Myelodysplastic Syndromes (MDS)

Myelodysplastic Syndromes (MDS)
Updated: 05/31/2018
© Jun Wang, MD, PhD

General features
  • Clonal disorders of marrow stem cells
  • Ineffective or disorderly hematopoiesis
  • Usually older patients
  • Need to be ruled out in any adults with unexplained cytopenias or monocytosis
  • Diagnostic approaches include CBC, bone marrow biopsy/aspirate, cytogenetics analysis, molecular studies including FISH, single nucleotide polymorphism arrays, DNA sequencing
Clinical presentations
  • Associated with lineage affected
  • Anemia
  • Defected platelet aggregation
Key risk factors
  • Cytotoxic chemotherapy
  • Radiation
  • Viral infection
  • Genotoxic chemicals, such as benzene
Key Laboratory findings
  • Increased cellularity in marrow with decreased circulating blood cells
  • Dysplasia in one or more myeloid cell lines
  • < 20% blasts
Key morphological features
Genetic abnormalities
  • Balanced chromosomal abnormality with fusion of oncogenes
  • Complex karyotypes
  • Normal karyotype may present
Current classification
  • Refractory anemia (RA): <5% blasts, <15% ringed sideroblasts, unilineage dysplasia >10% in one myeloid lineage
  • RA with Ringed Sideroblasts (RARS): 15% or more sideroblasts, less than 5% blasts, erythroid dysplasia only
  • Refractory cytopenia with multilineage dysplasia (RCMD): cytopenia, <5% blasts, dysplasia in >10% of cells in two or more lineages, no Auer rods
  • RA with Excess Blasts (RAEB): Unilineage or multilineage dysplasia with 5-9% blasts, no Auer rods in Type 1; or 10-19% blasts, Auer rods may be present in Type 2
  • MDS with isolated 5q- syndrome: anemia, <5% blasts, no Auer rods, hypolobulated megakaryocytes
  • Unclassified: cytopenia(s), dysplasia in <10% of cells in one or more myeloid cell lines, accompanied by cytogenetics abnormalities with presumptive evidence of MDS, <5% blasts
  • Therapy related MDS


Back to myeloid neoplasms
Back to contents

Comments

Post a Comment

Popular posts from this blog

Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4.  Benign blood cell disorders Anemia Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas M
Read more

Anemia

Anemia Updated: 07/13/2024 © Jun Wang, MD, PhD   General features Decrease in red cell mass Hemoglobin: Levels of hemoglobin in a specific volume of whole blood Hematocrit: Fraction of whole blood composed of red blood cells Red cell count: Quantity of red cell in a specific volume of whole blood Three major groups based on RBC sizes Microcytic Normocytic Macrocytic Three major causes Blood loss: acute or chronic Increased red cell destruction: hemolysis Reduced production: marrow failure due to genetic abnormality or nutritional deficiency Etiology Genetic abnormality Hemoglobinopathies RBC cytoskeletal abomorlities Enzyme abnormalities Fanconi anemia Nutritional deficiencies: iron, VB12, folate, general malnutrition Physical etiologies: trauma, burns, frostbite, prosthetic valves, etc Chronic diseases: chronic infections, neoplasia, autoimmune disorders, kidney or liver diseases, etc Infectious diseases: hepatitis, sepsis , malaria, etc
Read more

Practice questions Anemia I

Image
Practice questions Anemia I © Jun Wang, MD, PhD   Abbreviations: Hb: hemoglobin HCT: hematocrit MCV: Mean Corpuscular Volume MCHC: Mean Corpuscular Hemoglobin RDW: Red Cell Distribution Width TIBC: total iron binding capacity   1. Use this case for the next three questions . A 25-year-old woman is brought to the emergency department 2 hours after being involved in a motor vehicle accident. She is conscious but feels dizziness and weakness.   Her blood pressure is 90/60 mmHg, and her heart rate is 120 beats per minute. Physical examination reveals pale skin and mucosa and a 15 cm laceration on her left thigh with significant bleeding. What are most likely the CBC and TIBC results? A. Low Hb, low HCT, high MCV, high RDW, high TIBC B. Low Hb, low HCT, slightly low MCV, normal RDW, normal TIBC C. Low Hb, low HCT, slightly high MCV, normal RDW, normal TIBC D. Normal Hb, normal HCT, normal MCV, normal
Read more