Histiocytosis Follow-up

Updated: Feb 14, 2017
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Follow-up

Further Outpatient Care

Long-term follow-up care by a multidisciplinary team with knowledge of Langerhans cell histiocytosis (LCH) is critical for all patients, not just those with extensive multisystem disease or those treated with systemic chemotherapy. [305]

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Inpatient & Outpatient Medications

See the Medication section.

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Complications

Patients with Langerhans cell histiocytosis, especially those with multisystemic disease or multifocal skeletal involvement with a relapsing course, have a significant risk of developing adverse late sequelae from their disease or therapy.

  • Some estimate that more than one half of patients have at least 1 clinically significant late effect. Therefore, long-term follow-up is of utmost importance.
  • In a study of a subset of 40 patients followed up for a median of 16 years, 51% had pronounced late sequelae. [306] Those with multisystemic involvement had the greatest risk of late effects. They had 19% rate of CNS sequelae.
  • Some of the most important late effects involve the CNS and include diabetes insipidus and other deficiencies of hypothalamic-pituitary axis. These effects lead to stunted growth and failure to achieve sexual maturity.
  • Other late effects include orthopedic problems (particularly of the vertebral column), dental issues surrounding the loss of teeth and jawbone mass, hearing loss due to mastoid and inner-ear involvement (for which patients require cochlear implantation), [307] and scarring of involved areas of skin.
  • In patients who develop pulmonary or hepatic fibrosis, progression of disease may result in a need for organ transplantation.
  • Patients with Langerhans cell histiocytosis may have a lifelong susceptibility to pulmonary disease associated with cigarette smoking.
  • Pulmonary involvement of the young child may be extensive and characterized by micronodular involvement and cystic formation. However, adequate treatment can resolve the disease and normalize lung findings and function.

Neurocognitive and psychological problems are more frequently recognized likely because of intensified patient follow-up.

  • Patients with neurodegenerative CNS involvement often present with ataxia and decreased coordination.
  • Pathologic examination of biopsy material usually reveals gliosis, some neuronal cell death, and, sometimes, areas of active Langerhans cell histiocytosis. Although the condition of neurodegenerative involvement of the CNS can remain stable for years, clinical progression may occur in the absence of MRI findings. No definitive treatment has been developed for this manifestation of Langerhans cell histiocytosis. Radiation therapy does not appear to provide any benefits.
  • Neuropsychological sequelae of Langerhans cell histiocytosis can be substantial. In one study of 10 children with Langerhans cell histiocytosis (aged 5-17 y), 3 scored one standard deviation or more below the reference range on perceptual tasks, and 4 of 10 children had deficiency in performance on perceptual tasks. Decreases in attention, speed of performance, verbal working memory, and visual recall were noted. [308]

Proliferation of Kupffer cells may accompany the initial hepatic involvement with Langerhans cell histiocytosis and subsequently develop into sclerosing cholangitis. This, in turn, may lead to fibrosis and liver failure. Lung and liver transplantation have been successful in patients who develop organ failure due to progressive fibrosis.

Secondary malignancies are reported in patients with Langerhans cell histiocytosis. Malignancies include secondary leukemias (usually acute myelogenous leukemias) caused by exposures to alkylating agents and, in recent reports, to etoposide. Other cancers include thyroid carcinoma, lymphomas, and CNS tumors.

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Prognosis

The location of the lesions and the extent of the disease substantially affect the course of the disease and the patient’s prognosis.

Involvement of risk organs (hemopoietic system, liver, spleen, and lungs) at diagnosis and failure of response to the initial therapy are poor prognostic signs. Reactivation of risk organs is relatively rare. In one study, this reactivation occurred in 2% of patients. [309] Involvement of the risk organs at reactivation had relatively low impact on survival. The degree of organ involvement is correlated with the patient’s prognosis.

Although Langerhans cell histiocytosis involvement of the spine causes lesions and, sometimes, asymmetric collapse, it is not usually associated with long-term sequelae and deformity. Therefore, aggressive surgical management of this involvement is generally not indicated.

Rapidity of the response to chemotherapy may also have prognostic value.

A study by Zeng et al suggested that in LCH, the presence of BRAF-V600E mutation is related to poor disease-free survival, with the mutation leading to interference with host-tumor immune surveillance. [310]

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Patient Education

Known genetic factors, when applicable, must be explained to the patients and their families.

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