08/31/2017

NCCN has published updates to the NCCN Guidelines® and the NCCN Compendium® for Myelodysplastic Syndromes

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

• Initial evaluation (MDS-1)
  • Added bullets previously listed under additional testing on MDS-2:
    • HIV testing if clinically indicated
    • Consider evaluation of copper deficiency in patients with GI malabsorption, severe malnutrition, gastric bypass surgery, or patients on zinc supplementation
    • Consider distinction from congenital sideroblastic anemia (CSA)
  • Added a new pathway if diagnostic criteria for MDS not met but cytopenias are present.
  • Modified footnote "a": "MDS is also suspected in the presence of peripheral blood dysplasia, blasts, or MDS-associated cytogenetic abnormalities. Cytopenias are defined as values lower than standard lab hematologic levels, being cognizant of age, sex, ethnic, and altitude values. Greenberg PL, Tuechler H, Schanz J, et al. Blood 2016. 128 (16): 2096-2097."
  • Modified footnote "b": "If standard cytogenetics (with ≥20 metaphases) not obtained, then MDS-related fluorescence in situ hybridization (FISH) panel should be performed."
  • Modified footnote "d": "Patients with karyotypes t(8;21), t(15;17), or inv(16) are considered to have AML even if the marrow blast count is less than 20%. (See NCCN Guidelines for AML)."
  • Modified footnote "g": "In younger patients, CSA is due to disordered mitochondrial heme synthesis, often with distinctive mutational and clinical features. Some of these patients will respond to pyridoxine or thiamine. CSA is not MDS. (Fleming MD, ASH Education Book vol.201(1), 525-531). Consider congenital bone marrow syndromes (eg, dyskeratosis congenita, Shwachman-Diamond syndrome)."
• Additional testing (MDS-2)
  • Removed "Consider HLA-DR15 typing” (also on MDS-5)
  • Added bullet previously listed under initial evaluation on MDS-1:
    • Consider additional genetic screening for patients with familial cytopenias, particularly for younger patients
• Treatment (MDS-3)
  • Clinically relevant thrombocytopenia or neutropenia or increased marrow blasts, disease progression or no response, added “Consideration for hypomethylating agents (if not already receiving).” Added a new footnote with references.
    • New footnote: “For patients with severe or refractory thrombocytopenia, eltrombopag or romiplostim can be considered. Oliva EN, Alati C, Santini V, et al. Lancet Hematol 2017; 4(3):e127-e136. Fenaux P, Muus P, Kantarjian H, et al. Br J Haematol. 2017; doi: 10.1111/bjh.14792 [epub ahead of print]. See Discussion.
• Recommendations for Flow Cytometry (MDS-E)
  • Added two new bullets:
    • "Flow cytometric abnormalities are often seen in MDS, and in some cases may correlate with observed morphologic abnormalities. They may also help diagnostically in patients with clinical suspicion of MDS who have no significant morphologic dysplasia and whose chromosome/FISH studies are either negative or normal."
    • "FCM is most useful in detecting aberrant immature myeloid lineages often observed in myelodysplastic syndromes. Flow analysis will detect aberrant expression of B or T cell antigens on myeloid precursors, and selective loss or gain of additional markers (eg, loss or dim expression of CD33, CD34, CD56, CD38, or CD117) on myeloid precursors. Flow will help in cytopenia associated with LGL expansion by detecting increase of CD56/CD57+ cells. CMML-associated monocytic aberrancies can be easily detected by combination of CD64/CD14, and CD16 loss or dim expression. In addition, qualitative abnormalities in mature myeloid lineages, eg, hypogranular late myelocytes, bands/Pelger-Huet cells and neutrophils will have abnormal flow patterns (low or negative for CD16 or CD10). However, the erythroid lineage dysplasia (dyserythropoiesis) detection by FCM is limited due to variable RBC lysing methods used in preparing flow mononuclear cell suspension. Megakaryocytic dysplasia cannot be assessed in FCM."