Langerhans Cell Histiocytosis: Treatment, Procedure, Cost and Side Effects

Langerhans cell histiocytosis is also known as blood disease, blood disorder, Hand Schuller-Christian disease, Schuller- Christian disease

Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multi system disease. LCH is part of a group of clinical syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages).These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas.

As with some types of cancer, doctors sometimes treat LCH with chemotherapy. Many people with the disorder get care from cancer specialists like oncologists and haematologists. But unlike most cancers, limited forms of LCH sometimes spontaneously go away on their own.Beside chemotherapy, options for treatment include:Low-dose radiation to targeted part of the body,Surgery to remove LCH lesions,Steroids like prednisone or anti-inflammatory drugs,Ultraviolet light therapy for skin conditions,Stem cell transplant,Bone marrow, liver, or lung transplants in very serious casesA great majority of people with LCH recover with treatment. If the disease is in your spleen, liver, or bone marrow, it’s called a high-risk LCH. About 80% of people with that type survive.

Guidelines for management of patients up to 18 years with Langerhans cell histiocytosis has been suggested. Treatment is guided by extent of disease. Solitary bone lesion may be amenable through excision or limited radiation, dosage of 5-10 Gy for children, 24-30 Gy for adults. However systemic diseases often require chemotherapy. Use of systemic steroid is common, singly or adjunct to chemotherapy. Local steroid cream is applied to skin lesions. Endocrine deficiency often require lifelong supplement e.g. desmopressin for diabetes insipidus which can be applied as nasal drop. Chemotherapeutic agents such as alkylating agents, antimetabolites, vinca alkaloids either singly or in combination can lead to complete remission in diffuse disease.

Diagnosis is confirmed histologically by tissue biopsy. Hematoxylin- eosin stain of biopsy slide will show features of Langerhans Cell e.g. distinct cell margin, pink granular cytoplasm. Presence of Birbeck granules on electron microscopy and immuno- cytochemical features e. g. CD1 positivity are more specific. Initially routine blood tests e.g. full blood count, liver function test, U&Es, bone profile are done to determine disease extent and rule out other causes. Radiology will show osteolytic bone lesions and damage to the lung

LCH provokes a non-specific inflammatory response, which includes fever, lethargy, and weight loss. Organ involvement can also cause more specific symptoms.Bone: The most-frequently seen symptom in both unifocal and multifocal disease is painful bone swelling. The skull is most frequently affected, followed by the long bones of the upper extremities and flat bones. Infiltration in hands and feet is unusual. Osteolytic lesions can lead to pathological fractures.Skin: Commonly seen are a rash which varies from scaly erythematous lesions to red papules pronounced in intertriginous areas. Up to 80% of LCH patients have extensive eruptions on the scalp.

Bone marrow: Pancytopenia with superadded infection usually implies a poor prognosis. Anemia can be due to a number of factors and does not necessarily imply bone marrow infiltration.Lymph node: Enlargement of the liver in 20%, spleen in 30% and lymph nodes in 50% of Histiocytosis cases.People who have not faced any such problems are far from the danger.

There is no specific information available.

Every patient should be followed by the local physician and if at any time a particular issue needs to be addressed, referral to a specialist is recommended. All patients should be followed for a sufficient time period, defined as (i) at least 5 years after the end of therapy; or (ii) 5 years after the last disease reactivation, in those who did not receive systemic therapy; or (iii) until final growth and pubertal development have occurred.LCH is a rare disease potentially resulting in death or permanent sequelae. The burden of therapy may also be extremely heavy. There is an obvious need for a full assessment of each patient with a rational treatment tailored to the risks of the individual patient, which contributes to further fundamental and clinical research in this field.

The clinical course of Langerhans cell histiocytosis (LCH) is variable. Patients with unifocal disease generally have an excellent prognosis. After initial bone scanning and radiographic survey to assess the extent of the disease, follow-up studies after treatment should be performed at 6-month intervals for 3 years. If no additional lesions are present at 1 year, the development of subsequent lesions is unlikely. A full recovery is also expected in cases of solitary lymph node involvement or isolated skin disease

No specific information is available.

More than half the patients younger than 2 years with disseminated Langerhans cell histiocytosis (LCH) and organ dysfunction die of the disease, whereas unifocal LCH and most cases of congenital self-healing histiocytosis are self-limited. Multifocal chronic LCH is self-limited in most cases, but increased mortality has been observed among infants with pulmonary involvement. After initial bone scanning and radiographic survey to assess the extent of the disease, follow-up studies after treatment should be performed at 6-month intervals for 3 years. A full recovery is also expected in cases of solitary lymph node involvement or isolated skin disease.