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Hand-Schuller-Christian Syndrome
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- In this disease of the immune system, abnormal scavenger
cells called histiocytes and eosinophils (another immune
system cell type) grow uncontrollably, proliferating usually
in the lungs and bone, often forming scars that interfere
with normal functioning. These cells constitute malignant or
benign macrophages in the tissues, or Langerhans' cells in
the bone marrow.
- This syndrome is characterized by three, simultaneous
conditions:
- Diabetes Insipidus
- Exopthalmus
- Destructive Bone Lesions
- Diabetes Insipidus is
a condition in which the body fails to produce the hormone
vasopressin (anti-diuretic hormone), or the kidneys fail to
use vasopressin properly. The result is loss of large
volumes of water through excretion (urination).
Exophthalmos means protrusion of the globe of the eye (i.e.,
the "eyeball" protrudes). It is caused by damage to
the pituitary gland.
- Hand-Schuller-Christian syndrome often occurs with lymphoma, carcinoma,
and multifocal eosinophilic granuloma (it occurs in 25% of
all cases of multifocal eosinophilic granuloma).
- Treatment involves treating the underlying cause, such as lymphoma, and manifestations, such as diabetes insipidus. See sections on specific underlying diseases and/or manifestations for more details.
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