Medication Summary
No specific treatment exists for the reactive form. Therapy is focused on the underlying disease, if any. For the malignant form, promptly initiate chemotherapy after making the diagnosis.
Antineoplastics, Other
Class Summary
Chemotherapy protocols similar to that applied in classic lymphoma or leukemia treatment are a widely accepted method of medical intervention.
Cyclophosphamide
Cyclophosphamide is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Cyclophosphamide may be administered at 50-100 mg/m2/d PO or 400-1000 mg/m2 PO in divided doses every 4-5 days; alternatively, administer 400-1800 mg/m2 (30-40 mg/kg) IV in divided doses over 2-5 days; this may be repeated at 2- to 4-week intervals; 10-15 mg/kg IV q7-10d or 3-5 mg/kg bid.
Doxorubicin (Adriamycin)
Doxorubicin inhibits topoisomerase II and produces free radicals, which may cause the destruction of DNA. The combination of these 2 events can, in turn, inhibit the growth of neoplastic cells. Doxorubicin may be administered at 60-75 mg/m2 IV as a single dose; repeat q21d. Alternatively, administer 20-30 mg/m2/d IV for 2-3 d; repeat in 4 weeks in adults. In children, it may be administered at 35-75 mg/m2 IV as a single dose; repeat q21d. Alternatively, administer 20-30 mg/m2 IV qwk.
Vincristine (Vincasar PFS)
The mechanism of action is uncertain, but it may involve a decrease in reticuloendothelial cell function or an increase in platelet production; however, neither of these mechanisms would fully explain the effect in TTP and HUS. Vincristine may be administered at 2 mg IV push in adults. Administer 1.4 mg/m2 IV push, not to exceed 2 mg, in children.
Corticosteroids
Class Summary
These agents are used for their immunosuppressant effects.
Prednisone
Prednisone is an immunosuppressant for the treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production. It may also cause profound and varied metabolic effects, particularly in relation to salt, water, and glucose tolerance. Alternative corticosteroids may be used in equivalent dosages.
Prednisone may be administered at 5-60 mg/d PO qd or divided bid/qid; taper over 2 weeks as symptoms resolve in adults. In children, it may be administered at 4-5 mg/m2/d PO; alternatively, administer 0.05-2 mg/kg PO divided bid/qid; taper over 2 weeks as symptoms resolve.
Prednisolone (Orapred, Pediapred, Millipred)
Prednisolone is an immunosuppressant for the treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisolone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production. It may also cause profound and varied metabolic effects, particularly in relation to salt, water, and glucose tolerance. Alternative corticosteroids may be used in equivalent dosages.
Prednisolone may be administered at 5-60 mg/d PO qd or divided bid/qid; taper over 2 weeks as symptoms resolve in adults. In children, prednisolone may be administered at 4-5 mg/m2/d PO; alternatively, administer 0.05-2 mg/kg PO divided bid/qid; taper over 2 weeks as symptoms resolve.
Retinoid-like Agents
Class Summary
Decreased cohesiveness of abnormal hyperproliferative keratinocytes may reduce potential for malignant degeneration. Retinoids modulate keratinocyte differentiation and have been shown to reduce risk of skin cancer formation.
Isotretinoin (Amnesteem, Claravis, Sotret)
Isotretinoin is an oral agent that treats serious dermatologic conditions. Isotretinoin is the synthetic 13-cis isomer of the naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to vitamin A. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.
A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.
Single cases of diffuse dermal angiomatosis have responded to treatment. A dose of 0.5 mg/kg is suggested.
