How Long Can You Live With Myelodysplastic Syndrome?

Myelodysplastic syndrome (MDS) is a heterogeneous group of hematologic disorder that affects bone marrow hematopoiesis causing reduced number of mature blood cells. As the name implies, myeloid stem cell lineage is affected that may involve one or all of the cell lineages. This may lead to reduced number of red blood cells, white blood cells and/or platelets causing anemia, leucopenia and/or thrombocytopenia. Since myelodysplastic syndrome follows a heterogeneous disease course, it may even transform into acute myeloid leukemia (AML). It is considered a premalignant condition and found usually in older adult population over the age of 65 years. It is more prevalent in males than in females.

Since myelodysplastic syndrome is a heterogeneous disorder, the survival of the patients with myelodysplastic syndrome is also highly varied. The survival of patients depends on various factors, such as severity of cytopenia (single lineage or multilineage), in addition to cytogenetics. In some patients, the disease is very slow to develop and in others there are severe cytopenias that may result in infections or bleeding. Most of the deaths occur due to these complications and the rest of the patients develop acute myeloid leukemia.

There are various risk stratification systems for the prognosis of myelodysplastic syndrome. These include classification by WHO (World Health Organization), FAB (French American British Cooperative Group) and IPSS and IPSS-R (Revised International Prognostic Scoring System) by Myelodysplastic Syndrome Risk Analysis Workshop. IPSS is the widely used risk stratification for myelodysplastic syndrome that helps predict the clinical course of the disease, treatment course and survival of the patients with myelodysplastic syndrome.

The IPSS classification was revised in 2012 and it considers five factors that help determine the prognosis of the patients. These factors include level of hemoglobin, platelet count, neutrophil count, percentage of bone marrow blasts and cytogenetic category of the disease. The IPSS system classified patients into four classes, namely, low risk, intermediate 1 risk, intermediate 2 risk and high risk patients. However, IPSS-R classifies patients into five subgroups, which are very low risk, low risk, intermediate risk, high risk and very high risk groups. The median survival of the patients varies from 8.8 years to 0.8 years depending of the class of risk. ⁽¹⁾

• Patients with very low risk have a median survival of 8.8 years.
• Patients with low risk have a median survival of 5.3 years and it takes around 10.8 years to develop acute myeloid leukemia (AML).
• Patients with intermediate risk have a median survival of 3.0 years and it may take 3.2 years for them to develop AML.
• Patients with high risk have a median survival of 1.6 years and it takes around 1.4 years for them to transition to AML.
• Patients with very high risk have a median survival of 0.8 years and it takes around 0.7 years for them to transition to AML.
• The mean survival of the patients is around 18-24 months, if they have single or mild cytopenias, normal chromosomes/single chromosomal abnormality (excluding chromosome 7) and <10% myeloblasts in bone marrow.
• The mean survival reduces to 6-12 months, if they have pancytopenia, mutiple chromosome/chromosome 7 abnormalities and >10% of myeloblasts in bone marrow. ^{(1) (2)}

Myelodysplastic syndrome occurs when there is decrease in myeloid stem cell formation due to clonal disorders. It is considered to be either primary (idiopathic or de novo) or secondary to various environmental factors, such as exposure to prior chemotherapy (alkylating agents, topoisomerase II inhibitors), radiation, chemicals (benzene) and viral infection. Familial cases of myelodysplastic syndrome are also seen, but they are rare. The secondary myelodysplastic syndrome is mostly related to treatment with prior chemotherapeutic agents, also known as treatment related myelodysplastic syndrome. Treatment related myelodysplastic syndrome (t-MDS) has a chance of transformation into acute myeloid leukemia (AML) in about 55% cases, while de novo myelodysplastic syndrome has a chance of transformation into AML in about 30% cases. The overall prognosis for t-MDS is also poor with a median survival of 30 weeks. ⁽²⁾

References:

Also Read:

• What is Myelodysplastic Syndrome: Causes, Types, Signs, Symptoms
• Myelodysplastic Syndromes: Staging, Risk Factors, Complications, Diagnosis
• Myelodysplastic Syndromes Treatments: Chemotherapy, Immunotherapy, Bone Marrow Transplant
• Myelodysplastic Syndromes or MDS: Treatment for Side Effects, Children with MDS
• Myelodysplastic Syndromes: Survival Rates, Prognosis, Recurrence, Remission, Lifestyle Changes, Prevention
• Coping with Myelodysplastic Syndromes & its Follow-Up Care