Chediak-Higashi Syndrome Medication

Updated: Aug 08, 2019
  • Author: Roman J Nowicki, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

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Antiviral agents

Class Summary

These agents inhibit DNA synthesis and viral replication. Nucleoside analogs are initially phosphorylated by viral thymidine kinase to eventually form a nucleoside triphosphate. These molecules inhibit HSV polymerase with 30-50 times the potency of human alpha-DNA polymerase.

Use as suggested and dosing below is not for antiviral properties but for cellular antimitotic activity at high doses for the accelerated phase.

Acyclovir (Zovirax)

Acyclovir inhibits the activity of both HSV-1 and HSV-2. It has affinity for viral thymidine kinase and, once phosphorylated, causes DNA chain termination when acted on by DNA polymerase.

Use as suggested, and dosing below is not for antiviral properties but for cellular antimitotic activity at high doses for the accelerated phase.

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Immune modulators

Class Summary

These agents inhibit key steps in immune reactions.

Immune globulin intravenous (Sandoglobulin, Gammagard, Gamimune, Gammar-P)

Intravenous immune globulin neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks the complement cascade; promotes remyelination; and may increase CSF IgG (10%).

Interferon alfa-2a and -2b (Roferon-A, Intron A)

Interferon alfa-2a and -2b are protein products manufactured by recombinant DNA technology. Their mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles.

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Antineoplastic agents

Class Summary

These agents inhibit cell growth and proliferation. Useful in the accelerated phase of the disease.

Vincristine (Vincasar PFS, Oncovin)

Vincristine's mechanism of action is uncertain. It may involve a decrease in reticuloendothelial cell function or an increase in platelet production. Reduce the dose by 50% if the direct bilirubin level is greater than 3 mg/100 mL.

Vinblastine (Alkaban-AQ, Velban)

Vinblastine inhibits microtubule formation, which, in turn, disrupts the formation of the mitotic spindle, causing cell proliferation to arrest at metaphase. Use hematologic parameters as a guide. If the direct bilirubin level is greater than 3, reduce the dose by 50%.

Local injection of hyaluronidase and application of moderate heat to the area of extravasation help disperse drug and are thought to minimize discomfort and the possibility of cellulitis.

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Anti-inflammatory agents

Class Summary

Systemically interfere with events leading to inflammation.

Colchicine

Colchicine decreases leukocyte motility and phagocytosis in inflammatory responses.

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