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Myelodysplastic Syndrome Development In Sickle Cell Disease

Myelodysplastic syndrome MDS, is a medical defect of a heterogeneous group of closely related clonal hematopoietic disorders, all of which are characterized by one or more peripheral blood cytopenias. 

A myelodysplastic syndrome is a collective group of disorders that causes the disruption of the production of blood cells. This is also caused by blood cells that are poorly formed or do not work properly. Bone marrows are usually hypercellular; they also display aberrant morphology and maturation (dysmeylopoieisis) that results in inefficient and ineffective blood cell production.

The myelodysplastic syndrome affects hematopoiesis at the stem cell level; this is indicated by cytogenetic abnormalities, molecular mutations, morphologic abnormalities, physiologic abnormalities in maturation, and differentiation of one or more of the hematopoietic cell lines. The myelodysplastic syndrome may one, or two, or all three myeloid hematopoiesis cell lineages namely; erythrocytic, granulocytic, and megakaryocytic, depending on the subtype and stage of the disease. 

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Photo Credit: Blood cancer cure

Sickle cell disease patients with myelodysplastic syndrome MDS, may show clinical manifestations of anemia, thrombocytopenia, and or neutropenia. The myelodysplastic syndrome occurs from something amiss in the inside of the bones where the blood cells are made from the bone marrow. 

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Photo Credit: Reblozyl

The proper management and care often slows down the disease, eases the symptoms, and prevents complications. Common measures of treatment include blood transfusions and medications to boost blood cell production. In most cases, patients would require a bone marrow transplant, also known as a stem cell transplant, which is only possible when both the donor and recipient are compatible. 

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Photo Credit: New England Journal of Medicine

The symptoms seen in sickle cell disease patients with a developed case of the myelodysplastic syndrome are; fatigue, shortness of breath, unusual paleness (pallor) which occurs due to a low red blood cell count (anemia), easy or unusual bruising or bleeding which occurs due to a low blood platelet count (thrombocytopenia), pinpoint-sized red spots just beneath the skin that are caused by bleeding (petechiae), and frequent infections which occur due to a low white blood cell count (leukopenia). 

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Photo Credit: MD Anderson Cancer Center

Content created and supplied by: Dr-Kikiope (via Opera News )


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