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Lymphoproliferative Disorders

Lymphoproliferative Disorders is a topic covered in the 5-Minute Pediatric Consult.

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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 congenital or acquired
  • Based on recent advances in next generation sequencing technology, new disorders are defined constantly.
  • Most common in children include
    • Autoimmune lymphoproliferative syndrome (ALPS)
    • Castleman disease (CD)
    • Rosai–Dorfman disease (RDD)
    • EBV-associated lymphoproliferative disorder (ELD)
    • X-linked lymphoproliferative syndrome (XLP)
  • Rarer disorders (not discussed in detail)
    • Angioimmunoblastic lymphadenopathy
    • Caspase-8 deficiency syndrome (CEDS)
    • Dianzani autoimmune lymphoproliferative disease
    • Kikuchi-Fujimoto syndrome
    • Lymphomatoid granulomatosis
    • Lymphomatoid papulosis
    • Ocular adnexal lymphoid proliferation
    • RAS-associated leukoproliferative disorder (RALD)
    • p110δ activating mutation causing senescent T cells lymphadenopathy and immunodeficiency (PASLI)
    • CTLA-4 haploinsufficiency with autoimmune infiltration (CHAI)
    • LRBA deficiency with autoantibodies, regulatory T-cell defects, autoimmune infiltration and enteropathy (LATAIE)
    • X-linked immunodeficiency with magnesium defect, EBV infection, and neoplasia (X-MEN)
    • Interleukin-2-inducible T-cell kinase (ITK) deficiency

Epidemiology

All uncommon

Risk Factors

Often multifactorial with inherited genetic defect and acquired infection

Genetics

  • ALPS (80% of patients have identifiable mutation)
    • 60–70% germline mutation in FAS (TNFRSF6)
    • 10% somatic mutation in FAS
    • 2% germline mutation in CASP10
    • <1% germline mutation in FASL
  • XLP
    • Majority of cases mutation in SH2DIA
    • XLP-like syndrome caused by X-linked inhibitor of apoptosis protein (XIAP) mutations

Pathophysiology

  • ALPS
    • Defective FAS-mediated apoptosis leads to abnormal lymphocyte survival with subsequent lymphoproliferation, autoimmunity, and cancer.
  • CD
    • Largely unknown but can be triggered by HHV-8 infection, especially in immunocompromised patients
  • ELD
    • EBV triggered lymphoproliferative disorder found in patients on chronic immune suppression typically after organ or bone marrow transplant (post-transplant lymphoproliferative disorder [PTLD]) or with inherited immune deficiency
  • XLP
    • Mutation in SH2D1A leads to abnormal production of SAP protein in NK and T cells, leading to defective SAP-SLAM signaling and inability to appropriately respond to EBV infection.

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Citation

Cabana, Michael D., editor. "Lymphoproliferative Disorders." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617757/all/Lymphoproliferative_Disorders.
Lymphoproliferative Disorders. In: Cabana MD, ed. 5-Minute Pediatric Consult. 8th ed. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617757/all/Lymphoproliferative_Disorders. Accessed April 25, 2019.
Lymphoproliferative Disorders. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult. Available from https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617757/all/Lymphoproliferative_Disorders
Lymphoproliferative Disorders [Internet]. In: Cabana MD, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2019 April 25]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617757/all/Lymphoproliferative_Disorders.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Lymphoproliferative Disorders ID - 617757 ED - Cabana,Michael D, BT - 5-Minute Pediatric Consult UR - https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617757/all/Lymphoproliferative_Disorders PB - Wolters Kluwer ET - 8 DB - Pediatrics Central DP - Unbound Medicine ER -