Langerhans cell histiocytosis (Histiocytosis X)

Description Langerhans cell his...

Description

Langerhans cell histiocytosis is a rare disorder caused by the abnormal growth of a certain type of white blood cell called histiocytes. Langerhans cells are found in the skin, lymph nodes, spleen, bone marrow and lungs. They normally help fight off infection but, in this disease, their abnormal growth causes tissue damage and inflammation. The exact cause of this disorder is unknown. In the majority of cases, diagnosis occurs in childhood or early adulthood.

Symptoms

Symptoms may include bone pain or fracture, loose teeth, shortness of breath, cough or skin rash. Some people may experience enlarged glands or enlarged liver.

Tests

Common tests used for diagnosis and treatment

Workup:
A complete history and physical exam will be performed. The disease is difficult to diagnose. Blood and imaging tests will most likely be required. Ultimately, a tissue biopsy may be required to confirm the diagnosis.

Tests:
Complete blood count (CBC), Comprehensive metabolic panel (CMP), CT Scan, Positron emission tomography (PET) scan, X-ray

Other Specific Tests: tissue biopsy

Specialists:
Internal Medicine, Orthopedic Surgery, Pediatrics, Pulmonology, Blood and Cancer Care (hematology and oncology), Pediatric Hematology and Oncology

Treatment

Treatment depends on the extent of the illness. Corticosteroids have been used with success. Some individuals are treated with chemotherapy or radiation therapy. Bone marrow transplantation may be considered in severe cases.