Langerhans cell histiocytosis medical therapy: Difference between revisions
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{{CMG}} {{AE}} {{HL}} | {{CMG}} {{AE}} {{HL}} | ||
==Overview== | ==Overview== | ||
Observation is recommended for all pediatric patients with an isolated Langerhans cell histiocytosis [[skin]] involvement. Medical therapy is suggested only for symptomatic [[cutaneous]] disease that presents with either extensive [[rash]], sever pain, skin [[ulcer|ulceration]], or [[bleeding]]. Medical therapies used for the treatment of isolated skin lesions of Langerhans cell histiocytosis include topical [[steroid]]s, [[nitrogen mustard]], and oral [[thalidomide]]. [[Curettage]] is the mainstay treatment for single [[skull]] lesions that involve the [[frontal]], [[parietal]], or [[occipital]] [[bone]]s. The most commonly used systemic [[chemotherapy]] regimen for the management of multiple bone lesions is a combination of [[vinblastine]] {{and}} [[prednisone]]. The standard therapy recommended for Langerhans cell histiocytosis involving the [[spleen]], [[liver]], or [[bone marrow]] (high-risk organs) consists of [[vinblastine]] {{and}} [[prednisone]] {{and}} [[6-mercaptopurine]] for a 12 month period. | Observation is recommended for all pediatric patients with an isolated Langerhans cell histiocytosis [[skin]] involvement. Medical therapy is suggested only for symptomatic [[cutaneous]] disease that presents with either extensive [[rash]], sever pain, skin [[ulcer|ulceration]], or [[bleeding]]. Medical therapies used for the treatment of isolated skin lesions of Langerhans cell histiocytosis include topical [[steroid]]s, [[nitrogen mustard]], and oral [[thalidomide]]. [[Curettage]] is the mainstay treatment for single [[skull]] lesions that involve the [[frontal]], [[parietal]], or [[occipital]] [[bone]]s. The most commonly used systemic [[chemotherapy]] regimen for the management of multiple bone lesions is a combination of [[vinblastine]] {{and}} [[prednisone]]. The standard therapy recommended for Langerhans cell histiocytosis involving the [[spleen]], [[liver]], or [[bone marrow]] (high-risk organs) consists of [[vinblastine]] {{and}} [[prednisone]] {{and}} [[6-mercaptopurine]] for a 12 month period.<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
==Treatment== | ==Treatment== | ||
===Low-risk Disease (Single-system Unifocal Disease)=== | ===Low-risk Disease (Single-system Unifocal Disease)=== | ||
====Isolated Skin Involvement==== | ====Isolated Skin Involvement==== | ||
* Observation is recommended for all pediatric patients with isolated [[skin]] involvement of Langerhans cell histiocytosis. | * Observation is recommended for all pediatric patients with isolated [[skin]] involvement of Langerhans cell histiocytosis.<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
* Medical therapy is suggested only for symptomatic [[cutaneous]] disease that presents with either extensive [[rash]], sever pain, skin [[ulcer|ulceration]], or [[bleeding]]. | * Medical therapy is suggested only for symptomatic [[cutaneous]] disease that presents with either extensive [[rash]], sever pain, skin [[ulcer|ulceration]], or [[bleeding]]. | ||
* Medical therapies used for the treatment of isolated skin lesions of Langerhans cell histiocytosis include: | * Medical therapies used for the treatment of isolated skin lesions of Langerhans cell histiocytosis include: | ||
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* [[Curettage]] is the mainstay treatment for single [[skull]] lesions that involve the [[frontal]], [[parietal]], or [[occipital]] bones. | * [[Curettage]] is the mainstay treatment for single [[skull]] lesions that involve the [[frontal]], [[parietal]], or [[occipital]] bones. | ||
* In certain cases, curettage plus an injection of [[methylprednisolone]] may be used | * In certain cases, curettage plus an injection of [[methylprednisolone]] may be used | ||
* The use of low-dose [[radiation therapy]] is limited among pediatric patients due to severe side effects. | * The use of low-dose [[radiation therapy]] is limited among pediatric patients due to severe side effects.<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
* Patients with isolated lesions to the [[mastoid]], [[temporal]], or [[orbital]] [bone]s are treated with systemic therapy, as to prevent the development of [[diabetes insipidu]]s among such patients. | * Patients with isolated lesions to the [[mastoid]], [[temporal]], or [[orbital]] [bone]s are treated with systemic therapy, as to prevent the development of [[diabetes insipidu]]s among such patients. | ||
* Comparison of diabetes insipidus incidence with no systemic therapy (40%) versus 6 months of [[vinblastine]] {{and }} [[prednisone]] (20%) strongly supports systemic treatment of of such bony lesions. | * Comparison of diabetes insipidus incidence with no systemic therapy (40%) versus 6 months of [[vinblastine]] {{and }} [[prednisone]] (20%) strongly supports systemic treatment of of such bony lesions. | ||
====Vertebral or Femoral Bone Lesions at Risk for Collapse==== | ====Vertebral or Femoral Bone Lesions at Risk for Collapse==== | ||
* Observation is recommended for the management of a single [[vertebral body]] lesion that has no soft tissue extension into the extradural space. | * Observation is recommended for the management of a single [[vertebral body]] lesion that has no soft tissue extension into the extradural space.<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
* Low-dose radiation therapy may be used to try to promote resolution in an isolated vertebral body lesion or a large femoral neck lesion at risk of fracture | * Low-dose radiation therapy may be used to try to promote resolution in an isolated vertebral body lesion or a large femoral neck lesion at risk of fracture | ||
* Despite the low dose required (700–1,000 cGy), [[radiation therapy]] should be used with caution in the area of the thyroid gland, brain, or any growth plates. | * Despite the low dose required (700–1,000 cGy), [[radiation therapy]] should be used with caution in the area of the thyroid gland, brain, or any growth plates. | ||
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===Multiple Bone Lesions (Single-system Multifocal Bone Disease)=== | ===Multiple Bone Lesions (Single-system Multifocal Bone Disease)=== | ||
* The most commonly used systemic chemotherapy regimen for the management of multiple [[bone]] lesions is a combination of [[vinblastine]] and [[prednisone]]. | * The most commonly used systemic chemotherapy regimen for the management of multiple [[bone]] lesions is a combination of [[vinblastine]] and [[prednisone]].<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
* [[Vinblastine]] treatment is recomended for 12 months. Weekly doses for the first 7 weeks, which is followed by a dose every 3 weeks for the rest of the management period. | * [[Vinblastine]] treatment is recomended for 12 months. Weekly doses for the first 7 weeks, which is followed by a dose every 3 weeks for the rest of the management period. | ||
* [[Prednisone]] (40 mg/m2) is given daily for 4 weeks then tapered over 2 weeks. Afterwards prednisone is given for 5 days at 40 mg/m2 every 3 weeks with the vinblastine injections. | * [[Prednisone]] (40 mg/m2) is given daily for 4 weeks then tapered over 2 weeks. Afterwards prednisone is given for 5 days at 40 mg/m2 every 3 weeks with the vinblastine injections. | ||
===Multiple Bone Lesions in Combination with Skin, Lymph Node, or Diabetes Insipidus (High-risk Multisystem Disease)=== | ===Multiple Bone Lesions in Combination with Skin, Lymph Node, or Diabetes Insipidus (High-risk Multisystem Disease)=== | ||
* Vinblastine {{and}} prednisone combination therapy is the mainstay treatment for low-risk multisystem Langerhans cell histiocytosis. | * Vinblastine {{and}} prednisone combination therapy is the mainstay treatment for low-risk multisystem Langerhans cell histiocytosis.<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
* The same chemotherapy regimen of vinblastine and prednisone as described above is used for 12 months. | * The same chemotherapy regimen of vinblastine and prednisone as described above is used for 12 months. | ||
* Other chemotherapeutic agents used for the treatment of low-risk multisystem Langerhans cell histiocytosis include: | * Other chemotherapeutic agents used for the treatment of low-risk multisystem Langerhans cell histiocytosis include: | ||
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===Treatment of High-risk Multisystem Disease=== | ===Treatment of High-risk Multisystem Disease=== | ||
* The standard therapy length recommended for Langerhans cell histiocytosis involving the [[spleen]], [[liver]], or [[bone marrow]] (high-risk organs) consists of [[vinblastine]] {{and}} [[prednisone]] {{and}} [[6-mercaptopurine]] for a 12 month period. | * The standard therapy length recommended for Langerhans cell histiocytosis involving the [[spleen]], [[liver]], or [[bone marrow]] (high-risk organs) consists of [[vinblastine]] {{and}} [[prednisone]] {{and}} [[6-mercaptopurine]] for a 12 month period.<ref name="cancer">Langerhans Cell Histiocytosis Treatment–for health professionals (PDQ®). National Cancer Institute (2015) http://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_91 Accessed on February, 8 2016</ref> | ||
===Treatment of CNS Langerhans cell histiocytosis=== | ===Treatment of CNS Langerhans cell histiocytosis=== | ||
* There are three types of Langerhans cell histiocytosis [[CNS]] lesions: | * There are three types of Langerhans cell histiocytosis [[CNS]] lesions: | ||
Revision as of 14:59, 8 February 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Observation is recommended for all pediatric patients with an isolated Langerhans cell histiocytosis skin involvement. Medical therapy is suggested only for symptomatic cutaneous disease that presents with either extensive rash, sever pain, skin ulceration, or bleeding. Medical therapies used for the treatment of isolated skin lesions of Langerhans cell histiocytosis include topical steroids, nitrogen mustard, and oral thalidomide. Curettage is the mainstay treatment for single skull lesions that involve the frontal, parietal, or occipital bones. The most commonly used systemic chemotherapy regimen for the management of multiple bone lesions is a combination of vinblastine AND prednisone. The standard therapy recommended for Langerhans cell histiocytosis involving the spleen, liver, or bone marrow (high-risk organs) consists of vinblastine AND prednisone AND 6-mercaptopurine for a 12 month period.[1]
Treatment
Low-risk Disease (Single-system Unifocal Disease)
Isolated Skin Involvement
- Observation is recommended for all pediatric patients with isolated skin involvement of Langerhans cell histiocytosis.[1]
- Medical therapy is suggested only for symptomatic cutaneous disease that presents with either extensive rash, sever pain, skin ulceration, or bleeding.
- Medical therapies used for the treatment of isolated skin lesions of Langerhans cell histiocytosis include:
- Topical steroids
- Oral methotrexate (20 mg/m2) weekly for 6 months
- Oral thalidomide 50 mg to 200 mg each night
- Topical application of nitrogen mustard can be effective for cutaneous Langerhans cell histiocytosis that is resistant to oral therapies, but not for disease involving large areas of skin.
- Psoralen and long-wave ultraviolet A radiation (PUVA) and UVB can be effective in skin Langerhans cell histiocytosis but its use is limited by the potential for late skin cancers.
Skull Involvement
- Curettage is the mainstay treatment for single skull lesions that involve the frontal, parietal, or occipital bones.
- In certain cases, curettage plus an injection of methylprednisolone may be used
- The use of low-dose radiation therapy is limited among pediatric patients due to severe side effects.[1]
- Patients with isolated lesions to the mastoid, temporal, or orbital [bone]s are treated with systemic therapy, as to prevent the development of diabetes insipidus among such patients.
- Comparison of diabetes insipidus incidence with no systemic therapy (40%) versus 6 months of vinblastine AND prednisone (20%) strongly supports systemic treatment of of such bony lesions.
Vertebral or Femoral Bone Lesions at Risk for Collapse
- Observation is recommended for the management of a single vertebral body lesion that has no soft tissue extension into the extradural space.[1]
- Low-dose radiation therapy may be used to try to promote resolution in an isolated vertebral body lesion or a large femoral neck lesion at risk of fracture
- Despite the low dose required (700–1,000 cGy), radiation therapy should be used with caution in the area of the thyroid gland, brain, or any growth plates.
- Patients with soft tissue extension from vertebral lesions are often treated successfully with chemotherapy.
Multiple Bone Lesions (Single-system Multifocal Bone Disease)
- The most commonly used systemic chemotherapy regimen for the management of multiple bone lesions is a combination of vinblastine and prednisone.[1]
- Vinblastine treatment is recomended for 12 months. Weekly doses for the first 7 weeks, which is followed by a dose every 3 weeks for the rest of the management period.
- Prednisone (40 mg/m2) is given daily for 4 weeks then tapered over 2 weeks. Afterwards prednisone is given for 5 days at 40 mg/m2 every 3 weeks with the vinblastine injections.
Multiple Bone Lesions in Combination with Skin, Lymph Node, or Diabetes Insipidus (High-risk Multisystem Disease)
- Vinblastine AND prednisone combination therapy is the mainstay treatment for low-risk multisystem Langerhans cell histiocytosis.[1]
- The same chemotherapy regimen of vinblastine and prednisone as described above is used for 12 months.
- Other chemotherapeutic agents used for the treatment of low-risk multisystem Langerhans cell histiocytosis include:
Treatment of High-risk Multisystem Disease
- The standard therapy length recommended for Langerhans cell histiocytosis involving the spleen, liver, or bone marrow (high-risk organs) consists of vinblastine AND prednisone AND 6-mercaptopurine for a 12 month period.[1]
Treatment of CNS Langerhans cell histiocytosis
- There are three types of Langerhans cell histiocytosis CNS lesions:
- Mass lesions or tumors in the cerebrum, cerebellum, or choroid plexus
- Mass lesions of the hypothalamic-pituitary axis that are always associated with diabetes insipidus
- Neurodegenerative syndrome
- Drugs that cross the blood-brain barrier, such as cladribine, or other nucleoside analogs, such as cytarabine, are used for active CNS Langerhans cell histiocytosislesions.
- Immunochemotherapeutic agents used for the treatment of Langerhans cell histiocytosis neurodegenerative syndrome include: