Myelodysplastic syndrome

myelodysplasiamyelodysplastic syndromesMDSmyelodysplasticmyelodysplastic syndrome (MDS)preleukemia Myelodysplastic syndromes (MDS)5q-syndromeanemia, refractoryanemia, refractory, with excess of blasts
Myelodysplastic syndromes (MDS) are a group of cancers in which immature blood cells in the bone marrow do not mature and therefore do not become healthy blood cells.wikipedia
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Lenalidomide

RevlimidCC-5013lenaldomide
Drug therapy may include the medication lenalidomide, antithymocyte globulin, and azacitidine. The recognition of epigenetic changes in DNA structure in MDS has explained the success of two (namely the hypomethylating agents 5-azacytidine and decitabine) of three (the third is lenalidomide) commercially available medications approved by the U.S. Food and Drug Administration to treat MDS. By 2005, lenalidomide, a chemotherapy drug, was recognized to be effective in MDS patients with the 5q- syndrome, and in December 2005, the US FDA approved the drug for this indication.
Lenalidomide, sold under the trade name Revlimid among others, is a medication used to treat multiple myeloma (MM) and myelodysplastic syndromes (MDS).

Acute myeloid leukemia

acute myelogenous leukemiaacute myeloid leukaemiaAML
Some types may develop into acute myeloid leukemia.
Risk factors include smoking, previous chemotherapy or radiation therapy, myelodysplastic syndrome, and exposure to the chemical benzene.

Decitabine

5-aza-2' deoxycitidine5-aza-2-deoxycytidine5-aza-20-deoxycytidine
The recognition of epigenetic changes in DNA structure in MDS has explained the success of two (namely the hypomethylating agents 5-azacytidine and decitabine) of three (the third is lenalidomide) commercially available medications approved by the U.S. Food and Drug Administration to treat MDS.
It is a drug for the treatment of myelodysplastic syndromes, a class of conditions where certain blood cells are dysfunctional, and for acute myeloid leukemia (AML).

Sideroblastic anemia

X-linked sideroblastic anemiaAnemia, sideroblasticringed sideroblast
Children with Down syndrome are susceptible to MDS, and a family history may indicate a hereditary form of sideroblastic anemia or Fanconi anemia.
The disorder may be caused either by a genetic disorder or indirectly as part of myelodysplastic syndrome, which can develop into hematological malignancies (especially acute myeloid leukemia).

Chromosome 5q deletion syndrome

5q- syndrome5q deletion syndrome5q-
By 2005, lenalidomide, a chemotherapy drug, was recognized to be effective in MDS patients with the 5q- syndrome, and in December 2005, the US FDA approved the drug for this indication.
This chromosome abnormality is most commonly associated with the myelodysplastic syndrome.

Azacitidine

5-azacytidineazacytidineVidaza
Drug therapy may include the medication lenalidomide, antithymocyte globulin, and azacitidine. The recognition of epigenetic changes in DNA structure in MDS has explained the success of two (namely the hypomethylating agents 5-azacytidine and decitabine) of three (the third is lenalidomide) commercially available medications approved by the U.S. Food and Drug Administration to treat MDS.
Azacitidine and its deoxy derivative, decitabine (also known as 5-aza-2′-deoxycytidine), are used in the treatment of myelodysplastic syndrome.

GATA2

GATA-22
GATA2 deficiency is a group of disorders caused by a defect, familial or sporadic inactivating mutations, in one of the two GATA2 genes.
GATA2 deficiency often begins with seemingly benign abnormalities but if untreated progresses to life-threatening opportunistic infections, virus-induced cancers, lung failure, the myelodysplastic syndrome (i.e. MDS), and/or acute myeloid leukemia, principally acute myeloid leukemia (AML), less commonly chronic myelomonocytic leukemia (CMML), and rarely a lymphoid leukemia.

International Prognostic Scoring System

IPSS
Patients with isolated 5q-, low IPSS risk, and transfusion dependence respond best to lenalidomide.
The International Prognostic Scoring System (IPSS), published in 1997, is used by many doctors to help assess the severity of a patient's myelodysplastic syndrome (MDS).

Iron overload

haemochromatosisiron overload disorderhemochromatosis
Long-term transfusion of packed red blood cells leads to iron overload.

Cyclophosphamide

(C)'''yclophosphamidealdophosphamideCytoxan
Some people have a history of exposure to chemotherapy (especially alkylating agents such as melphalan, cyclophosphamide, busulfan, and chlorambucil) or radiation (therapeutic or accidental), or both (e.g., at the time of stem cell transplantation for another disease).
When AML occurs, it is often preceded by a myelodysplastic syndrome phase, before developing into overt acute leukemia.

Fanconi anemia

Fanconi anaemiaFanconi's anaemiaFanconi anemia C
Children with Down syndrome are susceptible to MDS, and a family history may indicate a hereditary form of sideroblastic anemia or Fanconi anemia. Other pre-existing bone marrow disorders like acquired aplastic anemia following immunosuppressive treatment and Fanconi anemia can evolve into MDS.
As FA is now known to affect DNA repair, specifically homologous recombination, and given the current knowledge about dynamic cell division in the bone marrow, finding patients are more likely to develop bone marrow failure, myelodysplastic syndromes, and acute myeloid leukemia (AML) is not surprising.

Anemia

anaemiaanemicanaemic
Problems with blood cell formation result in some combination of low red blood cells, low platelets, and low white blood cells.
Refractory anemia, an anemia which does not respond to treatment, is often seen secondary to myelodysplastic syndromes.

Hypomethylating agent

DNMT inhibitorDNA methyltransferase inhibitorDNA methyltransferase inhibitors
The recently approved DNA methyltransferase inhibitors take advantage of this mechanism by creating a more orderly DNA methylation profile in the hematopoietic stem cell nucleus, and thereby restoring normal blood counts and retarding the progression of MDS to acute leukemia.
Currently two members of the class, azacitidine and decitabine are FDA-approved for use in the United States in myelodysplastic syndrome and are being investigated for use in a number of tumors.

Copper deficiency

Coppercopper deficienthypocupremia
Copper deficiency can have many hematological consequences, such as myelodysplasia, anemia, low white blood cell count, and low count of neutrophils (a type of white blood cell that is often called "the first line of defense" of the immune system).

Chromosome 5

chromosome five5Chromosome 5 (human)
Since at least 1974, the deletion in the long arm of chromosome 5 has been known to be associated with dysplastic abnormalities of hematopoietic stem cells.

Chronic myelomonocytic leukemia

chronic myelomonocytic leukaemiachronic myelomonocyticCLL
CMML shows characteristics of a myelodysplastic syndrome (MDS); a disorder that produces abnormal looking blood cells, and a myeloproliferative neoplasm (MPN); a disorder characterised by the overproduction of blood cells.

Blood

human bloodhematologicaloxygen consumption
The GATA2 protein is a transcription factor critical for the embryonic development, maintenance, and functionality of blood-forming, lympathic-forming, and other tissue-forming stem cells.

Aplastic anemia

aplastic anaemiaaplasticacquired aplastic anemia
Other pre-existing bone marrow disorders like acquired aplastic anemia following immunosuppressive treatment and Fanconi anemia can evolve into MDS.
In addition, 10–15% of severe aplastic anemia cases evolve into myelodysplastic syndrome and leukemia.

Thrombocytopenia

low platelet countthrombocytopaenialow platelets
Problems with blood cell formation result in some combination of low red blood cells, low platelets, and low white blood cells.

Refractory cytopenia of childhood

Refractory cytopenia of childhood is a subgroup of myelodysplastic syndrome (MDS), having been added to the World Health Organization classification in 2008.

Myelodysplastic–myeloproliferative diseases

myelodysplastic/myeloproliferative disordermyelodysplastic–myeloproliferative syndromemyelodysplastic-myeloproliferative
Myelodysplastic–myeloproliferative diseases are a category of hematological malignancies disorders which have characteristics of both myelodysplastic and myeloproliferative conditions.

Hematopoietic stem cell transplantation

bone marrow transplantbone marrow transplantationstem cell transplant
Treatments may include supportive care, drug therapy, and stem cell transplantation.
Other conditions treated with stem cell transplants include sickle-cell disease, myelodysplastic syndrome, neuroblastoma, lymphoma, Ewing's sarcoma, desmoplastic small round cell tumor, chronic granulomatous disease, Hodgkin's disease and Wiskott–Aldrich syndrome.

Dyserythropoiesis

The features generally used to define a MDS are blood cytopenias, ineffective hematopoiesis, dyserythropoiesis, dysgranulopoiesis, dysmegakaropoiesis, and increased myeloblasts.
Acquired causes include nutrient deficiency/malnutrition (e.g. cobalamine, folate, and iron), myelodysplasia, HIV infection, and certain medications (e.g. zidovudine).

Refractory anemia with excess of blasts

Refractory anemia with excess blastsRefractory anemia with excess blasts in transformationsmoldering leukemia
Refractory anemia with excess of blasts (RAEB) is a type of myelodysplastic syndrome with a marrow blast percentage of 5% to 19%.