Flashcards in EXAM #3: MDS/MPD Deck (57)
What is MDS? What are the general characteristics of MDS?
MDS= Myelodysplastic Syndrome
- Dysplasia or bad growth of the bone marrow cells
- Increased number of cells i.e. hypercellular bone marrow with poor products
- Results in various cytopenias (low numbers)
- Blasts less than 20% in the bone marrow
****Greater than 20% blasts= leukemia*****
What is the major etiology of MDS?
Most myelodysplasias result from de novo mutations
What causes secondary MDS?
- Genetic factors
- Environmental factors
- Antineoplastic drugs
What are the signs and symptoms of MDS?
- Organomegaly/ Lymphadenopathy
- Sweet's Syndrome
What is Sweet's Syndrome?
Acute febrile neutrophilic dermatosis
How is MDS diagnosed?
Bone marrow biopsy
What are some of the cell types seen on PBS that are typical of MDS?
1) Giant platelets
2) Pseudo-Pegler Huet anomaly
3) Ringed Sideroblasts
List the differential diagnoses of MDS.
- Vitamin B12 and Folate Deficiency
- Viral infection e.g. HIV
- Lead toxicity
- Erythropoietic agents e.g. EPO or G-CSF
What types of chemotherapeutic agents cause MDS?
- Alkylating agents
- Topoisomerase inhibitors
Which class of chemotheraputic drugs can more quickly cause lead to MDS?
Which class of chemotheraputic drugs more commonly leads to MDS?
What is 5q-Syndrome? What patient population is this seen in?
Subtype of MDS that causes:
- Macrocytic anemia
- Platelet counts elevated
****This is seen in FEMALES****
What is the prognosis for 5q Syndrome?
What drug is used to treat 5q Syndrome?
What is CMML?
Chronic Myelomonocytic Leukemia
What are the characteristics of CMML?
- Increased monocytes
- Variable degrees of dysplasia
What drugs can be used to treat CMML?
What is hypocellular MDS?
MDS with clinical features that resemble aplastic anemia
What is low intensity treatment for MDS?
- Supportive care
- Growth factor support
What is high intensity treatment for MDS?
- Stem cell transplant
- Clinical trials
What are the "growth factors" that are used to treat MDS?
When does EPO tend to work better in patients with MDS?
When EPO level is already less than 500
In patients with MDS, what class of drugs have been shown to decrease time to leukemia and transfusion dependence?
Hypomethylating agents i.e. Azacytidine
What is the role of transplant in the treatment of MDS? What type of transplant is curative?
Allogeneic transplant is curative; however, patients have to be less than 40
What is a myeloproliferative disorder?
Overproduction of one or more blood cell line
What are the classic myeloproliferative disorders?
1) Chronic myeloid leukemia
2) Polycythemia vera
3) Essential thrombocytosis
4) Primary myelofibrosis
What mutation is seen with PV and ET?
What is the mutation seen in CML?
What are the symptoms of CML?