Langerhans Cell Histiocytosis Medication

  • Author: Christopher R Shea, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 29, 2010
 

Medication Summary

The goals of pharmacotherapy for Langerhans cell histiocytosis (LCH) are to reduce morbidity and to prevent complications.

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

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Prednisone (Deltasone, Liquid Pred, Meticorten, Orasone, Prednicen-M, Sterapred)

 

Also known as deltacortisone and deltadehydrocortisone. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Use lowest effective dose in elderly patients. Pediatric dosing depends on condition being treated and response of patient; dose for infants and children should be based on severity and response of the patient rather than on strict adherence to dose indicated by age, weight, or body surface area.

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Prednisolone (AK-Pred, Articulose-50, Delta-Cortef, Econopred, Inflamase)

 

Also known as delta hydrocortisone, metacortandralone, prednisolone acetate, and prednisolone sodium phosphate. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.

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Methylprednisolone (Adlone, A-methaPred, depMedalone, Depoject, Depopred)

 

Also known as 6-alpha-methylprednisolone, methylprednisolone acetate, and methylprednisolone sodium succinate. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation.

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Nonsteroidal anti-inflammatory drugs

Class Summary

These agents are the most commonly used medications to control mild to moderate pain and to decrease inflammation.

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Indomethacin (Indocin)

 

Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation; inhibits prostaglandin synthesis.

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Antineoplastic agents

Class Summary

These agents inhibit cell growth and proliferation.

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Mechlorethamine (Mustargen)

 

Forms interstrand and intrastrand cross-links in DNA, which, in turn, results in miscoding, breakage, and failure of replication, inhibiting cell growth. Dispensed as either an aqueous solution or an ointment. Contents of a 10-mg vial are rehydrated with 50 mL of tap water. Patient should wear protective plastic gloves while applying solution. Unused preparation may be stored in refrigerator.

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Vinblastine (Alkaban-AQ, Velban)

 

Inhibits microtubule formation, which, in turn, disrupts formation of mitotic spindle, causing cell proliferation to arrest at metaphase.

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Etoposide (Toposar, VePesid)

 

Inhibits topoisomerase II and causes DNA strand breakage, causing cell proliferation to arrest in late S or early G2 portion of the cell cycle.

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Mercaptopurine (Purinethol)

 

Purine analog that inhibits DNA and RNA synthesis, causing cell proliferation to arrest.

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Methotrexate (Folex, Rheumatrex)

 

Antimetabolite that inhibits DNA synthesis and cell reproduction in malignant cells. Adjust dose gradually to attain satisfactory response. Refer to individual protocols; may be administered through various routes.

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2-CdA/Cladribine (Leustatin)

 

Synthetic antineoplastic agent for continuous IV infusion. The enzyme deoxycytidine kinase phosphorylates this compound into active 5+-triphosphate derivative, which, in turn, breaks DNA strands and inhibits DNA synthesis. Disrupts cell metabolism, causing death to resting and dividing cells.

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Cytarabine (Cytosar-U, Tarabine PFS)

 

Converted intracellularly to active compound cytarabine-5'-triphosphate, which inhibits DNA polymerase. Cell cycle S-phase specific. Blocks progression from G1 to S phase and, in turn, kills cells that undergo DNA synthesis in S phase of cell proliferation cycle.

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Phototherapy agents

Class Summary

PUVA has been a successful therapy for some patients. The goal of treatment is to induce remission of skin diseases by repeated and controlled phototoxic reaction. Photoconjugation of psoralens with DNA produces an antiproliferative reaction in the skin, generates programmed cell death (apoptosis), and induces down-regulation of the cutaneous immune system.

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Methoxsalen (8-MOP, Oxsoralen, Oxsoralen Ultra)

 

Inhibits mitosis by binding covalently to pyrimidine bases in DNA when photoactivated by UV-A. Doses are based on lean body weight.

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Bisphosphonate derivatives

Class Summary

These agents inhibit bone resorption by osteoclasts and in turn mitigate associated bone pain.

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Zoledronate (Zometa)

 

Used to treat multiple myeloma and solid tumor bone metastases. Also used for hypercalcemia of malignancy. Inhibits bone resorption, possibly by acting on osteoclasts or osteoclast precursors. Median duration of complete response (maintaining normalized calcium levels) and time to relapse reported as 32 and 30 d, respectively. Administer daily calcium and vitamin D when used for multiple myeloma or metastatic bone disease.

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Contributor Information and Disclosures
Author

Christopher R Shea, MD  Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago

Christopher R Shea, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, Arthur Purdy Stout Society, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois Dermatological Society, International Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Markus D Boos, MD, PhD  Resident, University of Chicago Comer Children's Hospital

Markus D Boos, MD, PhD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Specialty Editor Board

Bernice R Krafchik, MBChB, FRCPC  Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto

Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Letterer-Siwe disease. Bilateral inguinal erosive plaques and erythematous papules on the abdomen. Courtesy of Dr Neil S. Prose.
Abdominal area of an infant with multiple erythematous papules covered by scale and/or crust.
Typical purpuric lesions in Langerhans cell histiocytosis (must be distinguished from seborrheic dermatitis).
On a plain skull radiograph, lesions are typically lytic, with sharp borders and a punched out appearance.
High-power views. Marked epidermotropism is noted (left). The lesional cells are large, with abundant pink cytoplasm and reniform nuclei. An admixture of inflammatory cells, including occasional eosinophils, is present (right).
High-power views. Diffuse immunoreactivity for S-100 protein (right). Langerhans cells and lymphocytes (left, hematoxylin and eosin).
Widespread positivity for CD1a. Note the presence of epidermotropism (right). Langerhans cells and lymphocytes are present in the epidermis and the papillary dermis (left, hematoxylin and eosin).
Electron microscopy. Tennis racquet form of Birbeck granules with a small terminal expansion.
 
 
 
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