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136 Cards in this Set
- Front
- Back
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MDS should be suspected in any adult with ___ or ___
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unexplained blood cytopenias or monocytosis
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MDS with thrombocytosis?
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- unusual
- isolated 5q- syndrome (type of refractory anemia) |
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MDS, reticuloctyopenia despite anemia leads to fewer __ seen in the PB
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polychromatophilic cells
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ALIP
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abnormal localization of immature precursors
- evidence of dysgranulopoiesis - normally, most immature granulocytes are paratrabecular or adjacent to blood vessels |
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pseudo-Pelger-Huet change
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- hyposegmented neutrophils
- evidence of dysgranulpoiesis |
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Dysmegakaryopoiesis leads to the following changes seen in the BM
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- increased or decreased numbers
- clustering - micromegakaryocytes - monolobation or hypolobation - odd numbered nuclei - multiple widely separated nuclei (good evidence of dysplasia) |
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Dimorphic RBC population in PB
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- transfusion
- treated anemia (Fe def) - sideroblastic anemia - MDS (dyserythropoiesis) |
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In MDS, bone marrow cellularity is typically __
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- normo- or hypercellular (but ineffective)
- only 10-15% hypocellular |
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In MDS, iron stores are typically __
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- increased, due to ineffective hematopoiesis
- esp. transfusion dependent patients (refractory anemia!) |
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Myelofibrosis in MDS?
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- occasionally seen in a de novo MDS (more associated with CMPD's)
- among MDS, more commonly seen with therapy-related MDS |
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Most common morphologic change seen in MDS
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dyserythropoiesis
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Flow cytometry in MDS
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- CD56+ granulocytes and monocytes
- aberrant expression of lymphoid markers - increased blasts (CD34+) |
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___ should be performed on every MDS case
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cytogenetics
- prognositic information - classification - more likely to find a cytogenetic abn in RCMD or RAEB (~50%) compared to RA or RARS - NO specific cytogenetic abn for MDS - IMPORTANT: the finding of AML associated cytogenetic abnormalities despite MDS range blast counts (<20%) will be classified as AML! |
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AMLs with specific cytogenetic abn, but MDS range blast counts (<20%), are still considered AML!!
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- so do cytogenetics on very MDS case!
- t(8;21) - inv(16) t(16;16) - 11q23 (MLL) abn |
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Most common cytogeneitc abn in de novo MDS is __
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del(5q)
- alone or not Note: isolated del(5q) syndrome |
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Monosomy 7 is the ___ cytogenetic abn seen in adult and pediatric MDS
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Monosomy 7
- most common in pediatric MDS - second most common in adult MDS, following del(5q) - monosomy 7 is present in 50% of therapy-related MDS and 10-15% of de novo MDS |
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In MDS, complex cytogenetic abn and abn of chromosome 7 are a/w __
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poor prognosis
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MDS w/ del(17p) is a/w what in the PB smear
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- prominent pseudo-Pelger-Huet changes
- small vaculated neutrophils - poor prognosis |
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abnormalities in PMN function can be seen in about ___of MDS cases
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50%
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In MDS, RBC HbF can be ___
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elevated
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5q- syndrome is a/w ___
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- anemia (RA)
- thrombocytosis (unique among MDS) - numerous hypolobated megakaryocytes - stable clinical course (however may be transfusion dependent) - more common in women |
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Classification systems for MDS: ___ replaced __
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WHO classificaton replaced the FAB (French-American-British)
- reduced the percentage of blasts for a diagnosis of AML from 30% to 20% - moved CMML to a new category MDS/MPD |
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Refractory anemia characterized by
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- ineffective erythropoiesis
- anemia w/ reticulocytopenia - BM erythroid hyperplasia |
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RA peripheral blood smear
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- anemia
- normocytic or macrocytic - oval macrocytes common - no dysplasia in platelets or neutrophils - monos <1 |
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evidence of dyserythropoiesis in the BM includes
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- megaloblastoid chromatin
- nuclear budding - karyorrhexis - multinuclearity - +/- cytoplasmic vacuolization (PAS +) - ringed siderblasts (>15%) |
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In RA, clonal BM cytogenetic abn are found in up to __% of cases, therefore _
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- 25%
- 75% will not have cytogenetic evidence of clonality, and you MUST r/o secondary causes of anemia - AOCD/infection - endocrinopathies - renal failure - medication |
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RA typically has a __ course
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- chronic course, with some becoming transfusion dependent
- only a small number progress to an aggressive MDS with bone marrow failure or AML |
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% of blasts allowed in PB in RA
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NONE!
- none in RA, RARS - only rare blasts allowed in RCMD |
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% of myleblasts allowed in BM in RA
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- less than 5%
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RARS differs from RA
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- 15% or more RS
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ringed sideroblasts
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erythroid precursor w/ 2/3rd or more of the nucleus surrounded by siderotic granules on an iron stained aspirate smear
- can be seen in many types of MDS!! - "benign RS" a/w non-clonal causes of anemia: - alcoholism - anti-TB drugs |
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RARS PB smear
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- commonly dimorphic RBC (nml RBCs and microcytic and/or hypochromic poikilocytes)
- coarse basophilic stippling - minimal or no neutropenia or thromocytopenia |
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in RARS BM, ___ is the most prominent feature
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- numerous ringed sideroblasts
- erythroid hyperplasia - markedly increased iron stores - mild to moderate dyserythropoiesis |
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RARS has to be distinguished from ___, because of differences in aggressiveness
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- RARS vs. RCMD-RS
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__% of RARS progress to AML
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- less than 5%
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RCMD is characterized by
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- cytopenias in at least one lineage
- dysplasia in at least 10% of at least 2 lineages - PB monos still <1 |
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Myeloblasts in RCMD in the PB and BM
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- PB none or rare
- <5% in BM (otherwise it would be RAEB) |
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RCMD PB smears are a little more striking than RA and RARS
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- dysplasia is variable
GRANULOCYTES - neutrophils commonly hypogranulated and hyposegmented RBCs - dyserythropoiesis may be more striking than that seen in RA or RARS PLATELETS - atypical large and abnormally granulated platelets |
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Can you see Auer rods in RCMD?
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NO
- presence of Auer rods, regardless of myeloblast count is automatically type 2 RAEB |
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RAEB PB smear
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- multilineage dysplasia common (note: however, its a refractory anemia with excess blasts, not called multilineage dysplasia)
- NRBCs - immature granulocytes w/ myeloblasts (but <20%) - occ. micromegak |
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RAEB BM shows __
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- normo- or hypercellular
-- usually erythroid and granulocytic hyperplasia seen - myeloblasts at least 5% but less than 20% - +/- Auer rods (type 2 RAEB) |
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type 1 vs type 2 RAEB
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type 1 RAEB: (excess blast in the marrow)
- 5-9% myeloblasts BM - <5% myeloblast in PB - no Auer rods type 2 RAEB: (excess in blasts in marrow and blood) - 10-19% myeloblasts BM - 5-19% myeloblasts in PB despite <10% blast in BM - Auer rods present in RAEB(regardless of blast count) type 2 RAEB is more aggressive with a greater chance of progression to AML and shorter survival |
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clincal significance of Auer rods with a blast percentage <20% in BM or blood
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- unknown
- but recommended that their presence in RCMD or RAEB type 1, lead to classification as type 2 RAEB Note: MDS w/ Auer rods and <5% blasts in PB and BM is an MDS, unclassifiable (unknown significance of Auer rods in this situation) |
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the most common type of MDS diagnosed is __
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RAEB
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clinical course of RAEB is __
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-aggressive w/ worsening cytopenias and progression to BM failure
- 33% progress to AML - poor px |
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Important distinction before making a diagnosis of RAEB
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- absence of AML specific cytogenetic abn!!
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5q- syndrome is characterized by
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- refractory anemia (normocytic or macrocytic)
- thrombocytosis!! (may be normal!!) - isolated del(5q) |
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Blast count in 5q- syn
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- <5% in blood and marrow
- NO Auer rods |
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Megakaryocyte abnormalities in MDS
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- NOT seen in RA or RARS (by definition there is erythroid dysplasia ONLY)
- start seeing dysmegakaryopoiesis when you see multilineage dysplasia (RCMD, RAEB, MDS unclassifiable and 5q- syndrome) - micromegakaryocytes - hypolobation - nuclear separation - odd number of nuclei - clustering |
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MDS with isolated 5q- is seen primarily in __ and has a __ px
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- older women
- stable clinical course (may be tfn dependent) - good px (but some may progress to more aggressive MDS or AML) |
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MDS, unclassifialbe
- features that may make classification of an MDS difficult |
- Auer rods but blasts <5% in PB and BM
- isolated neutropenia or thrombocytopenia - leukocytosis or thrombocytosis - therapy related MDS - hypocellular marrow - myelofibrosis |
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presence of Auer rods with a low blast count (<5%)
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- rare
- cases with features of RA, RARS, RCMD but with Auer rods - significance of Auer rods is not clear, but evidence suggest these are aggressive MDSs |
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60 yo women with transfusion dependent anemia and mild thrombocytosis, if she had an MDS, what would be a good guess
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- MDS a/w isolated del(5q)
- identified by BM cytogenetics |
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Therapy-related MDS
- major types of drugs |
- cytotoxic or radiotherapy
- major drugs: -- alkylating agents: - more common cause - 4-5 yrs lag -- topoisomerase II inhibitors: - more commonly a/w therapy-related AML, but may present with MDS that quickly progresses to AML - 2.5-3 years (sooner) |
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Therapy-related MDS a/w alkylating agents often presents with __
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- unexplained pancytopenia 4-5 years after treatment
- marrow shows trilineage dysplasia - blast often <5% (like RCMD) - marrow cytogenetic abn present in almost all cases (often complex and involve chr 5 and/or 7) |
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Therapy-related MDS a/w topoisomerase II inhibitor
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- more commonly a/w AML
- typically a/w prominent monocytic lineage involvement - often a/w 11q23 rearrangements |
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Importantly, in the WHO classification, therapy-related MDSs are classified under the __
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AMLs!
- includes therapy related MDS and AML!! |
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hypocellular MDS is more commonly a/w __
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- therapy related MDSs
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Hypocellular MDS: marrow cellularity less than __
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- less than 30% in youger patients
- less than 20% in pts >60 yo |
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hypocellular MDS can be difficult to distinguish from ___ or ___ (DDX)
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DDX: hypocellular MDS, AML, and aplastic anemia
CLUES - cytogenetic abn helps to differentiate from aplastic anemia - distinction from hypocellular AML is based on blast % or findings of AML specific cytogenetic abn (t(8;21), inv(16), 11q23 abn) |
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Myelofibrosis and MDS?
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- 10-15% of de novo MDS
- less common than w/ CMPD - more commonly a/w therapy-related MDS - myelofibrosis leads to difficulty obtaining marrow aspirate to adequately assess marrow and blast percentage |
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MDS with myelofibrosis (usually pancytopenia w/ trilineage dysplasia) may be difficult to distinguish from __ or ___
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1. acute panmyelosis with myelofibrosis (included in the AML classification)
- usually distinquished by 20% or more blast, but with myelofibrosis it may be difficult to accurately enumerate the blast % -- may try CD34 on the core bx 2. acute megakaryoblastic leukemia - may present with profound myelofibrosis |
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MDS w/ myelofibrosis is distinguished from the CMPD CIMF by __
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- presence of blood cytopenias
- trilineage dysplasia - increased and dysplastic megakaryocytes in the BM core bx - ABSENCE of splenomegaly |
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MDS vs MPD
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MDS:
- cytopenias - ineffective hematopoiesis - dysplasia - NO splenomegaly MPD: - normal or elevated blood counts - effective hematopoiesis - NO dysplasia - splenomegaly present |
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MDS/MPD include:
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- CMML
- atypical CML - JMML - MDS/MPD, unclassifiable |
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CMML actually includes __ and ___ leading to a broad spectrum of clinical and hematologic presentations
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- MDSs and MPDs!, but with increased monocytes!!
- a case may look like a RA, RCMD, or RAEB but have a monocytosis (>1) - cases may have leukocytosis and monocytosis, organomegaly and minimal or no dysplasia or increased blasts |
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Before a dx of CMML is made in a case with minimal or no dysplasia and no increase in blasts or cytogenetic abn...
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- basically: unexplained monocytosis only!!
- pt has to be followed for at least 3 months and all other causes of monocytosis have to be excluded |
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MDS picture w/ splenomegaly or other tissue infiltration (lymphadenopathy)- think about__
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- MDS/MPD like CMML
- splenomegaly present in about 30-50% of CMML - 20% hepatomegaly - lymphadenopathy w/ extensive proliferation of plasmcytoid MONOCYTES may be the major finidng in CMML - of course, the PB monocytosis will be present |
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Monocytes in CMML
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- >1.0 x 10^9/L
- may be nml or abn: -- hyperlobated, increased cytoplasmic basophilia, abn granulation |
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In CMML, promonocytes and monoblasts may be present, but___
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- usually less than 5% of the WBCs and ALWAYS less than 20%
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In CMML, can neutrophils, RBCs and platelets all show dysplastic features?
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YES!!
- CMML is an MDS or MPD with increased monocytes! - could have cytopenias or elevated counts |
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CMML with leukocytosis
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- monocytosis
- monocytosis and neutrophilia - basophilia and/or eosinophila may be present |
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CMML with eosinophilia
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- if PB monocytosis (>1) with PB eosinophils >1.5
- special designation because this may be a/w complications fo hypereosinophilia |
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CMML bone marrow is usually ___
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- hypercellular
- prominent monocytic component - dysplasia often present - may see some degree of myelofibrosis - blasts and promonocytes usually <10% (always <20%) |
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type 1 vs type 2 CMML
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type 1 CMML:
- <5% blasts and promonocytes in blood - <10% in marrow type 2 CMML: - 5-19% blasts and promonocytes in blood - 10-19% in marrow - presence of Auer rods (as in type 2 RAEB) |
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CMML: cytogenetic abn found in ___
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33%
- similar to those found in MDSs |
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CMML with isolated i(17q)
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- unique MDS/MPD with features of CMML plus:
- striking dysgranulopoiesis w/ severe hyposegmentation (prominent pseudo Pelger-Huet change) - HIGH RISK for transformatin to AML |
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MDS/MPD's have mixed __ and __ features
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dysplastic (MDS) and proliferative (MPD) features
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CMML prognosis is __
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variable
- may be indolent - may be highly aggressive and transform to AML or lead to BM failure in a few months - 25% transform to AML |
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the main feature distinguishing CMML from a MDS or MPD
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- persistent unexplained monocytosis >1 (longer than 3 months)
- NO Ph chromosome or BCR/ABL translocation |
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features of CMML
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- persistent monocytosis (>1) for at least 3 months
- no Ph chr or BCR/ABL - <20% blasts in marrow and PB - dysplasia or cytogenetic abn (33%) - splenomegaly (30-50%) |
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atypical CML has features of both __ and __
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MDS and MPD
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features of aCML
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- leukocytosis w/ predominately mature and maturing granulocytes
- mature granulocytes predominate but immature granulocytes account for >10% of WBC in PB or marrow - monocytes <1 (monocytes are always <10% of WBC) - no or minimal basophilia - prominent dysgranulpoiesis (dysplasia) - <20% blasts includes: - rare BCR/ABL negative leukemia composed of mature and maturing granulocytes - MDS w/ high leukocyte count - MPD w/ myelodysplastic features |
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aCML BM is often __
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- hypercellular w/ granulocytic hyperplasia and dysplasia
- myeloblasts usually <5% (always <20%) |
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Features that differ between aCML and CML
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aCML:
- minimal to no basophilia - dysplasia is a prominent feature in the neutrophils - anemia and thrombocytopenia - no Ph chr or BCR/ABL |
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Importantly, the px of aCML
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- aggressive
- median survival of 2 yrs - anemia and thrombocytopenia are poor px signs - bone marrow failure - 33% transform to AML |
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In aCML, cytogenetic abn are found in __ cases
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- most cases
- not Ph or BCR/ABL |
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MDS/MPD, unclassifiable
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example:
- a cases w/ features of RARS but with marked thrombocytosis (features of ET, plt > 600) |
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MDSs in children are __, and if they occur there is a good chance there is an underlying __
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- rare
- predisposing genetic abn (33%) like Down syn, NF-1, prior CTX - MDSs in children often have features of MDS and MPD (MDS/MPD) - if there is a genetic predisposing condition, a child usually presents with myelodysplasia before 2 years old!! |
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MDS in children is divided into two categories
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1. disorders unique to infants and very young children
- JMML - transient MPD a/w trisomy 21 2. MDSs w/ features similar to adult-type MDS |
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JMML includes all childhood leukemias formerly designated as:
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- juvenile CML
- CMML - infantile monosomy 7 syndrome |
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MDS is rare in children (3-9% pedi hematologic malignancies), but among these JMML accounts for
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18-36% of MDS in children
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JMML occurs mostly in __
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- infants and very young children (60% <2 yo)
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__ % of JMML occur in children with NF-1
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10-15%
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Hallmark clinical features of JMML
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- hepatosplenomegaly
- lymphadenopathy - recurrent infections - recurrent bleeding - maculopapular rash (>33%) - cafe au lait spots in pts w/ NF-1 |
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WHO criteria for JMML
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1. blood monos >1 (monocytosis)
2. blasts and promonocytes < 20% 3. no Ph chr or BCR/ABL Plus 2 or more of the following: - increased Hb F - immature granulocytes in PB - WBC >10 - clonal chr abn - GM-CSF hypersensitivity of myeloid progenitors in vitro! |
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JMML PB smear
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- usually leukocytosis and monocytosis
- monos often >5 - blasts and promonocytes <5% (always <20%) - often immature granulocytes and monos - dysplasia (usually mild) - usually NRBCs - THROMBOYCTOPENIA common and often severe NOTE: the PB smear is often more helpful than the BM exam in JMML |
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JMML BM
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- usually hypercellular w/ granulocytic hyperplasia
- myeloblasts < 20% - NO Auer rods - Megakaryocytes often decreased |
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Two very useful test in JMML dx include:
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1. HbF
- often >10% (2/3 cases) - helpful clue in early stage of disease (persistently elevated HbF) 2. in vitro GM-CSF hypersensitivity |
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JMML, cytogenetic abn are found in __%, with >50% consisting of __
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- 33-60% of JMML
- >50% consists of isolated monosomy 7 or del(7q) |
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Major DDX consideration in JMML
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RULE OUT UNDERLYING INFECTION!
- monocytosis and leukemoid reaction found in JMML can simulate: - persistent EBV infection - CMV - HHV-6 - histoplasmosis - mycobacterial infections Demonstration of clonality helps! - cytogenetic abn (50%) - molecular evidence of ras mutations (20-30%) |
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Clinical course in JMML is variable
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1/3: rapid, aggressive from the start (death w/ or w/o treatment)
1/3: initial indolent course but followed by rapid progression 1/3: intermediate between the two 10-15% transform to AML |
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JMML diagnosed in pts < 1 yo is a/w a __ survival
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- better long-term survival
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JMML: poor px signs include:
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- low plts (<33)
- high HbF (>15%) - abn cytogenetics (2 or more clonal abn) |
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Adult-type MDS in children
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- usually dx's in children older than the typical JMML pt (median age: 8)
- most common types are RCMD and RAEB - MDS involving a single lineage (like RA, RARS) is rare - these MDSs are more likely to be unclassifiable - most have cytogenetic abn; most common are monosomy 7 and del(7q) |
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as with JMML, dx of adult-type MDS in children < ___ years old is a/w a better px
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- <1 yo
The adverse px signs for JMML are the same for adult-type MDS |
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DDX for MDS (in general), non-clonal causes of myelodysplasia
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- megaloblastosis due to vit B12 or folate def
- heavy metal intoxicaiton (arsenic, lead,..) - acute alcohol intoxication - drug effects (most CTX) - viral infection: parvovirus B19 - congenital dyserythropoietic anemia - chronic infectious diseases - AIDS - AML (M2 AML w/ maturation, M4 myelomonocytic, M6 erythroleukemia) - other |
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DDX for ringed siderblasts
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- MDS
- hereditary sideroblastic anemia - anti-TB drugs or chloramphenicol - alcohol toxicity - chronic lead poisoning |
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HIV/AIDS can be a/w ____ that mimics a primary MDS
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- cytopenias and myelodysplasia
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___ should always be in the DDX for MDS
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HIV/AIDS
- can show dyslplasia in all lineages |
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Overall, __% of MDS transform into AML
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30%
- less with RA and RARS and more with multilineage dysplasias (RCMD, RAEB, therapy-related MDS) |
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In MDS, cytogenetic abn a/w a better px
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- isolated del(5q)
- isolated del(20q) - isolated del(Y) - normal cytogenetics Poor px: - abn of chr 7 - complex abn (3 or more) |
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In MDS, the presence of ringed siderblasts, is associated with a __ px
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better!!
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Ineffective hematopoiesis is characterized by __
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- blood cytopenias
- normo- or hypercellular BM w/ increased hematopoietic precursors in most cases most common example: ineffective erythropoiesis w/ - anemia - reticulocytopenia - erythroid hyperplasia in BM |
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In MDSs, the cytopenias characteristically __ with time
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progress
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Dyserythropoiesis (PB smear)
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- anemia
- anisopoikilocytosis oval macrocytes hypochromic cells dimorphic populations - decreased polychromatophilic cells - basophilic stippling - NRBCs - vacuolated RBCs - Howell-Jolly bodies |
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Dyserythropoiesis (BM)
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- erythroid hyperplasia
- N:C asynchrony - megaloblastic(oid) chromatin - karyorrhexis - multinuclearity - internuclear bridging - nuclar fragments - ringed sideroblasts - PAS+ erythroblasts |
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ALIP
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- finding immature granyloctye precursors remote from the usual paratrabecular location
- may be a/w poor px and greater likelihood of transformation to AML |
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Increased myeloblasts are found in __ MDSs
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more aggressive
- the % of BM myeloblast is the defining feature of the MDS classification - myeloblasts <20% in blood or marrow (>19% it is AML!) |
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Erythroid precursors in MDS are often show aberrant immunophenotypic prophiles
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- abn levels of transferrin receptor (CD71)
- dyssynchronous expression of CD45/CD71, CD45/glycophorin A, and CD71/glycophorin A |
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Problem with the use of flow cytometry in evaluation of MDS?
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- lack of knowledge in non-neoplastic conidtions that may simulate MDS
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WHO classification of MDS
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RA
RARS RCMD - RCMD/RS RAEB - type 1: 5-9% myeloblast in BM w/ <% in PB - type 2: 10-19% in BM and/or 5-19% in PB MDS, unclassifiable MDS a/w isolated del(5q) |
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the degree of dyserthropoiesis in RA is typically __
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- minimal to moderate in degree
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In some cases of RA, the diagnosis is delayed __
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and only made after following the patient for months and ruling out other causes of anemia (AOCD, endocrinopathies, renal failure, medication-induced anemia...)
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DDX for RARS includes
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- RARS
- RCMD-RS - benign causes of sideroblastic anemia (alcoholism, anti-TB drugs..) |
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In RCMD, Auer rods are __
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absent!
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RCMD differs from RAEB by __
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having <5% myeloblasts
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Myeloblasts in PB in RCMD?
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absent or rare!
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RAEB PB smear
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- NRBCs
- immature granulocytes (including myeloblasts) - occasional micromegak's |
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MDS with Auer rods is automatically classified as __ or __
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- RAEB type 2
- if <5% blasts in PB and BM, than MDS, unclassifiable |
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Refractory neutropenia or refractory thrombocytopenia
(MDS with isolated neutropenia or thrombocytopenia) |
- diagnoses made with caution
- unilineage dysplasia (similar to RA) - usually these are classified as MDS, unclassifiable - these names have been proposed by some - require convincing evidence of unilineage dysplasia and ruled out other causes or have clonal BM chromosome abn |
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CMML without recognizable dysplasia, no increased blasts, and no identifiable cytogenetic abn??
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- unexplained monocytosis only! after following pt for at least 3 months and ruling out other causes
- eventually the pt will evolve clinically like a MDS or MPD |
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In CMML, splenomegaly is present in
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30-50% cases
- primarily red pulp infiltration |
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In CMML, lymph node involvement may be seen
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- proliferation of plasmacytoid monocytes (may be the presenting manifestation of CMML)
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CMML with isolated i(17q)
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- unique MDS/MPD w/ features of CMML with isolated i(17q)
- striking dysgranulpoiesis w/ severe hyposegmentation of neutrophil nulcei - high risk of transformation ot AML |
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MDS/MPDs are characterized by mixed __
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proliferative and dysplastic features
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