MDS & MPD
Quiz yourself by thinking what should be in
each of the black spaces below before clicking
on it to display the answer.
Help!
|
|
||||
|---|---|---|---|---|---|
| Myelodysplastic Syndromes Overview ? | * A group of clonal stem cell disorders characterized by maturation defects
---Associated with ineffective hematopoiesis
---High risk of transformation to AML
--- can see anemias, infections, and bleeding---- blast count below 20%
🗑
|
||||
| MDS Testing ? | * CBC – pancytopenia
----Peripheral blood – dysplasia in one or all cell lines
----Bone marrow aspiration & biopsy
<20% blasts---- Flow cytometry – confirms absence of AML
----Cytogenetics ...... * nothing in blood can make Dx , need BM biopsy
🗑
|
||||
| MDS BM Morphology ? | * Erythroid dysplasia - see Ringed sideroblasts..... * Neutrophil dysplasia - Decreased secondary granules (hypogranular)and Pseudo Pelger-Huet cells .... * Megakaryocyte dysplasia - Myeloblasts MUST be less than 20%
and NO Auer rods
🗑
|
||||
| MDS PB Morphology ? | * Pseudo-Pelger-Huet cells
---Hypogranular neutrophils
---Giant platelets
---Macrocytes
---Poikilocytes (schistocytes and spherocytes are NOT seen)
---Relative or absolute monocytosis
---Myeloblasts MUST be <10% of the leukocytes
🗑
|
||||
| MDS WHO Classification ? | * Refractory cytopenia with unilineage dysplasia (RCUD)--
Refractory anemia, refractory neutropenia, refractory thrombocytopenia
<5% of cases
.....*Refractory anemia with Ringed Sideroblasts (RARS)
--- <5% of cases
🗑
|
||||
| continued.... | * Refractory cytopenia with multilineage dysplasia (RCMD)
-- 70% of cases – in more than 1 cell line
.....* Refractory anemia with excess blasts (RAEB)
-- 25% of cases – morethan 3, less than 20
🗑
|
||||
| continued... | * MDS with isolated del(5q)
--5% of cases
....*MDS, unclassified
-- <5% of cases
🗑
|
||||
| Refractory Cytopenia with Unilineage Dysplasia (RCUD) ? | * <5% blasts in the BM.... * Dysplasia in >10% of cells in one lineage
🗑
|
||||
| Refractory Anemia with Ringed Sideroblasts (RARS) ? | * >15% ringed sideroblasts in the bone marrow
---- 10 or more iron-laden mitochondria occupying more than 1/3 of nuclear rim
🗑
|
||||
| Refractory Cytopenia with Multilineage Dysplasia (RCMD) ? | * Severe dysplasia in two or more cell lines.... * RCMD-RS --> increased ringed sideroblasts in addition to above
🗑
|
||||
| Refractory Anemia with Excess Blasts (RAEB) ? | * 5-9% = RAEB-I ...... * 10-19% = RAEB-II shorter survival and increased risk of developing AML ...... * Dysplasia present
..... *NO Auer rods
🗑
|
||||
| MDS with Isolated del(5q) ? | * 5q- Syndrome, and best prognosis .... * Severe anemia, normal platelet count ..... * RBC transfusions may be necessary
.....*BM with micromegakaryocytes (mono or bilobed nuclei), <5% myeloblasts
🗑
|
||||
| Myeloproliferative Disorders Overview ? | * Group of disorders with increased proliferative drive in BM.... * BM fibrosis and PB cytopenias.... * BM doesn't make enough RBCs bc of fibrosus.... * also see high numbers of one type of cells
🗑
|
||||
| MPD Testing ? | * CBC – increase in one or more cell lines
.... * Peripheral blood – maturation without excess blasts, should not be there ..... * Bone marrow aspiration & biopsy
- <20% blasts
-- Maturation of involved cell lines
🗑
|
||||
| continued.... | * Erythropoietin levels - markedly decreased in PV....
* Uric acid – increased due to cell turn-over
🗑
|
||||
| Chronic Myeloid Leukemia (CML) ? | * synthesis of active BCR-ABL tyrosine kinase --> t(9;22)the Philadelphia Chromosome .... * Sea-blue histiocytes .... * all levels of granulocytic maturation (<5% blasts); nucleated RBCs; increased Bo
& Eo; few promyelocytes; giant platelets
🗑
|
||||
| Chronic Myeloid Leukemia (CML) clinically ? | * splenomegaly - giant spleens ... * indigestion and fatigue ... * high WBC count with low blast count .... * can get ALL or AML
🗑
|
||||
| Polycythemia Rubra Vera (PV) ? | * mutations in tyrosine kinase JAK2 --> decreased need for erythropoietin in RBC production .... * Increased total blood volume viscosity with abnormal platelet function --> bleeding and thrombosis .... * Hemoglobin = 14-28 gm/dl *Hematocrit = >60%
🗑
|
||||
| Essential Thrombocythemia (ET) ? | * activating point mutations in JAK2 or MPL --> decreased need for thrombopoietin.... * megakaryocytes markedly increased ... * Giant platelets .... * Erythromelalgia – throbbing & burning of hands and feet .... * super high platelets and Bleed
🗑
|
||||
| Primary Myelofibrosis ? | * activating JAK2 or MPL mutations.... * see BM fibrosus, so causes a dry tap on biopsy .... * see Osteosclerosis... * erythroblastosis; tear drop RBCs; giant platelets; increased basophils .... * die in 3 - 4 years
🗑
|
Review the information in the table. When you are ready to quiz yourself you can hide individual columns or the entire table. Then you can click on the empty cells to reveal the answer. Try to recall what will be displayed before clicking the empty cell.
To hide a column, click on the column name.
To hide the entire table, click on the "Hide All" button.
You may also shuffle the rows of the table by clicking on the "Shuffle" button.
Or sort by any of the columns using the down arrow next to any column heading.
If you know all the data on any row, you can temporarily remove it by tapping the trash can to the right of the row.
To hide a column, click on the column name.
To hide the entire table, click on the "Hide All" button.
You may also shuffle the rows of the table by clicking on the "Shuffle" button.
Or sort by any of the columns using the down arrow next to any column heading.
If you know all the data on any row, you can temporarily remove it by tapping the trash can to the right of the row.
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.
Normal Size Small Size show me how
Normal Size Small Size show me how
Created by:
thamrick800
Popular Medical sets