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Langerhans Cell Histiocytosis

Updated : April 18, 2024





Background

Langerhans cell histiocytosis (LCH) is an unexplained disorder characterized by aberrant Langerhans cell proliferation.The disease exhibits both aberrant reactive process and neoplastic process features.

It may initially manifest as a minor rash, but it can rapidly spread to the following organs or sites:

  • Bone marrow
  • Liver
  • Lungs
  • Spleen
  • Lymph Nodes
  • GI tract and
  • Pituitary Gland

Depending on the appearance and organ involvement, the prognosis varies. Individuals of all ages can be affected by LCH but it is most prevalent between infants until the age of 15. Individuals vary greatly in their presentation, treatment, and prognosis. Referral to an oncologist and long-term follow-up are essential in the treatment of LCH.

Epidemiology

Langerhans cell histiocytosis is an extremely uncommon condition. It affects one to two infants per million every year.In children 15 years old, the incidence is between 4-5 cases per million each year.

The incidence of Langerhans disease in adults is between 1-2 per million each year. It can occur at any age but is more prevalent in children younger than 15 years old.

Anatomy

Pathophysiology

No definite information is available about the pathophysiology of Langerhans cell histocytosis. The aberrant cells in LCH exhibit improper proliferation and a diminished capacity for antigen presentation.

Macrophages, inflammatory cells and cytokines are present in LCH lesions, so it has been hypothesized that aberrant Langerhans cells keep multiplying due to the interactions of these cells, or because of certain combinations of cells.

Regardless of presentation site, aberrant growth into tissue causes illness. Infiltration of the bone marrow, for instance, may reduce blood cell production.

Etiology

There is a lack of consensus about this matter, but most researchers have agreed that Langerhans Cell Histocytosis is either a neoplastic or reactive process. Multiple cytokines are involved in LGH, and there is a good survival rate for isolated lesions, but spontaneous remissions are possible.

These attributes facilitate a reactive process. Nonetheless, LGH can also cause organ invasion. At least one study has demonstrated a link between the BRAF gene mutation and the endemic disease, which is associated with an increase in mortality and normally responds to chemotherapy. These attributes facilitate a neoplastic process.

Genetics

Prognostic Factors

Approximately 50% of children who develop disseminated LCH before the age of 2 will suffer a fatal outcome, but children with localized disease will survive for longer. Only patients who have not developed additional lesions within 12-24 months following treatment can anticipate a complete recovery.

When LCH has affected the lungs, the prognosis is generally poor. Half of the patients who have LCH are extremely susceptible to a variety of dangerous life-changing symptoms.

Some of these include liver cirrhosis, pulmonary impairment, diabetes insidious, musculoskeletal disabilities, and retardation of growth. These patients can also suffer from pulmonary issues and neuropsychiatric problems such as anxiety, depression, and impairment in intellectual development.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 

vemurafenib

960

mg

Tablet

Orally 

every 12 hrs

Continue the therapy until disease progression or unacceptable toxicity occurs



 

cladribine

Children and adolescents:

5

mg/m^2

Intravenous (IV)

once a day

over 2 hrs for 5 days for a 21-day cycle



vemurafenib

Safety and efficacy are not studied  



 

vemurafenib

Refer adult dosing  

Refer adult dosing  



Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK430885/

Langerhans Cell Histiocytosis

Updated : April 18, 2024




Langerhans cell histiocytosis (LCH) is an unexplained disorder characterized by aberrant Langerhans cell proliferation.The disease exhibits both aberrant reactive process and neoplastic process features.

It may initially manifest as a minor rash, but it can rapidly spread to the following organs or sites:

  • Bone marrow
  • Liver
  • Lungs
  • Spleen
  • Lymph Nodes
  • GI tract and
  • Pituitary Gland

Depending on the appearance and organ involvement, the prognosis varies. Individuals of all ages can be affected by LCH but it is most prevalent between infants until the age of 15. Individuals vary greatly in their presentation, treatment, and prognosis. Referral to an oncologist and long-term follow-up are essential in the treatment of LCH.

Langerhans cell histiocytosis is an extremely uncommon condition. It affects one to two infants per million every year.In children 15 years old, the incidence is between 4-5 cases per million each year.

The incidence of Langerhans disease in adults is between 1-2 per million each year. It can occur at any age but is more prevalent in children younger than 15 years old.

No definite information is available about the pathophysiology of Langerhans cell histocytosis. The aberrant cells in LCH exhibit improper proliferation and a diminished capacity for antigen presentation.

Macrophages, inflammatory cells and cytokines are present in LCH lesions, so it has been hypothesized that aberrant Langerhans cells keep multiplying due to the interactions of these cells, or because of certain combinations of cells.

Regardless of presentation site, aberrant growth into tissue causes illness. Infiltration of the bone marrow, for instance, may reduce blood cell production.

There is a lack of consensus about this matter, but most researchers have agreed that Langerhans Cell Histocytosis is either a neoplastic or reactive process. Multiple cytokines are involved in LGH, and there is a good survival rate for isolated lesions, but spontaneous remissions are possible.

These attributes facilitate a reactive process. Nonetheless, LGH can also cause organ invasion. At least one study has demonstrated a link between the BRAF gene mutation and the endemic disease, which is associated with an increase in mortality and normally responds to chemotherapy. These attributes facilitate a neoplastic process.

Approximately 50% of children who develop disseminated LCH before the age of 2 will suffer a fatal outcome, but children with localized disease will survive for longer. Only patients who have not developed additional lesions within 12-24 months following treatment can anticipate a complete recovery.

When LCH has affected the lungs, the prognosis is generally poor. Half of the patients who have LCH are extremely susceptible to a variety of dangerous life-changing symptoms.

Some of these include liver cirrhosis, pulmonary impairment, diabetes insidious, musculoskeletal disabilities, and retardation of growth. These patients can also suffer from pulmonary issues and neuropsychiatric problems such as anxiety, depression, and impairment in intellectual development.

vemurafenib

960

mg

Tablet

Orally 

every 12 hrs

Continue the therapy until disease progression or unacceptable toxicity occurs



cladribine

Children and adolescents:

5

mg/m^2

Intravenous (IV)

once a day

over 2 hrs for 5 days for a 21-day cycle



vemurafenib

Safety and efficacy are not studied  



vemurafenib

Refer adult dosing  

Refer adult dosing  



https://www.ncbi.nlm.nih.gov/books/NBK430885/

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