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Histiocytosis in children

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Histiocytosis refers to a group of rare disorders characterized by the overproduction and accumulation of white blood cells called histiocytes. These histiocytes can infiltrate various tissues and organs in the body. In children, the two main forms of histiocytosis are Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH). Here’s an overview of histiocytosis in children:

Langerhans Cell Histiocytosis (LCH):

  • Prevalence: LCH is the most common form of histiocytosis in children. It primarily affects children under the age of 5, but it can occur at any age.
  • Symptoms: Symptoms of LCH can vary widely depending on the organs involved, but common manifestations include bone pain, skin rashes or sores, swelling, fever, and irritability.
  • Diagnosis: Diagnosis involves a combination of clinical evaluation, imaging studies (such as X-rays and CT scans), and biopsy of affected tissues. Elevated levels of a specific protein called CD1a on histiocytes are often seen in LCH.
  • Treatment: Treatment for LCH varies based on the extent and severity of the disease. Options include observation without treatment for mild cases, topical steroids or other local treatments for skin lesions, and systemic therapies such as chemotherapy in more severe cases. Multisystem involvement may require a multidisciplinary approach.

Hemophagocytic Lymphohistiocytosis (HLH):

  • Prevalence: HLH is less common but more severe than LCH. It can occur in infants, children, and adolescents.
  • Symptoms: HLH is characterized by systemic inflammation, and symptoms include fever, hepatosplenomegaly (enlarged liver and spleen), cytopenias (low blood cell counts), and dysfunction of multiple organs.
  • Diagnosis: Diagnosis is based on clinical and laboratory criteria, including genetic testing for specific mutations associated with familial HLH. A bone marrow biopsy may reveal hemophagocytosis, a hallmark of the condition.
  • Treatment: HLH is a medical emergency and requires aggressive treatment. It typically involves immune suppression with corticosteroids and chemotherapy to suppress the abnormal immune response. Hematopoietic stem cell transplantation may be considered for severe or familial cases.

Other Forms of Histiocytosis:

There are other rare forms of histiocytosis that can affect children, such as Erdheim-Chester disease and non-Langerhans cell histiocytosis. These conditions may involve different histiocyte subsets and can have distinct clinical features and treatment approaches.


  • The prognosis for children with histiocytosis varies depending on the specific form, extent of disease, and response to treatment.
  • Many cases of LCH have a good prognosis, especially when they are localized or involve only a few systems.
  • HLH is a more aggressive condition and can be life-threatening, but early diagnosis and treatment are crucial for improving outcomes.

Histiocytosis in children is managed by pediatric hematologists/oncologists and other specialists as needed, depending on the specific form and organ involvement. Treatment plans are tailored to the individual child’s condition and may involve a combination of therapies to address the disease’s manifestations. Ongoing monitoring and long-term follow-up are important to detect any relapses or late effects and to provide supportive care for affected children and their families.

The content is provided for informational purposes only and is not intended as medical advice or as a substitute for medical advice of a physician