Lymphoproliferative disorders

The diagnosis of a lymphoproliferative disorder in a pediatric patient is often difficult: These rare conditions are frequently unrecognized by primary care providers, and access to subspecialty dermatology care is limited for many patients. Upon the establishment of a correct diagnosis, patient and parental concerns escalate while the dermatologist ponders the severity of the disease and the risks and benefits of each possible therapeutic option. Treatment modalities vary from observation with judicious follow-up to antibiotics, immunosuppressants, and phototherapy. Many variables are balanced when the treatment regimen is enacted, including the following: the general health of the patient, patient and family ability for compliance, insurance coverage of off-label therapeutic medication usages, and geographic ease of in-office therapies and follow-up exams. Regardless of the treatment method chosen, a strong educational foundation for the patient, a patient-provider relationship of trust, and physician comfort with the treatment method used are imperative for optimal patient outcomes. In this chapter, treatment modalities, from simple to complex, for lymphoproliferative disorders such as pityriasis lichenoides, lymphomatoid papulosis, plasmacytoma, and cutaneous lymphoma will be reviewed.