Myelodysplastic Syndromes
- Date: 2007-04-23 - Word Count: 551
Share This!
Myelodysplastic syndrome (MDS) refers to a group of clonal stem cell disorders characterized by maturation defects resulting in ineffective hematopoiesis and an increased risk of transformation to AML. In patients with MDS, the bone marrow is partly or wholly replaced by the clonal progeny of a mutant multipotent stem cell that retains the capacity to differentiate into red cells, granulocytes, and platelets, but in a manner that is both ineffective and disordered. As a result, the bone marrow is usually hypercellular or normocellular, but the peripheral blood shows pancytopenia. MDS arises in two distinct settings:
Idiopathic or primary MDS,occuring mainly in patients over age 50, often develops insidiously.
Therapy related MDS (t-MDS), a complication of previous myelosuppressive drug or radiation therapy, usually appears 2 to 8 years after exposure.
All forms of MDS can transform to AML, but transformation occurs most rapidly and with highest frequency in patients with t-MDS. Although characteristic morphologic changes are typically seen in the marrow and the peripheral changes are typically seen in the marrow and the peripheral blood, definitive diagnosis frequently requires correlation with other laboratory tests. Cytogenetic analysis is particularly helpful in confirming the diagnosis becauswe certain chromosomal aberrations are often observed.
The pathogenesis of MDS is unknown. Although the marrow is usually hypercellular at diagnosis it may also be normocellular or , less commonly , hypocellular. Given this, the usual explanation for suppression of normal hematopoiesis (crowding out of normal elements) is difficult to apply , leading to the suggestion that MDS may arise out of a background of stem cell damage. Both primary MDS and t-MDS occuring after exposure to radiation or alkylating chemotherapeutic drugs are associated with similar clonal chromosomal abnormalities, including monosomy 5 and monosomy 7 deletions of 5q and 7q trisomy 8 and deletions of 20q
Clinical Course
Primary MDS affects primarily individuals older than 60 years of age. As in acute leukemia patients with this disorder present with weakness, infections, and hemorrhages, all owing to pancytopenia. Approximately half of the patients are asymptomatic, and the disease is discovered after incidental blood tests.
On the basis of specific morphologic features in the marrow and peripheral blood , primary MDS is divided into five categories, each with a somewhat different risk for transformation to overt AML. In general subtypes that are defined by having a higher proportion of blast cells in the marrow or peripheral blood are associated with a poorer prognosis. The presence of multiple clonal chromosomal abnormalities and the severity of peripheral blood cytopenias are independent risk factors that also portend a worse outcome.
The median survival in primary MDS varies from 9 to 29 months, but some individuals in good prognostic groups may live for 5 years or more. Overall, progression to AML occurs in 10 to 40% of individuals and is often accompanied by the appearance of additional clonal cytogenetic changes. Other patients succumb to complications of thrombocytopenia (bleeding) and neutropenia (infection). The outlook is even more grim in ptients with t-MDS, who have an overall median survival of only 4 to 8 months. Cytoopenias tend to be more severe than in primary MDS, and many patients progress rapidly to AML.
Treatment options in MDS are limited. In younger patients, allogeneic bone marrow transplantation offers some hope for reconstitution of normal hematopoiesis and long term survival. Older patients with MDS are treated supportively with antibiotics and blood product transfusions.
Idiopathic or primary MDS,occuring mainly in patients over age 50, often develops insidiously.
Therapy related MDS (t-MDS), a complication of previous myelosuppressive drug or radiation therapy, usually appears 2 to 8 years after exposure.
All forms of MDS can transform to AML, but transformation occurs most rapidly and with highest frequency in patients with t-MDS. Although characteristic morphologic changes are typically seen in the marrow and the peripheral changes are typically seen in the marrow and the peripheral blood, definitive diagnosis frequently requires correlation with other laboratory tests. Cytogenetic analysis is particularly helpful in confirming the diagnosis becauswe certain chromosomal aberrations are often observed.
The pathogenesis of MDS is unknown. Although the marrow is usually hypercellular at diagnosis it may also be normocellular or , less commonly , hypocellular. Given this, the usual explanation for suppression of normal hematopoiesis (crowding out of normal elements) is difficult to apply , leading to the suggestion that MDS may arise out of a background of stem cell damage. Both primary MDS and t-MDS occuring after exposure to radiation or alkylating chemotherapeutic drugs are associated with similar clonal chromosomal abnormalities, including monosomy 5 and monosomy 7 deletions of 5q and 7q trisomy 8 and deletions of 20q
Clinical Course
Primary MDS affects primarily individuals older than 60 years of age. As in acute leukemia patients with this disorder present with weakness, infections, and hemorrhages, all owing to pancytopenia. Approximately half of the patients are asymptomatic, and the disease is discovered after incidental blood tests.
On the basis of specific morphologic features in the marrow and peripheral blood , primary MDS is divided into five categories, each with a somewhat different risk for transformation to overt AML. In general subtypes that are defined by having a higher proportion of blast cells in the marrow or peripheral blood are associated with a poorer prognosis. The presence of multiple clonal chromosomal abnormalities and the severity of peripheral blood cytopenias are independent risk factors that also portend a worse outcome.
The median survival in primary MDS varies from 9 to 29 months, but some individuals in good prognostic groups may live for 5 years or more. Overall, progression to AML occurs in 10 to 40% of individuals and is often accompanied by the appearance of additional clonal cytogenetic changes. Other patients succumb to complications of thrombocytopenia (bleeding) and neutropenia (infection). The outlook is even more grim in ptients with t-MDS, who have an overall median survival of only 4 to 8 months. Cytoopenias tend to be more severe than in primary MDS, and many patients progress rapidly to AML.
Treatment options in MDS are limited. In younger patients, allogeneic bone marrow transplantation offers some hope for reconstitution of normal hematopoiesis and long term survival. Older patients with MDS are treated supportively with antibiotics and blood product transfusions.
Related Tags: stress, depression, treatment, medical, symptoms, disease, bronchiectasis
For more information about disease and treatment visit www.medicalhealthcenter.net Your Article Search Directory : Find in Articles
Recent articles in this category:
- How Safe Are Prescription Topical Non - Steroidal Drugs... Particularly in the Elderly?
The treatment of osteoarthritis, to date, is essentially palliative.Osteoarthritis is a degenerative - The Biggest Acne Myths Dispelled
Acne scars are sort of like being hit while you are down. First you live with the misery of the init - Natural Cure For Eczema - Become Eczema Free, The Natural Way!
When it comes to eczema, doctors' tend to have little advice to offer other than using creams and lo - Childhood Obesity Cured With Hypnosis
It is very unfortunate when a child is suffering from obesity. It is a very major medical problem th - Children Changing With Hypnosis
The challenges we face everyday in the world may cause difficulties in life for not only adults, but - Don't Suffer in Silence - Remove Your Warts and All
Many people suffer with skin conditions that can leave them worried, scared and feeling like they co - Treat Red and Irritated Skin Easily
Wash your skin with a gentle cleanser, apply a cold compress, soak in a warm oatmeal bath, apply Alo - How to Effectively Treat Stretch Marks to Diminish Them
Stretch marks or striae appear as red or purple marks on the skin and are actually a form of scarrin - Medicine: A Science and Art
Medicine is all about dealing with the healing human ailments. It is both a science as well as art a - Six Important Facts About Babesia
Babesia, which causes Babesiosis, generally requires two hosts throughout its lifespan. It originate
Most viewed articles in this category:
- Sinus Headache Relief
Sinus headache is a result of inflammation in the passage behind the cheeks, nose and eyes. It cause - Simple Lifestyle Changes That Can Help You Get Rid Of Acne
As anyone who has ever suffered knows, acne can be a debilitating condition, causing humiliation and - Acne Treatment For Children
Acne is a very common thing for children and adolescents especially between the ages of nine and sev - Coping With An Ileostomy Due To Crohns Disease
Crohn's sufferers that have constipation due to a stricture, have a severe case of Crohn's that may - Are There Home Remedies For Acid Reflux?
Acid Reflux or heart burn as it is commonly called is a very unpleasant condition in which the liqui - How To Relieve Sinus Pressure
Swollen, inflamed sinuses are, to put it mildly, very uncomfortable. Sinuses get infected and inflam - The Pain Of The Psoriasis Patient
Psoriasis is one of the most difficult diseases people have to deal with, effecting many areas in th - Lipitor Memory Loss: When Memories Vanish, What Can You Do?
Lipitor memory loss has been experienced by patients taking this statin medication to reduce cholest - Are Discount Vitamins For Everyone?
And the news is --- discount vitamins really are for just about everyone! Young, old, male, female, - Are Pharmaceutical Sales Reps Really Needed?
Aside from a few free samples, some cheap pens and calendars, or maybe a sweet magnet to put on your