Myelodysplastic Syndrome (MDS)

What Is Myelodysplastic Syndrome?

The myelodysplastic syndrome includes several hematopoietic system diseases characterized by defective maturation of stem cells in the bone marrow. Due to the insufficiency of the bone marrow, the three blood cell rows cannot mature in a usual manner, resulting in insufficient functional white blood cells, red blood cells, and platelets. 'Dysplastic' means 'lacking development, deformed' and describes the deformed cells in MDS. When examined under a microscope, they look different from normal cells, resulting in a lack of mature blood cells in the bloodstream. Likewise, a proliferation of stem cells results in an overflow of immature blood cells, called blasts, into the blood. Over time, this can become distinct to the extent that the myelodysplastic syndrome can no longer be distinguished from acute leukemia in the blood smear and can transition into acute leukemia. The disease occurs mainly in older age but is in principle possible in all ages. It is one of the most common malignant blood diseases in adults.

Is MDS a Cancer?

MDS is described as a malignant disease of the bone marrow and can transition into acute leukemia, i.e., acute blood cancer, in the further course of the disease.

Causes and Symptoms of MDS

In most cases, a so-called primary MDS occurs independently and without an identifiable cause. Causes can be gene alterations and mutations in the genetic material. In addition, genetic damage accumulates over time, which is why the risk of MDS increases with age.

Nevertheless, the blood disorder can also occur secondarily and manifest due to a trigger. These triggers include radiation exposure, exposure to toxins such as benzenes, and the post-cancer state with necessary radiation or chemotherapy. In about ten percent of cases, the disease can be observed in connection with former cancer therapy.

Symptomatically, the patients are noticed due to the loss of one, several, or all blood cell rows. Various symptoms manifest this. The red blood cells transport the oxygen absorbed through the lungs to the organs. If red blood cells are deficient, patients notice fatigue, exhaustion, pallor, drop in performance, shortness of breath on exertion, and dizziness. A deficiency of white blood cells leads to an increased susceptibility to infections with a weakened immune system. Platelets, also called thrombocytes, are responsible for the body's blood clotting system. If platelets are lacking, there is an increased tendency to bleed, with increased bruising, small hemorrhages in the skin, bleeding from the gums and nose, and increased menstrual bleeding.

Diagnostics

Diagnosis is based on a detailed patient interview and a physical examination. In addition, a blood smear can be taken from a vein. In this way, the blood count can be examined more closely, and anemia can be diagnosed. In any case, other possible diagnoses of anemia should be excluded, among others, deficiency in iron, vitamin B12, and folic acid. A bone marrow puncture can now be carried out for a more accurate assessment, usually taken from the patient's iliac crest. Likewise, a cytogenetic examination can provide more detailed information about the disease; in some cases, chromosomal changes can be detected.

Based on the appearance of the dysplastic cells under the microscope, MDS is divided into different subgroups. This has an impact on the therapy decision.

MDS Therapy

Depending on the form of MDS, patients are divided into low-risk and high-risk groups. Based on the number of blasts in the blood, the general condition, age, and other blood values, the best possible form of therapy can now be decided. Patients with very low risk can generally be treated initially with an observational, wait-and-see therapy regimen. However, stem cell transplantation is the best therapeutic option in high-risk cases with an excellent general condition and young age. It is also the only possibility to cure MDS. However, this should be discussed in advance, as stem cell transplantation is an intensive and stressful treatment that may involve risks and serious complications. Other options are a downregulation of the immune system, the so-called immunosuppression, drugs that modulate the immune system, or the implementation of chemotherapy. This is decided very individually based on the patient's disease state. Generally, there may be a need for blood transfusions. The complementary therapy also includes the direct treatment of possible infections with antibiotics and sufficient vaccination protection with necessary additional vaccinations to avoid severe courses of infection.

Chances of Cure and Prognosis

The only chance to cure MDS is stem cell transplantation. Prognostic statements about the course of the disease must be made very individual and depend on many factors. The disease can remain stable for a long time in some cases; in other cases, a rapid transition into leukemia is observed. Likewise, the anemia with a corresponding loss of function of the blood cell series can lead to the patient's death due to possible serious infections. The treating physician can make a predictive assessment based on validated scoring systems that include different factors.

Which Physicians and Clinics Are Specialized in MDS?

Hematologists and oncologists from the Department of Internal Medicine deal with the clinical picture of myelodysplastic syndrome. Therapy is carried out on an interdisciplinary basis in facilities that specialize in treating hematopoietic system diseases.

Every patient who needs a doctor wants the best medical care. Therefore, the patient is wondering where to find the best clinic. As this question cannot be answered objectively and a reliable doctor would never claim to be the best one, we can only rely on the doctor's experience.

We will help you find an expert for your condition. All listed doctors and clinics have been reviewed by us for their outstanding specialization in myelodysplastic syndrome and are awaiting your inquiry or treatment request.

Sources:

• Germing U. Myelodysplastische Syndrome (niedriges Risiko). In: Kreuzer K, Hrsg. Myelodysplastische Syndrome (niedriges Risiko). 1. Auflage. Stuttgart: Thieme; 2018. doi:10.1055/b-004-140282
• www.uniklinik-ulm.de/innere-medizin-iii/haematologie/myelodysplastisches-syndrom-mds.html
• www.kompetenznetz-leukaemie.de/content/patienten/leukaemien/mds/
• www.kinderkrebsinfo.de/erkrankungen/leukaemien/myelodysplastisches_syndrom__mds/kurzinformation/index_ger.html
• www.mds-register.de
• www.onkopedia.com/de/onkopedia/guidelines/myelodysplastische-syndrome-mds/@@guideline/html/index.html