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Lymphoproliferative Disorders, Pediatric

David T. Teachey, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Lymphoproliferative disorders are a class of nonmalignant diseases characterized by uncontrolled growth of lymphoid tissues (spleen, bone marrow, liver, lymph nodes).

  • Can be congenita...

DIAGNOSIS

HISTORY

  • ALPS

    • Typically presents at young age (average 18 months) with massive lymphadenopathy and splenomegaly

    • Many patients develop secondary autoimmune disease.

    • Most often, autoimmune destruct...

TREATMENT

MEDICATION

First Line

  • For ALPS: corticosteroids or IVIG for acute flares

  • For CD localized disease

    • Surgical resection or focal radiation. Steroids may be used to shrink lesions prior to surgery.

  • F...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Recommended follow-up imaging varies among institutions. Most physicians will repeat imaging if patient’s history changes OR to determine response to therapy. 

PROGNOSIS

ADDITIONAL READING

  • Dharnidharka VR, Webster AC, Martinez OM, et al. Post-transplant lymphoproliferative disorders. Nat Rev Dis Primers.  2016;2:15088. [View Abstract on OvidInsights]

  • George LA, Te...

CODES

ICD9

  • 238.79 Other lymphatic and hematopoietic tissues

  • 279.41 Autoimmune lymphoproliferative syndrome

  • 238.77 Post-transplant lymphoproliferative disorder (PTLD)

  • 759.89 Other specified congenital anom...

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