most early manifestation of acute HIV-
infections is a flu-like syndrome (at 40-70%
patients).
Symptoms of flu-like syndrome:
fever, lymphadenopathy, pharyngitis,
cough, myalgia, arthralgia, headache
and retroorbital pain, anorexia, nausea,

39. Diagnosis: detection of viral Ag, anti-
viral Ab
Prognosis: CD4/CD8 ratio

40. Herpes simplex - herpesvirus 1 type

41. (Herpes zoster – herpesvirus 3 type)

42. Candidosis of a buccal cavity

43. Kaposi’s sarcoma

44. Non-Hodgkin's lymphomas

45. Treatment:
1. Treatment of microbial infections
2. Anti-viral drugs
3. Immunorestoration: BM transplantation, Ig
injections, cytokines
Groups of preparations:
Inhibitors of reverse transcriptase:
а) nucleoside;
б) non-nucleoside.
2. Protease Inhibitors.
3. Fusion or Entry Inhibitors.

46. Inhibitors of reverse transcriptase (nucleoside)

47. Inhibitors of reverse transcriptase (nucleoside)

48. Inhibitors of reverse transcriptase
( nonnucleoside)
Fusion or Entry Inhibitors

49. Protease Inhibitors

50. • immunopotentiation;
• immunosuppression;
• immunomodulatory;
• immunosubstitutive;
• immunorehabilitation.
Immunotherapy

51. Immunothropic drugs
1. Preparations of microbal origin (BRONCHO-
MUNAL, IRS-19, ribumunyl).
2. Preparations of plants (Immunal, Echinacea).
3. Peptid preparations (Immunofan, Tactivinum).
4. Cytokines and preparations containing of
cytokines
4.1. Preparations on the basis of interferons
(intron, Interferon alfa2a).
4.2. Inductors of interferons (arbidol,
Cycloferonum).

52. 4.3. Preparations containing interleukines
(Roncoleukin).
4.4. Preparations containing the tumour necrosis
factor (Hebertrans).
5. Synthetic preparations (Galavitum,
levamisole).
6. Preparations containing natural components
6.1. Preparations of immunoglobulins (Biaven,
Sandoglobinum).
6.2. Immunoglobulins, containing the antibodies
classes of Ig M (Pentaglobin).
6.3. Specific Immunoglobulins (Cytotect).

53. Immunoprophylaxis
Active
vaccination
Passive
antibody:
Immunoglobulins
Serums

54. ImmunoImmunorehabilitationrehabilitation
- dosed physical loadings,
- removals of harmful habits,
-metabolic and antioxidative therapy,
- vitaminotherapy,
-stimulation of specific and nonspecific immunity,
- vaccination et cetera.