Chédiak-Higashi
syndrome
Features:
Giant lysosomal granules in blood cells due to fusion of granules
Frequent
pyogenic bacterial infections
Partial
oculocutaneous albinism
Mild to moderate hemorrhagic episodes depending on platelet count
Lymphocytic proliferation in spleen, liver, and bone marrow
Macrophage
accumulation in tissues
Peripheral
pancytopenia