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NCCN Flash Update: Systemic Light Chain Amyloidosis, Acute Lymphoblastic Leukemia, NCCN Templates®, & NCCN Guidelines with NCCN Evidence Blocks™

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) and NCCN Drugs & Biologics Compendium (NCCN Compendium®) for Systemic Light Chain Amyloidosis. These NCCN Guidelines® are currently available as Version 1.2018.

  • Initial diagnostic workup (AMYL-1)
    • Added “Chest CT without contrast” as indicated
    • Pathologic evaluation: abdominal fat pad aspirate, added a new footnote.
      • The new footnote states, “Alternate sites could include rectal or minor salivary gland biopsy.”
  • Clinical findings (AMYL-2)
    • Organ involvement based on amyloidosis consensus criteria: added “Evaluate for stem cell transplant (SCT) candidacy” with a footnote.
      • The new footnote states, “In those patients with very low tumor burden. If not a candidate for SCT at initial diagnosis, reassess after 2 cycles of systemic therapy.”
  • Treatment (AMYL-2)
    • Divided the treatment pathway into “Treatment of newly diagnosed disease” and “Relapsed/refractory disease.”
    • Added a new footnote: “Organ transplant, as clinically indicated” on therapy for newly diagnosed disease.
    • Removed the treatment regimens from this page and added a link to Systemic light chain amyloidosis therapy.
  • Systemic light chain amyloidosis therapy (AMYL-A)
    • This is a new page to the guideline.
    • The following regimens and corresponding references were removed from the guidelines:
      • Thalidomide/dexamethasone
      • Cyclophosphamide/thalidomide/dexamethasone
    • Added a new footnote: “Consider oral doxycycline as an adjunct to standard therapy.”
    • Added a new footnote to high-dose melphalan with stem cell transplant regimen: “The dose of melphalan as part of stem cell transplantation can be adjusted based on factors such as age, presence/absence of cardiac involvement, and number of organs involved. These risk-adapted approaches have not been evaluated in randomized studies."


NCCN has published updates to the NCCN Guidelines and the NCCN Compendium® for Acute Lymphoblastic Leukemia. These NCCN Guidelines are currently available as Version 3.2017.

  • Relapsed/Refractory Disease (ALL-9)
    • Ph-positive ALL (also ALL-D 3 of 6)
      • Tisagenlecleucel (category 2A) added as a treatment option for patients ≤25 y with refractory disease or ≥2 relapses and failure of 2 TKIs.
    • Ph-negative ALL (also ALL-D 4 of 6)
      • Tisagenlecleucel (category 2A) added as a treatment option for patients ≤25 y with refractory disease or ≥2 relapses.
  • Regimens for Relapsed/Refractory ALL (ALL-D 3 of 6/ALL-D 4 of 6)
    • Footnote k added: “Tisagenlecleucel is associated with cytokine release syndrome (CRS), including fatal or life-threatening reactions. Do not administer to patients with active infection or inflammatory disorders. Treat severe or life-threatening CRS with tocilizumab. Neurological toxicities, which may be severe or life-threatening, can occur following treatment, including concurrently with CRS. Monitor for neurological events after treatment. Provide supportive care as needed. Tisagenlecleucel is available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS). For details, see:

*For your reference, the previous update (Version 2.2017) to the NCCN Guidelines for Acute Lymphoblastic Leukemia, published on August 30, 2017 is available at the following link:


The following NCCN Chemotherapy Order Templates (NCCN Templates®) have been deleted to reflect the NCCN Guidelines for Systemic Light Chain Amyloidosis, Version 1.2018.

  • SLCA5: Cyclophosphamide/Thalidomide/Dexamethasone
  • SLCA8: Thalidomide/Dexamethasone


NCCN has published updates to the following NCCN Guidelines with NCCN Evidence Blocks™:

  • Pancreatic Adenocarcinoma, Version 3.2017


For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Compendium, the NCCN Biomarkers Compendium®, the NCCN Templates®, the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit

To view the NCCN Guidelines for Patients®, please visit

Free NCCN Guidelines apps for iPhone, iPad, and Android smartphones & tablets are now available! Visit

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