Myelodysplastic Syndromes MDS Flashcards
Therapies related MDS
( radiotherapy , some chemotherapies)
Secondary MDS due to
combination of radiation ☢️ +alkylating agents
DNA topoisomerase inhibitors
…………. And ………….And ……………. Can evolve into MDS
Aplastic anemia
Fanconi anemia
PNH
Cytogenetic abnormalities
11q23 following topoisomerase II inhibitors
Trisomy 8 MDS.
Sideroblastic anemia
responds to immunosuppressive therapy
(Eight/Hate / مثبط مناعة)
mutations in mitochondrial genes» ineffective erythropoiesis and disordered iron metabolism
Clinical Overlap / Associations
AML
Aplastic anemia
Myeloproliferative disease
LGL leukemia
Autoimmune diseases
Acute neutrophilic dermatitis
Sweet ‘s syndrome
…………..may become large and lack granules
Platelets
……………. contain Döhle bodies; and may be functionally deficient
Neutrophils
The total (WBC) count is usually normal or low, except in ……………..
CMML
الوحيدات ليلا فرة زياي بوود
Circulating…………… usually correlate with marrow blast numbers
myeloblasts
BM At MDS
Megaloblastic nuclei +ringed sideroblasts in the erythroid lineage;
hypogranulation and hyposegmentation in granulocytic precursors, with an increase in myeloblast
megakaryocytes showing reduced numbers or disorganized nuclei.
The prognosis strongly correlates with the proportion of .
Marrow blasts
Differential diagnosis
IF the BM shows ringed sideroblasts
vitamin B6 deficiency can be assessed by a therapeutic trial of pyridoxine
More difficult are the distinctions between hypocellular MDS and………….. or between refractory anemia with excess blasts and…………..
aplasia (A.A)
early acute leukemia
The WHO considers the presence of …………..in the marrow as the criterion that separates acute myeloid leukemia (AML) from MDS.
20 % blasts
Most patients die as a result of complications of…………..and not due to leukemic transformation; perhaps …………. succumb to other diseases unrelated to their MDS.
pancytopenia
One third
The outlook in therapy-related MDS, regardless of type, is extremely poor, and most patients progress within a few months to refractory AML.
The median survival varies greatly from years for patients with…………… to a few months in…………..
5q- or sideroblastic anemia
refractory anemia with excess blasts or severe pancytopenia associated with monosomy 7
TREATMENT OF MDS
Only stem cell transplantation offers cure.
but older patients are particularly prone to develop treatment related mortality and morbidity.
For older patient with MDS excess blast DNA Hypomethelating agent (HMA)Azacitidine OR Decitabine (more potent).
For MDS 5q- Lenalidomide
For low risk MDS (without blast) and HLA-DR15 +ve immunosuppression( ATG , cyclosporine ).
ATG+the anti-CD52 monoclonal antibody (Alemtuzumab) is especially effective in (younger than age 60 years) and who bear the HLA-DR15.
RBC transfusion support should be accompanied by iron chelation to prevent secondary hemochromatosis
