T CELL DEFFICIENCIES
• DEFECTS IN T CELLS LEAD TO
OPPORTUNISTIC INFECTIONS.
• GENETIC IN ORIGIN
• INCREASED SUSCEPTIBILITY TO
INFECTION
• S/S DEPENDS UPON THE TYPE OF
T CELLS AFFECTED
• 1. THYMIC HYPOPLASIA[
DIGEORGE SYNDROME]
• ABSENCE OF SEVERAL GENES
ON CHROMOSOME 22
• THYMUS GLAND FAILS TO
DEVELOP NORMALLY DURING
EMBRYOGENESIS
• SYMPTOMS PRESENT ALMOST
SOON AFTER BIRTH
• SIGN AND SYMPTOM
• HYPOTHYROIDISM WITH
HYPOCALCEMIA
• CONGENITAL HEART DISEASE
• RENAL ABNORMALITIES
• ABNORMAL FACES
• TETANY
• CONVULSIONS
• DIAGNOSIS
• T CELLS EVALUATION-
PERIPHERAL BLOOD
LYMPHOCYTE COUNT
• WILL FIND LYMPHOPENIA
• MANAGEMENT
• THYMUS TRANSPLANTATION
• HUMAN LEUKOCYTE ANTIGEN
MATCHED BONE MARROW TO THE
RECONSTITUTION OF
PERMANENT T CELL IMMUNITY
• 2. CHRONIC MUCOCUTANEOUS
CANDIDIASIS
• AUTOSOMAL RECESSIVE
INHERITANCE
• AUTOIMMUNE DISORDER IN
WHICH THE THYMUS AND OTHER
ENDOCRINE GLANDS ARE
INVOLVED IN AUTOIMMUNE
PROCESS
• EXTENSIVE MORBIDITY DUE TO
• S/S
• CHRONIC CANDIDAL INFECTION
• HYPOCALCEMIA
• TETANY SECONDARY TO THE
HYPOFUNCTION OF THE
PARATHYROID GLAND
• ADDISON’S DISEASE
• DIAGNOSIS IS DONE BY THE
DERMAL SENSITIZATION OF THE
PATIENT
•
• MANAGEMENT-
• ANTIFUNGAL AGENT
• IVIG

T cell defficiencies

  • 1.
  • 2.
    • DEFECTS INT CELLS LEAD TO OPPORTUNISTIC INFECTIONS. • GENETIC IN ORIGIN • INCREASED SUSCEPTIBILITY TO INFECTION • S/S DEPENDS UPON THE TYPE OF T CELLS AFFECTED
  • 3.
    • 1. THYMICHYPOPLASIA[ DIGEORGE SYNDROME] • ABSENCE OF SEVERAL GENES ON CHROMOSOME 22 • THYMUS GLAND FAILS TO DEVELOP NORMALLY DURING EMBRYOGENESIS • SYMPTOMS PRESENT ALMOST SOON AFTER BIRTH
  • 4.
    • SIGN ANDSYMPTOM • HYPOTHYROIDISM WITH HYPOCALCEMIA • CONGENITAL HEART DISEASE • RENAL ABNORMALITIES • ABNORMAL FACES • TETANY • CONVULSIONS
  • 5.
    • DIAGNOSIS • TCELLS EVALUATION- PERIPHERAL BLOOD LYMPHOCYTE COUNT • WILL FIND LYMPHOPENIA
  • 6.
    • MANAGEMENT • THYMUSTRANSPLANTATION • HUMAN LEUKOCYTE ANTIGEN MATCHED BONE MARROW TO THE RECONSTITUTION OF PERMANENT T CELL IMMUNITY
  • 7.
    • 2. CHRONICMUCOCUTANEOUS CANDIDIASIS • AUTOSOMAL RECESSIVE INHERITANCE • AUTOIMMUNE DISORDER IN WHICH THE THYMUS AND OTHER ENDOCRINE GLANDS ARE INVOLVED IN AUTOIMMUNE PROCESS • EXTENSIVE MORBIDITY DUE TO
  • 8.
    • S/S • CHRONICCANDIDAL INFECTION • HYPOCALCEMIA • TETANY SECONDARY TO THE HYPOFUNCTION OF THE PARATHYROID GLAND • ADDISON’S DISEASE
  • 9.
    • DIAGNOSIS ISDONE BY THE DERMAL SENSITIZATION OF THE PATIENT • • MANAGEMENT- • ANTIFUNGAL AGENT • IVIG