B cell T cell deficienciesPresentation (16) (2).pdf

Information about B cell and T cell

1. B CELL & T CELL
DEFICIENCIES
Presented By : Anjali Ninama
Anjana Bhabhor
S.Y bsc nursing
GINERA

2. INTRODUCTION

3. What are B cells ?
•These cells are originate from stem cells
in the bone marrow and mature in bone
marrow.
•It also called B lymphocyte.

4. •B cells are involved in humoral
response.
•They connect to the surface of
invading bacteria & virus.
•Life span : Short ( 4-7 weeks ).

5. What are T cells ?
•T cells originate in the bone marrow and
mature in the thymus.
•These can be further divided into
1.T Helper Cells 3.Regulatory T Cells
2.T Cytotoxic Cell. 4.Memory T Cells

6. •They are responsible for removing the
pathogens from the body.
•T cells produce cytokines.
•They connect only to the virus antigen.

7. •Life span : long ( 9 years ).
•As soon as the foreign antigen enters
the cells , T cells trigger the B cells to
develope palsma cells and activates T
killer cells that kill the cells affected
by the invaders.

8. B CELL
DEFICIENCIES

9. •B cell deficiencies are inherited.
•It has two types
( i ) Lack of differentiation of B cells
precursors into mature B cells.
( ii ) B cell deficiency due to lack of
differentiate of B cells into plasma cells.

10. 1.Sex linked agammaglobulinemias (
Bruton’s disease )
-It is caused by BTN ( bruton
tyrosinekinase) gene mutation on x
chromosome.
-X linked a gammaglobulinemia is more
common in males if they have affected
male relative

11. -ImmunoglobulinsIgG,IgM,IgA,IgD,IgE
andBcellsareloworabsentinperipheral
blood.
-In this disease,all antibodies
disappear from plasma of the protien.

12. 1. Hypogammaglobulinemia :
-It is also called common variable
immuno deficiency disease ( CVID ).
-This B cells deficiency result for lack
of differentiation of B cell into plasma
cells..

13. -Diminished antibody production.
-CVID incorporate variety of defects
ranging from immunoglobulin A
deficiency.

14. 2. Panhypoglobulinemia :
-General lack of immunoglobulins ( IgG
, IgA , IgM ) in blood.

15. CLINICAL MANIFESTATIONS
•Sex-linked agammaglobulinemia
-Recurrent pyogenic infections.

16. •Hypogammaglobulinemia
-Autoimmune Diseases : Rheumatoid
Arthritis , Hypothyroidism
-Lymphoid hyperplasia of small intestine ,
spleen.
-Giardia lamblia infection.
-Pernicious anemia

17. DIAGNOSIS
•History of patient taken regarding
recurrent bacterial infections.
•Serum immunoglobulins test.

18. •Serology test.
•CBC test.
•Biopsy.

19. MEDICAL MANAGEMENT
1.Intravenous immunoglobulin.
2.Antimicrobial Treatment.
3.Metronidazol.

20. 4.Vitamin B12 injections.
5.Postural drainage.

21. T CELL
DEFICIENCIES

22. •T cell deficiencies are genetic disorders.
•T cells play a regulatory role in immune
system function.
•T cells loss of function occur along with
loss of B cell function.
•T cells deficiency persons have more
susceptibility to infections.

23. 1.Thymic Hypoplasia (
Digeorge Syndrome )
-It is a primary T cell immuno
deficiency disease.

24. •Pathophysiology
-Genetic condition : Chromosome 22
deletion.
-Thymus gland fails to develop normally
during embryogenesis which lead to T cell
deficiency.

25. CLINICAL MANIFESTATION
•Characteristic
facial appearance
: long face,small
teeth,broad nose.

26. •Developmental
abnormalities(
eg.cleft palate
).

27. •Cardiac defects.
•Thymus gland abnormalities.
•Hypoparathyroidism →
Hypocalcemia →Seizures , tetany ,
osteoporosis.

28. 2.Chronic Mucocutaneous
Candidiasis
-This T cells disorder associated with
selective defect in T cell immunity.
-It is caused by an autosomal recessive
inheritance.

29. -This disease occur with or without
endocrinopathy.
-This autoimmune disorder involve thymus
and other endocrine glands.

30. CLINICAL MANIFESTATION
•Chronic
candidiasis
infection of
mucosa
membrane,
skin & nails.

31. •Tetany.

32. •Hypocalcemia.
•Addison’s disease.

33. DIAGNOSIS
•T cell count evaluted
•Comprehensive immunologic analysis.
•FISH analysis ( For fluorescent in situ
hybridization ).

34. MEDICAL MANAGEMENT
1.Oral calcium supplements along with
vitamin D.
2.Surgical Intervention.

35. •3.Thymus graft : fetal thymus ,
postnatal thymus or humun leukocyte
antigen( HLA ) matched bone
marrow used for permanent
reconstitution of T cell immunity.

36. 4.IVIG therapy.
5.Chronic mucocutaneous candidiasis
antifungal agents.
-Miconazole ( Topical ).
-Clotrimazole & ketoconazole ( orally ).
-IV amphotericin B.

38. COMBINED B CELL AND
T CELL DEFICIENCIES

39. •Combined B cell and T cell deficiencies are
comprise a heterogenous group of disorders.
•Autosomal recessive and x linked conditions
present in these diorder.

40. ATAXIA
TELANGIECTASIA

41. •It is autosomal neurodegenerative
disorders.
•It is caused by mutations in gene on
chromosome 11 known the Ataxia
Tetagiectasia mutation.
•Rare inherited disorders.

42. •This disease is associated with neurological
vascular,endocrine,hepatic and cutaneous
abnormalities.
•It is accompained by progressive cerebellar
ataxia,telangiectansias,recurrent bacterial
infection of sinues and lungs and increase
incidence of cancer.

43. •Lack of coordination
•Eye movement abnormalities
•Difficulty walking and poor balance
•Swallowing difficulty
CLINICAL MANIFESTATIONS

44. •Jerky movement

45. •Discoloration of skin

46. •Chronic lung disease.
•Vision impairment.
•Speech impairment.

47. •History collection
•Physical examination
•Blood tests
DIGNOSTIC METHODS

48. •Magnetic resonance imaging (MRI)
•Karyotyping

49. •Antimicrobial therapy.
•Postural drainage and physical
therapy for the treatment of chronic
lung disease.
MEDICAL MANAGEMENT

50. •Intravenous Immunoglobuline
administration.
•Vaccination with influenza and
pneumococcal may be help.

51. SEVERE COMBINED IMMUNO
DEFICIENCY DISEASE

52. •Rare disorder.
•It is caused by mutation in differents
genes.
•These defects are characterised by
early onset of infection,defect in B
cell and T cell systems,lymphoid
aplasia,thymic dysplasia.
•This is x linked autosomal recessive.

53. •Autosomal recessive SCID is caused
by adenosine deaminase deficiency.
•In which infants lake the ADA enzyme
necessary for T cell survival.
•X linked SCID caused by mutations in
gene on the X chromosome.
-primarily affects male infants.

54. •Failire to gain weight
•Chronic diarrhea.
•GI infection
•Respiratory infection & pneumonia.
CLINICAL MANIFESTATIONS

55. •Vomitting.
•Fever.
•Frequent infection.
•Oral fungal infection.

56. •History collection
•Physical examination
•Blood test
•SCID screening
DIGNOSTIC METHODS

57. MEDICAL MANAGEMENT
•Stem cell & bone marrow
transplantation.
•IVIG administration can be given.
•Thymus gland transplantation.

58. WISKOTT ALDRICH
SYNDROME

59. •Wiskott aldrich syndrome is an
inherited imunodeficiency caused by
mutation in the WAS gene,which
provide instruction for production of a
protein called WASp.
•It reduced ability to form blood clots.

60. •Characteristics of this disease are
frequent infections,thrombocytopenia
with small platelets,eczema and
increased risk for autoimmune
disorders.

61. CLINICAL MANIFESTATIONS
•Vasculitis
•Anaemia
•Hay fever
•Swollen joints

62. •Swelling of lymph nodes .
•Kidney inflammation.
•Diarrhoea.

63. DIGNOSTIC METHODS
•Complete Blood Count ( CBC ) Test.
•Genetic test that reveals presence of a
mutation in the wiskott aldrich
syndrome.

64. MEDICAL MANAGEMENT
•Antimicrobial Therapy
-Microbiocidal Therapies
-Microbiostatic Therapies
•Intravenous Immunoglobulin (IVIG)
Infusion

66. •Bone marrow transplantation.
•For prophylaxis-continuous
antibiotic therapy given.
•For autoimmune diseases
-High dose of immunosuppressive
& steroids can be given.

67. NEZELOF SYNDROME

68. •It is an autosomal recessive congenital
immunodeficiency condition due to
underdevelopment of the thymus.
•It is characterized by severe
infections,lymhopenia,diminished
lymphoid tissue and agenesis of
thymus.

69. •Chronic diarrhoea.
•Hepatosplenomegaly.
•Bronchopulmonary
infections.
CLINICAL MANIFESTATIONS

70. •Eczematoid rash ( Atopic Dermatitis ).

71. •Pyoderma. •Monilia infection.

72. DIGNOSTIC METHODS
•Complete Blood Count ( CBC ) Test.
•X-ray of thymus.

73. MEDICAL MANAGEMENT
•Antimicrobiology therapy.
•IV immunoglobulins can be
administered.
•Bone marrow & thymus transplantations
can be done.

74. NURSING MANAGEMENT
•Nursing Diagnosis
1.Impaired gas exchnage related to altered
delivery of inspired oxygen as evidenced by
facial expression.
2.Anxiety related to change in health status as
evidenced by facial expression.

75. 3.Fatigue & activity intolerance related
to anaphylaxis.
4.Risk for infection related to
immunodeficiency.
5.Deficient knowledge related to
unfamiliarly with information resources
as evidenced by verbalizing inaccurate
information.

76. •Nursing interventions.
-Hand washing should be done to entering in
the room & touching the patient and prior to
any procedure.
-Check the weight of patient prior to
treatment.
-Check vital signs before,during and after IV
infusion of IVIG.
-Monitor lab values.

77. -Assess nutritional status.
-Be careful about reactions .
-Always monitor the patient in clinical
setting for infection.
-Monitor all haemodynamic function
of patient to assess risk of secondary
infectionswhich can be fatal.