The Search for Better Prognostic Models in Myelodysplastic Syndromes

Abstract

Myelodysplastic syndromes (MDS) are a group of heterogeneous bone marrow disorders characterized by a failure of hematopoiesis and an increased propensity for transformation to acute myeloid leukemia. Determining the prognosis of patients with MDS is essential for discerning the best therapy, which can vary from supportive care to allogeneic stem cell transplantation. The most widely used prognostic model in MDS is the International Prognostic Scoring System (IPSS), which estimates survival and risk of transformation to acute myeloid leukemia based on the percentage of blasts, karyotype, and number of cytopenias, but the IPSS has several limitations that preclude more widespread application. Over the past decade, several studies have reported on new prognostic factors for MDS, including transfusion dependency and DNA methylation abnormalities. More recently, two prognostic models for MDS that aim to overcome the limitations of the IPSS have been published. This review focuses on the most recent advances in this field, detailing current prognostic models and the more important risk factors in MDS.

Perioperative mortality in patients with myelodysplastic syndrome

“…Our high rate of perioperative mortality might suggest our patients who underwent operation were at a high risk of death based upon their diagnosis of MDS. However, the median IPSS score in our operative group was 0.5 (0.5–2.5), or category Intermediate‐1 6, and the median MDACC score was 4 1–7, or category 2 7. The median survival of patients with IPSS Intermediate‐1 is 3.5 years, and the median survival for MDS patients in MDACC category 2 is 51 months.…”

“…Myelodysplastic syndrome (MDS) comprises a spectrum of diseases in which too few healthy blood cells are produced in the bone marrow. Rather, immature versions of blood cells (blasts) are formed 1, 2 which either die in the bone marrow or shortly after entering systemic circulation. This process leads to a reduction in circulating healthy red blood cells, white blood cells and platelets.…”

“…The WHO system classifies disease based on the primary cell lines affected. There are also several prognostic scoring systems including the WHO classification‐based Prognostic Scoring System (WPSS), the International Prognostic Scoring System (IPSS), and MD Anderson Cancer Center (MDACC) classification 1–4, 6, 7.…”

Myelodysplastic Syndromes

“…The 1997 IPSS had several important limitations. 37 It was validated only for adult patients with de novo disease treated with supportive care or hematopoietic growth factors. It did not weight severity of cytopenias, included a limited repertoire of karyotypes, did not include risk factors such as transfusion dependence, and did not adequately stratify patients with MPN features or those with t-MDS.…”

An autopsy case of an elderly man with myelodysplastic syndromes in which postmortem computed tomography showed massive splenomegaly

“…Myelodysplastic syndromes (MDS) are a group of heterogeneous bone marrow disorders characterized by ineffective hematopoiesis [1][2][3][4][5][6]. The natural evolution of disease in MDS consists of bone marrow failure leading to infectious and hemorrhagic episodes or anemia-related complications and transformation to acute myeloid leukemia.…”