Our bodies are a huge enigmatic maze of wonders, with all our organs, arteries, muscles, joints, nerves, and even the tiniest fiber of our being working in unison to keep everything running smoothly.
Blood makes up 8% of our total body weight, and the various roads of arteries and vessels are an almost perfect circuitry until something goes wrong and we get a problem. In this case, MDS is one of the scene-stealing bad guys with an origin unknown.
Myelodysplastic syndrome (MDS) is a condition of the bone marrow, the spongy tissue inside bones that produces all blood cells and blood. The formation of normal blood cells such as platelets (the blood’s clotting mechanism) and white cells (immune cells that fight infection) is disrupted by this disorder.
MDS in childhood is a set of clonal bone marrow disorders characterized by peripheral cytopenia, dysplastic alterations in the bone marrow, and inefficient hematopoiesis.
It has a proclivity to evolve into acute myeloid leukemia (AML). Thus, MDS diseases have been dubbed “pre-leukemias”. Because of this danger, bone marrow transplantation is the most usual treatment.
MDS is uncommon in children, with just 1-4 occurrences per million children afflicted. Adults, particularly the elderly, are more likely to develop the condition, ranging from an acute, swiftly lethal sickness to a chronic, indolent illness.
However, in children, MDS is frequently seen in conjunction with genetic disorders and inherited bone marrow failure syndromes. Of particular concern is that, until recently, many patients with refractory anemia were not diagnosed early in the disease due to a lack of consensus on diagnostic criteria. As a result, some people die because of complications that go undiagnosed. Alternatively, a small number of infants with chronic unresponsive anemia die of frank leukemia because of the progression of their condition.
The cause of the myelodysplastic syndrome is still unknown, but MDS is more common in children with certain other medical problems, such as neurofibromatosis.
Symptoms, Tests, and Treatment
Being the ever-vigilant parents that we are, we tend to take a closer look at our children’s routines their behaviors and would notice anything amiss, strange, or different immediately, most especially in their bodies.
So, how would we suspect MDS in our children?
Symptoms
The most prevalent indications in youngsters are bruising owing to a low platelet count (the blood’s clotting mechanism) and recurrent infections. An enlarged spleen causes abdominal distension in some children.
As with any medical issue, an expert should be consulted so that a proper diagnosis can be made to determine whether your child has a disease.
Tests
Myelodysplastic syndrome is diagnosed by examining the peripheral blood (blood that circulates in veins and arteries) and the bone marrow. Genetic anomalies frequently accompany these illnesses in cells, which may help predict therapy response and outcome.
MDS is more difficult to identify than other bone marrow disorders, and it requires a highly skilled physician and pathologist to examine bone marrow specimens from children to make an accurate diagnosis. As a result, the diagnostic procedure may take some time. Additional blood and bone marrow testing are occasionally required to confirm the diagnosis.
Additional bone marrow tests will be performed to validate the diagnosis and the disorder’s particular cellular characteristics. An observation period may be required prior to the provision of therapy if the diagnosis is not obvious at the time of the tests.
Treatments
MDS is usually treated with supportive care, which helps control and treat the disease’s symptoms but does not cure it. Red cell and platelet transfusions to alleviate anemia symptoms and avoid bleeding. Because individuals with MDS are more susceptible to infections, any fevers should be evaluated by a health care practitioner. When a child has a fever, antibiotic treatment is often required and vital.
MDS in children is virtually always curable only through a bone marrow transplant, also known as a hematopoietic stem cell transplant (HSCT). HSCT involves high doses of chemotherapy or radiation therapy to kill all healthy and sick cells in the bone marrow. Healthy cells from another person’s bone marrow — either a family (typically a sibling) or an unrelated person — are infused into the patient to replace bone marrow that has been destroyed by chemotherapy or radiation therapy.
MDS develops into acute myelogenous leukemia in roughly one-third of individuals, usually within months to a few years.
MDS has a terrible prognosis without a stem cell transplant. Children diagnosed with MDS require ongoing follow-up care to assess therapy response, detect recurring disease, and manage drug side effects. Chemotherapy side effects, as well as second malignancies, are possible in MDS survivors.
Most often, the healthcare team would meet to analyze and discuss what they’ve discovered about your child’s condition after completing all necessary testing. After that, they’ll meet with you and your family to go over the results and discuss the best MDS treatment options or MDS clinical trials closest to your area for you to participate in.