Term
| T/F A myeloproliferative neoplasm is a type of myelodysplastic syndrome. |
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Definition
| false; they are different things |
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Term
| Whats another name for myeloproliferative neoplasms? |
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Definition
| myeloproliferative disorders |
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Term
| T/F Myelodysplastic syndrome is the same thing as a myeloproliferative disorder. |
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Definition
| falso, myeloproliferative disorder is myeloproliferative neoplasm |
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Term
| What do myelodysplastic syndromes and myeloproliferative disorders have in common? |
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Definition
| clonal stem cell disorder, both are quantitative, can transform to acute leukemias |
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Term
| What are the differences between myelodysplastic syndromes and myeloproliferative neoplasms? |
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Definition
MDS= quatitative and qualitative, cytopenias, abnormal morphology, cytologic dysplasia, ineffective hematopoiesis, minimal organomegaly
MPN: quantitative abnormality only, cytosis, normal morphology, no to minimal dysplasia, effective hematopoiesis, organomegaly (commonly spleen) |
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Term
| What is the age of patients with myelodysplastic syndromes? |
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Definition
| most commonly elderly (median age= 7th decade) but young (even children) also affected |
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Term
| What causes myelodysplastic syndrome? |
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Definition
| de novo, benzene, heavy metals, cigarette smoking (2 fold); therapy related- prior chemotherapy (alkylating agents) and radiation therapy |
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Term
| How is MDS diagnosed in asymptomatic patients? |
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Definition
| routine CBC reveals cytopenias with dysplastic changes |
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Term
| What are the symptoms/signs of MDS? |
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Definition
| infections (neutropenia, abnormally functioning WBCs) pneumonia, GU, sinus and many others; anemia (decreased RBCs); bleeding (thrombocytopenia, abnormally functioning platelets) mucocutaneous bleeding such as petechiae |
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Term
| How do most patients with MDS die? |
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Definition
| complications of bone marrow failure |
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Term
| MDS has a risk of transforming to what neoplasm? |
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Definition
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Term
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Definition
| stem cell abnormalities; cytogenic abnormalities in over 50% of cases; subtle molecular abnormalities |
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Term
| Subtle molecular abnormalities of MDS lead to cells that are... |
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Definition
| unable to respond to normal trophic signals and more sensitive to apoptotic signals |
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Term
| What cause of MDS is related to cytogenetic abnormalities? |
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Definition
| >50% of de novo and in the majority of therapy related MDS |
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Term
| What is the MC type of cytogenetic abnormality of MDS? |
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Definition
| loss of cytogenetic material |
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Term
| T/F MDS can have o cytogenetic abnormality. |
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Definition
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Term
| T/F MDS can have no cytogenetic abnormality. |
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Definition
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Term
| What cytogenetic abnormalities are most common with therapy-related MDS? |
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Definition
-5/del(5q) or -7/del(7q) in 90%
complex in 9% |
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Term
| What are common abnormalities in de novo MDS? |
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Definition
-5/del(5q) and complex most commonly
also, -7/del(7q), +8, 17p-, del(20q), -Y |
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Term
| What cytogenetics indicate a good prognosis? |
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Definition
| normal, isolated 5q-, isolated 20q-, -Y |
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Term
| What cytogenetics indicate a poor prognosis in MDS? |
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Definition
| complex changes (>3 abnormalities) or chromosome 7 abnormalities |
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Term
| Describe teh possible patient presentations for MDS. |
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Definition
| unexplained, persistent cytopenias, esp with a history of tx with cytotoxic agents or radiation, signs/symptoms are associated with cytopenias (infection, anemia, bleeding) |
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Term
| What must you find in PBS or BMA to make a dx of MDS? |
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Definition
| dysplasia in one or more cell lines with/without an increase in blasts; 10% of the cells in a line shoud be dysplastic (myeloid, erythroid, or megakaryotic) |
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Term
| What reactive, or "transient" dysplasias must be excluded for a diagnosis of MDS? |
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Definition
| dysplasia due to HIV, parvovirus infection, vitamin B12/folic acid deficiency, alcohol, medications/toxins, growth factor therapy, cytotoxic therapy, inherited diseases |
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Term
| What labs should you get for a patient with MDS? |
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Definition
| CBC, serum B12, folate, and ferritin levels, bone marrow examination, flow cytometry (evaluation of blasts), cytogenetics |
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Term
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Definition
| usually decreased (anemia) |
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Term
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Definition
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Term
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Definition
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Term
| What is the retic count in MDS? |
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Definition
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Term
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Definition
| normal to markedly decreased |
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Term
| What is the platelet count of MDS? |
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Definition
| normal to decreased; rarely thrombocytosis (isolated del(5q) syndrome) |
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Term
| What causes the bleeding and infection of patients with MDS? |
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Definition
| both DECREASED NUMBER and POOR FUNCTION of platelets and WBCs |
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Term
| What do the RBCs of paitents with dyserythropoiesis in MDS look like? |
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Definition
| usually macrocytic but may be dimorphic in sideroblastic subtypes (normochromic/normocytic with hypochromic/microcytic); may also see anisopoikilocytosis, basophilic stippling, howell-jolly bodies, NRBCs |
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Term
| The NRBCs of MDS often have... |
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Definition
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Term
| What are the characteristics on BMA of dyserthropoeisis of MDS? |
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Definition
| erythroid hyperplasia or hypoplasia, megaloblastoid changes (asynchronous n/c maturation), internuclear bridging, broad-based nuclear budding, multinuclearity, abnormal nuclear shapes, increased pyknosis, ringed sideroblasts |
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Term
| What are the characteristics of dysgranulopoiesis in the peripheral blood and bone marrow of patients with MDS? |
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Definition
| nuclear hyposegmentation in PMNs (bilobation= pseudo pelget-huet, or monolobation), hypogranular or hypergranular cytoplasm, doughnut-shaped nuclei, increased myeloblasts and immature granulocytes, neutropenia |
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Term
| Describe the characteristic evidence of dysmegakaryocytopoiesis in the peripheral blood of patients with MDS? |
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Definition
| thrombocytopenia, large/giant platelets, hypogranular platelets, abnormal platelet granules, micromegakaryocytes |
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Term
| What are evidences of dsymegakaryocytopoiesis in the bone marrow of patients with MDS? |
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Definition
| increased number of megakaryocytes, clustering, micromegakaryocytes/dwarf megakaryocytes, monolobulated megakaryocytes, megakaryocytes with detached small nuclei or seperated by thin strands of nuclear material |
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Term
| What lab is helpful to identify dysmegakaryocytopoiesis of BM biopsy? |
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Definition
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Term
| A megakaryocyte with three pyknotic, round nuclei is also known as a ___________. |
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Definition
| "pawn ball" megakaryocyte |
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Term
| What are the WHO classifications of MDS? |
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Definition
| refractory cytopenia with unilineage dysplasia (RCUD), refractory anemia with ringed sideroblasts (RARS), refractory cytopenia with multilineage dysplasia (RCMD and RCMD-RS), refractory anemia with excess blasts; MDS with isolated del(5q); myelodysplastic syndrome-unclassified (MDS-U); childhood MDS/Refractory cytopenia of childhood (very rare) |
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Term
| Waht are teh types of RCUD? |
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Definition
| refractory anemia (RBCs only), refractory neutropenia (neuts only), refractory thrombocytopenia (megs/plts only) |
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Term
| What are the two types of refractory cytopenia with multilineage dysplasia? |
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Definition
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Term
| What are teh tyeps of refractory anemia with excess blasts? |
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Definition
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Term
| Describe refractory anemia? |
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Definition
| anemia (usually macrocytic), no neutropenia or thrombocytopenia, ,dyserythropoiesis, no increase in blasts, no increase in ringed sideroblasts, neutrophilic and megakaryocytic cells without dysplasia |
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Term
| What is teh MC type of RCUD? |
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Definition
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Term
| Describe refractory neutropenia? |
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Definition
| very rare, neutropenia (no anemia or thrombocytopenia), dysplastic neutrophils, no increase in blasts, no erythroid, megakaryocytic dysplasia |
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Term
| Describe refractory thrombocytopenia? |
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Definition
| thrombocytopenia, no anemia or neutropenia, dysplastic megakaryocytes, no increase in blasts, no erythroid/neutrophilic dysplasia |
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Term
| What is teh prognosis of RA? |
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Definition
| median survival of 65 months, rate of progression to acute leukemia at 5 years is 2%; depends on cytogenetic abnormalities |
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Term
| What do you see in the marrow of patients with RARS? |
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Definition
| >15% ringed sideroblasts with increased number of hemosiderin laden macrophages |
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Term
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Definition
| accounts for 10-12% of cases of MDS |
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Term
| What MDS causes liver and spleen iron overload? |
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Definition
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Term
| What is the prognosis of RARS? |
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Definition
| depends on cytogenetic abnormalities, median survival of 70-100 months, approximately 1-2% of cases evolve to AML |
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Term
| What do you see on PBS of pts with RCMD? |
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Definition
| bi or pancytopenia, no to rare blasts (<1%), no auer rods (presence of Auer rods indicates RAEB-2) |
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Term
| What do you see in the marrow of patients with RCMD? |
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Definition
| dysplastic changes in at least 10% of the cells in at least 2 cell lines; less than 5% blasts, no auer rods, |
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Term
| How do you diagnose RCMD-RS? |
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Definition
| If BMA meets criteria for RCMD and they have at least 15% ringed sideroblasts, then diagnose as RCMD with ringed sideroblasts (RCMD-RS) |
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Term
| What determines the prognosis of RCMD? |
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Definition
| degree of cytopenia and dysplasia and cytogenetic abnormalities |
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Term
| What is the prognosis of RCMD? |
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Definition
| median survival is 30 months; 10% have evolution to acute leukemia at 2 years |
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Term
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Definition
| accounts for 25% of cases of MDS |
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Term
| What's the difference between RAEB-1 and RAEB-2? |
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Definition
1= 5-9% blasts in marrow; no auer rods
2= 10-19% blasts in marrow; may see auer rods (the presence of auer rods qualifies to be RAEB-2 even if blasts are less than 10%) |
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Term
| IS RAEB uni or multilineage dyplasia? |
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Definition
| can be either (blast cells show myeloid phenotype) |
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Term
| What is the % transformation to acute myeloid leukemia of RAEB-1 versus RAEB-2? |
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Definition
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Term
| What is the median survival of RAEB-1 versus RAEB-2? |
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Definition
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Term
| What is the typical patient with MDS with isolated del(5q)? |
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Definition
| middle aged to older women |
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Term
| What is the median survival of MDS with isolated del(5q)? |
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Definition
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Term
| What are the PBS characteristics of a pt with MDS with isolated del (5q)? |
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Definition
| refractory anemia, often severe, normal to occasionally increased plaetlet counts, no blasts |
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Term
| What are the characteristics of MDS with isolated del(5q) on marrow? |
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Definition
| normal to increased megakaryocytes, any with hypolobated/monolobated nuclei, <5% blasts and No Auer Rods |
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Term
| What is the criteria for diagnosing and MDS as therapy related? |
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Definition
| typically occurs 5-10 years after exposure to alkylating agents and/or ionizing radiation |
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Term
| What is the prognosis of therapy related MDS? |
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Definition
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Term
| Why are cytogenetics necessary for MDS? |
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Definition
| confirm clonality and thus the diagnosis as MDS, prognosis, sometimes therapy |
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