HJNO Jan/Feb 2021

50 JAN / FEB 2021 I  HEALTHCARE JOURNAL OF NEW ORLEANS ONCOLOGY DIAL GUE COLUMN ONCOLOGY THE term Dysplasia comes from the Greek word for abnormal (dys) formation (plasia). Healthy bone marrow stem cells produce mature RBC, WBC and platelets. When they become dysfunctional due to stresses of age or extrinsic factors, it leads to arrest in cell maturation or uncontrolled cell division. Many, but not all, MDS subtypes eventually convert to leukemia. Broadly, the dysplastic syndrome must have at least one cell line impaired to make a diagnosis. Epidemiology While any age can be afflicted with disease, it’s most commonly seen in the seventh de- cade of life. Incidence is about 75 per 100,000 population. There is 2:1 male predominance, and smoking often confers an increased risk. Patients with various autoimmune disorders have an increased propensity for MDS. Clinical Findings Symptoms are consistent with cytope- nia such as fatigue, bruising and tachycar- dia. Splenomegaly is common. In the MPN syndromes, increased clotting tendency can indolent; rarely turns into leukemia; • Chronic myeloid leukemia, BCR-ABL positive (CML) – decades of longevity now possible; • PNH is anMPN, where a clone sensitive to complement destruction proliferates and transforms into AML or fibrosis – very challenging disease, though two new drugs have decrease morbidity; • Therapy related myeloid neoplasms – has the worst prognosis with few effec- tive treatments, secondary to previous chemotherapy or radiation. “New” categories: • CHIP (clonal hematopoiesis indetermi- nate potential); • CCUS (clonal cytopenia of undefined significance). These may be present decades before the full manifestation of MDS as we know it. Pathophysiology With high throughput PCR, the complexi- ties of MDS/MPN have given us a deep un- derstanding of these syndromes. Multiple MYELODYSPLASTIC AND MYELOPROLIFERATIVE SYNDROMES (MDS, MPN) These long and scary-sounding diagnoses were simplistically called preleukemia. With our modern diagnostic tools, having led to better understanding of basic biology, we are now able to formulate rational treatments and predict their outcomes. pose a challenge. Many of these can prog- ress on to AML (acute myeloid leukemia) or myelofibrosis. Common MDS syndromes • 5q- syndrome • MDS with excess blasts type 1 or 2 (RAEB1 and RAEB2) • MDS with single lineage • MDS with multilineage • MDS with ring sideroblasts (RARS) Common MPN Syndromes JAK2 mutations are commonly pres- ent and often indicate a poorer prognosis. However, CALR mutation at diagnosis may indicate a more benign prognosis even if Jak2 is acquired later on. Other mutations can occur, too: • Chronic myelomonocytic leukemia (CMML) – indolent and quite respon- sive to treatment; • Polycythemia Vera (PV) – indolent for years but often convert to acute leuke- mia or Myelofibrosis; • Essential thrombocythemia (ET) – very