AMDS refers to any patient found in the MDS Diagnosis and Treatment Guidelines. The International Working Group Working Party classification, based on cytogenetic and WHO classification systems, has been chosen by the authors to designate this category. The patients in this category have a disease that is defined by an abnormal karyotype (i.e., chromosomal abnormality) that is generally not a chromosomal abnormality but rather related to a disease of cell division (i.e., cancer). However, there are numerous subcategories within MDS. A patient's MDS subcategory in this working group (AMDS) system is based on chromosome abnormalities, WHO classification, and marrow disease.
Myelodysplastic syndromes are more common in individuals older than 62 years and are twice as common in women relative to men. myelodysplastic syndromes have been associated and have been known to be a part of the syndrome known as chronic lymphocytic leukemia. The high prevalence of myelodysplastic syndrome in patients with chronic lymphocytic leukemia might be explained because of the tendency of patients with chronic lymphocytic leukemia to develop an autoimmune disease rather than a cancer.
Treatment for patients with myelodysplastic syndromes should be individualized based on the individual patient's clinical and disease characteristics. Drugs used for treatment of myelodysplastic syndromes are primarily used to treat disease-related features and symptoms.
There is limited evidence that myelodysplastic syndromes may be cured with azacitidine. More recent studies have shown that some myelodysplastic syndromes respond to high dose myeloablative chemotherapy but with minimal long-term benefits. The evidence needs more rigorous study and is at present limited to retrospective investigations. This may be due to the difficulties associated with evaluating small group patients with myelodysplastic syndrome. It is clear that more precise definition of disease type and prognosis are important to understand.
Many of the symptoms and findings discussed may be attributed to other diseases, particularly, solid tumors. Physicians must be aware of other conditions that may cause similar signs and symptoms. An underlying diagnosis will allow for more effective management and treatment during the initial evaluation.
The occurrence of MDS cannot be explained by a single stimulus or genetic factor. It is likely due to a multiplicity of factors or combinations of factors.
IFN-γ plays an important pathophysiological role and regulates the production of immunoregulatory mediators (e.g., IL-10, IL-12, IL-17, TGF-β1), Th1 cell differentiation, and T cell function and apoptosis. Thus it is not surprising that IFN-γ administration leads to an increased number of CD123+ MDS cells, and that IFN-γ reduces the CD123 levels and results in a reversal of CD123+ cell dysfunction.
Patients with refractory or refractory/relapsing disease did not have a statistically significant advantage to clinical trial enrollment. The main reason for exclusion from clinical trials was the presence of other active disease by the time of trial inclusion but the reasons for exclusion of patients from clinical trials were as follows: older patients (<65 years) excluded due to a higher mortality rate; and patients with very poor nutritional status excluded for lack of appropriate study treatment.
IFN-γ was the most frequently used treatment, regardless of the combination with other treatments. It was used most frequently in patients with MDS and in those receiving cladribine plus IFN-γ alone. It is recommended that clinicians consider using IFN-γ injections as a first choice treatments for MDS. If IFN-γ injections were used in combination with other treatments, it was mainly used in combination with cladribine.
IFN-γ injection results in improvements in QOL for MDS patients. The IFN-γ therapy also provides a safe and effective therapy for selected MDS patients who are ineligible for ASCT.
A patient with a BMF of 13 to 20% has an OS of 2.8 months, and one with a BMF of 22% has an OS of 2.9 months. Most patients with a BMF < 20%, and those with < 20% on DSIP score, should have further evaluation for MDS. Patients with a BMF of 22% or 30% have an OS of 2.5 months. Patients with a BMF of > 50% have an OS of 2.1 months. Patients with a BMF of 50% on DSIP score should have further evaluation for MDS.
A growing evidence support the benefit of the addition of azacitidine to thalidomide for the treatment of refractory myelodysplastic syndrome. The role of demethylating agents at the initial treatment is unclear.