Myelodysplastic syndrome overview
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Differentiating Myelodysplastic syndrome from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]
Overview
The myelodysplastic syndromes was first described in 1900 by Leube.[1] Myelodysplastic syndromes may be classified into several subtypes based on French-American-British (FAB) classification and World Health Organization (WHO) classification method.[2][3][4][5] Cytogenetic abnormalities involved in the pathogenesis of myelodysplastic syndrome include isolated deletion of 5q, monosomy 7, and monosomy 8.[6] Myelodysplastic syndrome is associated with Fanconi syndrome, Diamond-Blackfan anemia, Shwachman-Diamond syndrome.[7] There are no characteristic findings of myelodysplastic syndrome on gross pathology. On microscopic histopathological analysis, dyserythropoiesis, dysgranulopoiesis, and dysmegakaryocytopoiesis are findings of myelodysplastic syndrome.[6] The cause of primary myelodysplastic syndrome has not been identified.[3] Common causes of secondary myelodysplastic syndrome include radiation, busulfan, nitrosourea, procarbazine, DNA topoisomerase inhibitors, acquired aplastic anemia, and Fanconi's anemia.[3] Myelodysplastic syndrome must be differentiated from other diseases that cause anemia, neutropenia, and thrombocytopenia, such as: aplastic anemia, fanconi anemia, pure red cell aplasia, Shwachman-Diamond syndrome, paroxysmal nocturnal hemoglobinuria, parovirus B19 infection, and vitamin B12 defeciency.[8][9][10] The incidence of myelodysplastic syndrome is approximately 4.4 to 4.6 cases per 100,000 individuals in the United States.[11] Myelodysplastic syndrome commonly affects older patients.[11] Males are more commonly affected with myelodysplastic syndrome than females.[11] Myelodysplastic syndrome usually affects individuals of the Caucasian race.[11] Common risk factors in the development of myelodysplastic syndrome are past treatment with chemotherapy, radiation therapy, past exposure to tobacco smoke, ionizing radiation, organic chemicals, and heavy metals.[11]
Historical Perspective
Myelodysplastic syndrome was first described in 1900 by Leube.[1]
Classification
Myelodysplastic syndrome may be classified into several subtypes based on French-American-British (FAB) classification and World Health Organization (WHO) classification method.[2][3][4][5]
Pathophysiology
Cytogenetic abnormalities involved in the pathogenesis of myelodysplastic syndrome include isolated deletion of 5q, monosomy 7, and monosomy 8.[6] Myelodysplastic syndrome is associated with Fanconi syndrome, Diamond-Blackfan anemia, Shwachman-Diamond syndrome.[7] There are no characteristic findings of myelodysplastic syndrome on gross pathology. On microscopic histopathological analysis, dyserythropoiesis, dysgranulopoiesis, and dysmegakaryocytopoiesis are findings of myelodysplastic syndrome.[6]
Causes
The cause of primary myelodysplastic syndrome has not been identified.[3] Common causes of secondary myelodysplastic syndrome include radiation, busulfan, nitrosourea, procarbazine, DNA topoisomerase inhibitors, acquired aplastic anemia, and Fanconi's anemia.[3]
Differentiating Myelodysplastic syndrome from other Diseases
Myelodysplastic syndrome must be differentiated from other diseases that cause anemia, neutropenia, and thrombocytopenia, such as: aplastic anemia, fanconi anemia, pure red cell aplasia, Shwachman-Diamond syndrome, paroxysmal nocturnal hemoglobinuria, parovirus B19 infection, and vitamin B12 defeciency.[8][9][10]
Epidemiology and Demographics
The incidence of myelodysplastic syndrome is approximately 4.4 to 4.6 cases per 100,000 individuals in the United States.[11] Myelodysplastic syndrome commonly affects older patients.[11] Males are more commonly affected with myelodysplastic syndrome than females.[11] Myelodysplastic syndrome usually affects individuals of the Caucasian race.[11]
Risk Factors
Common risk factors in the development of myelodysplastic syndrome are past treatment with chemotherapy, radiation therapy, past exposure to tobacco smoke, ionizing radiation, organic chemicals, and heavy metals.[11]
Screening
Natural History, Complications and Prognosis
Diagnosis
History and symptoms
Symptoms of myelodysplastic syndrome include bleeding, easy bruising, and fatigue.[11]
Physical Examination
Laboratory Findings
CT
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical therapy
Surgery
Prevention
References
- ↑ 1.0 1.1 Nimer, S. D. (2008). "Myelodysplastic syndromes". Blood. 111 (10): 4841–4851. doi:10.1182/blood-2007-08-078139. ISSN 0006-4971.
- ↑ 2.0 2.1 Classification of myelodysplastic syndrome. Radiopaedia (2015). http://radiopaedia.org/articles/myelodysplastic-syndrome. Accessed on December 7, 2015
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Pathologic systems of myelodysplastic syndrome. National Cancer Institute (2015). http://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq/#link/_204_toc. Accessed on December 7, 2015
- ↑ 4.0 4.1 French-American-British (FAB) classification of myelodysplastic syndrome. Wikipedia (2015). https://en.wikipedia.org/wiki/Myelodysplastic_syndrome. Accessed on December 7, 2015
- ↑ 5.0 5.1 World Health Organization classification of myelodysplastic syndrome. Wikipedia (2015). https://en.wikipedia.org/wiki/Myelodysplastic_syndrome. Accessed on December 8, 2015
- ↑ 6.0 6.1 6.2 6.3 Cytogenetics of myelodysplastic syndromes. Librepathology (2015). http://librepathology.org/wiki/index.php/Myelodysplastic_syndromes. Accessed on December 8, 2015
- ↑ 7.0 7.1 Associations of myelodysplastic syndromes. Librepathology (2015). http://librepathology.org/wiki/index.php/Myelodysplastic_syndromes. Accessed on December 8, 2015
- ↑ 8.0 8.1 Differential diagnosis of myelodysplastic syndromes. Librepathology (2015). http://librepathology.org/wiki/index.php/Myelodysplastic_syndromes. Accessed on December 9, 2015
- ↑ 9.0 9.1 Merrill, Andrea L.; Smith, Hedy (2011). "Myelodysplastic Syndrome and Autoimmunity: A Case Report of an Unusual Presentation of Myelodysplastic Syndrome". Case Reports in Hematology. 2011: 1–4. doi:10.1155/2011/560106. ISSN 2090-6560.
- ↑ 10.0 10.1 DeZern, A. E.; Sekeres, M. A. (2014). "The Challenging World of Cytopenias: Distinguishing Myelodysplastic Syndromes From Other Disorders of Marrow Failure". The Oncologist. 19 (7): 735–745. doi:10.1634/theoncologist.2014-0056. ISSN 1083-7159.
- ↑ 11.00 11.01 11.02 11.03 11.04 11.05 11.06 11.07 11.08 11.09 11.10 Incidence and mortality of myelodysplastic syndromes. National Cancer Institute 2015. http://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq#link/_291_toc. Accessed on December 3, 2015