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Shashidhar Venkatesh Murthy
A/Prof & Head of Pathology
College of Medicine & Dentistry
Congenital WBC Disorders
2
Congenital WBC disorders:
 Leucocytosis:
 Idiopathic Neutrophilia. Increased neutrophils. Asymptomatic.
 Leucopenia:
 Ethnic or Benign familial Neutropenia. (Jews, Blacks & Arab)
 Cyclical Neutropenia. – cyclical variation in WBC counts.
 Kostmann’s syndrome: Congenital agranulocytosis – lack of G-CSF
severe neutropenia & severe infections.
 Felty’s syndrome: Rheumatoid arthritis & severe neutropenia.
 Severe Combined Immunodeficiency: absent T Lymphocytes.
 WBC Function disorders:
 Chronic Granulomatous Disease:
Defective microbicidal function.
 Lazy leukocyte syndrome.
“Everyone is a genius. But if you
judge a fish by its ability to climb a
tree, it will spend its whole life
believing it is stupid…!”
- Albert Einstein

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Congenital wbc disorders

  • 1. 1 Quick PATH Bytes Shashidhar Venkatesh Murthy A/Prof & Head of Pathology College of Medicine & Dentistry Congenital WBC Disorders
  • 2. 2 Congenital WBC disorders:  Leucocytosis:  Idiopathic Neutrophilia. Increased neutrophils. Asymptomatic.  Leucopenia:  Ethnic or Benign familial Neutropenia. (Jews, Blacks & Arab)  Cyclical Neutropenia. – cyclical variation in WBC counts.  Kostmann’s syndrome: Congenital agranulocytosis – lack of G-CSF severe neutropenia & severe infections.  Felty’s syndrome: Rheumatoid arthritis & severe neutropenia.  Severe Combined Immunodeficiency: absent T Lymphocytes.  WBC Function disorders:  Chronic Granulomatous Disease: Defective microbicidal function.  Lazy leukocyte syndrome.
  • 3. “Everyone is a genius. But if you judge a fish by its ability to climb a tree, it will spend its whole life believing it is stupid…!” - Albert Einstein