Medical Archive

C153 Neutrophils

C154 Monocytes, Macrophages, and Dendritic Cells

C155 Eosinophils

C156 Disorders of phagocytic function

S.aureus? ?? ????? ???

Leukocyte adhesion deficiency (type 1; AR)

Defects of LFA-1 integrin (CD 18) on phagocytes; impaired migration and chemotaxis

Recurrent skin and bacterial mucosal infections, absent pus, impaired wound healing, delayed (>30days) seperation of umbilical cord.

?Neutrophils in blood

<Pathology> C2 Inflammation, Inflammatory Disorders, and Wound Healing

Chronic granulomatous disease

Pathophysiology

• ?: XR, ?: AR. Phagocytosis ? lysosome? ??? ??? ?? d/t NADPH oxidase deficiency
  • Bacteria? ??? ? H2O2? ???? MPO system? ??. Catalase+ bacteria? ???? (e.g., S.aureus, Aspergillus)
  • <Harrison> C342 Introduction to the Immune System

Clinical findings

• Severe cutaneous and pulmonary infections with catalase-positive bacterial and fungal organisms
  • S.aureus, Serratia, Burkholderia, Aspergillus, Nocardia
• Abnoraml oxidative burst
  • NBT reduction test (superoxide ?? ??): fails to turn blue
  • DHR flow cytometry: abnormal – ?green fluoresscence

Management

• BMT? ??? ???.
• Antimicrobial prophylaxis
  • TMP-SMX
  • Itraconazole
  • With severe phenotype – ?? ???? ?? INF-?.

Chediak-Higashi (AR)

• ???? ? ???? ? ??? ?? ?? ??
  • Defect in lysosomal trafficking regulator gene (LYST). 
  • Microtubule dysfunction in phagosome-lysosome fusion; 
• Findings
  • PLAIN: Progressive neurodegeneration, Lymphohistiocytosis, Albinism (partial), recurrent pyogenic Infections by staphylococci and streptococci, peripheral Neuropathy.
  • Neutropenia d/t intramedullary death of neutrophils
  • Giant granules in neutrophils and monocytes[B].
  • Defective primary hemostasis d/t abnormal dense granules in platelets
  • Abnormal melanin storages in melanocytes ? albinism
• ???, ?? ??? ? ??? Vit.C, BMT

C157 Leukopenia

C158 Leukocytosis