Letterer

Letterer–Siwe disease is a rare, usually fatal, rapidly progressive disease that affects children under the age of 3. It is a type of Histiocytosis, which is a group of diseases that occur when there is an over-production of white blood cells known as histiocytes that can lead to organ damage and tumor formation. This disease is named after Ernst Siwe, a Swedish pediatrician, and Karl Letterer, a German physician.

Symptoms

The symptoms of Letterer–Siwe disease can vary greatly from patient to patient. However, some common symptoms include skin rash, enlarged liver (Hepatomegaly), enlarged spleen (Splenomegaly), lymph node swelling (Lymphadenopathy), and bone lesions. Other symptoms may include fever, weight loss, and anemia.

Causes

The exact cause of Letterer–Siwe disease is unknown. However, it is believed to be caused by an overactive immune response. This leads to an overproduction of histiocytes, which can cause damage to organs and tissues.

Diagnosis

Diagnosis of Letterer–Siwe disease is often difficult due to the rarity of the disease and the variability of symptoms. However, a biopsy of affected tissue can confirm the diagnosis. Other tests may include blood tests, bone marrow biopsy, and imaging studies such as X-rays or CT scans.

Treatment

Treatment for Letterer–Siwe disease typically involves chemotherapy to reduce the number of histiocytes. Other treatments may include radiation therapy, surgery to remove tumors, and steroids to reduce inflammation.

Prognosis

The prognosis for Letterer–Siwe disease is generally poor, with most children not surviving past the age of 3. However, with early diagnosis and aggressive treatment, some children may survive longer.

See also

• Histiocytosis
• Langerhans cell histiocytosis
• Hand–Schüller–Christian disease

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