The myelodysplastic
syndromes (MDS) comprise a heterogeneous group of cancerous stem cell disorders
characterized by dysplastic (abnormal) and ineffective blood cell production
and a variable risk of transformation to acute leukemia. These disorders
may occur de novo or arise years after exposure to potentially mutagenic
therapy (eg, radiation exposure, chemotherapy).
The risk of developing
MDS increases with age. Signs and
symptoms at presentation of MDS are non-specific. Many patients are
asymptomatic, with the diagnosis established upon routine laboratory screening.
Others present with symptoms resulting from a previously unrecognized anemia,
including fatigue, weakness, exercise intolerance, angina, dizziness, cognitive
impairment, or an altered sense of well being.
While low white blood
cells count is largely responsible for the high incidence of infection in MDS,
granulocyte dysfunction (eg, impaired microbial killing) may also contribute.
Bacterial infections predominate; the skin is the most common site of
infection.
The 5q- syndrome — There is a special subtype of MDS
called 5q minues. It can be present in patients otherwise diagnosed as having
refractory anemia; however, its karyotypic and clinical distinctiveness sets it
apart from the other
In a study of 88 consecutive patients defined by the 2008 WHO
criteria, a relatively unique pattern was seen:
Female predominance with a
median age at diagnosis of 74 years
Transfusion-dependent anemia
Low incidence of neutropenia,
thrombocytopenia, infection, and bleeding
Normal or increased platelet
counts along with bone marrow hyperplasia of hypolobulated
micromegakaryocytes
Low incidence of transformation
into acute leukemia
How to
diagnose MDS: Other important causes of low blood counts need to be ruled out
including poor nutritional status, alcohol and drug use, copper level,
medications such as valproic acid, occupational exposure to toxic chemicals,
prior treatment with antineoplastic agents or radiotherapy, and risk factors
for and/or treatment of human immunodeficiency virus (HIV) infection should be
elicited and vitamin B12 and folate deficiency must be excluded.
Your doctor
will examine your blood under the microscope and ultimately will require a bone
marrow biopsy.
Prognostication
is based upon the IPSS score:
Percentage of Bone
Marrow Blasts
< 5 percent (0
points)
5 to 10 percent (0.5
points)
11 to 20 percent (1.5
points)
21 to 30 percent (2.0
points)
Karyotype
Normal, Y-, 5q-, 20q-
(0 points)
Abnormal chromosome 7
or 3 or more abnormalities (1.0 points)
No cytopenia or
cytopenia of 1 cell type (0 points)
Cytopenia of 2 or 3
cell types (0.5 points)
You are
given a score based up on the blasts, chromosome analysis and number of reduced
blood cells and your doctor will be able to roughly prognosticate the aggressiveness
of the MDS.
Treatment of
MDS:
Treatment options for patients with MDS typically fall into one of
three categories:
Supportive care includes the use of antibiotics for infection and red
cell and platelet transfusions in the setting of symptomatic anemia and
thrombocytopenia, respectively. Prophylactic antibiotics are generally not
helpful and this strategy may select for antibiotic resistance. Supportive
care is an important adjunct to the management of all patients with MDS
and can be considered as the sole treatment modality for a subset of
patients with lower risk MDS.
Low intensity therapies include hematopoietic growth factors, DNA
hypomethylating agents (vidaza or decitabine), immunosuppressive therapy,
and lenalidomide. These can be administered in the outpatient setting and
have a low risk of treatment-related morbidity and mortality. Low
intensity treatments can improve symptoms and quality of life, but are not
curative.
High intensity therapies include intensive combination chemotherapy and
allogeneic hematopoietic cell transplantation (HCT). They require
hospitalization and entail significant risk of treatment-related
mortality. However, these treatments may improve blood counts more quickly
than less intensive therapy, reduce the risk of death from MDS, and may
alter the MDS disease course.
Thanks uptodate.com
Here, Dr. Tony Talebi discusses “What is Myelodysplastic Syndrome?”
with Dr. Byrnes, professor of Hematology at the University of Miami. Discussion includes MDS prognosis, what causes MDS, signs of MDS, MDS symptoms, MDS, MDS stage 4, treatment of MDS, MDS association, stage four MDS, small cell MDS, symtoms of MDS, causes of MDS, MDS chemotherapy, what is MDS, MDSs, MDS information, MDS prevention, stage 4 MDS, information about MDS, stage iv MDS, MDS signs, MDS symptom, is MDS curable, survival rates for MDS, symptoms of MDS, MDS survivors, MDS symptons, MDS survival, treatments of MDS, symptons of MDS, MDS statistics, MDS, chemo for MDS, MDS survival rate, large cell MDS, effects of MDS, MDS screening, MDS diagnosis, MDS society, MDS clinical trials, MDS metastasis, survival rate MDS, symptom of MDS, info on MDS, new treatments for MDS, how common is MDS, vidaza, blood cell
growth factors, stem cell transplant for MDS, blood transfusions for MDS, IPSS score and treatment of MDS.
Dr Byrnes' crendentials:
Professor Hematology and Chief of Hematology/Oncology at the Miami VA Hospital.