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Hematology Leukemia, MPD, MDS Buzz Words
Hematology Leukemia, MPD, MDS Buzz Words
64
Medical
Professional
09/21/2010

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Term
Ddx leukoerythroblastosis (3)
Definition
Marrow stress
Myelophthisis = met. tumor or granuloma
MPDs esp. CIM
Term
Cellular origin of MPDs
Definition
PHSC
Term
Characteristics of MPDs (3)
Definition
Proliferation
Hypercellular BM
Pancytosis
Term
Symptoms of MPDs
Definition
Splenomegaly
Absolute basophilia
Inc. serum B12
Term
MPDs must be distinguished from...
Definition
Benign cell proliferations
Term
CML: age range, symptoms (3)
Definition
25-60
Anemia, fatigue, weakness
Anorexia, weight loss
Ab pain from splenomegaly
Term
CML labs: WBC, RBC, plt, BM
Definition
WBC >50,000
RBC mild anemia
Plt elevated
BM 100% cellular w/ myelopoiesis dominating
Term
CML morphology: WBC
Definition
Myeloid precursors
Absolute basophilia
Term
Absolute basophilia
Definition
CML
Term
CML: cytogenetics, prognosis, tx
Definition
t(9;22) BCR/ABL tyrosine kinase
Blast crisis 3-5 years
BM transplant, imatinib/Gleevec
Term
CIM: age range, symptoms (2)
Definition
Older folks
Anemia
Splenomegaly from myeloid metaplasia
Term
CIM labs: WBC, RBC, plt, BM
Definition
WBC elevated but <25,000
RBC anemia
Plt nl/elevated
BM hypercellular w/ fibrosis and megakaryocyte clusters
Term
CIM morphology: WBC, RBC, plt
Definition
Leukoerythroblastosis
RBC tear-drop cells
Plt large, hypogranular
Term
CIM: cytogenetics, prognosis, tx
Definition
JAK2 mutation
5-10 years, most will gradually replace BM w/ fibrosis, die of BM failure = anemia, hemorrhage, infection
Some will have blast crisis
Tx: none
Term
PV: age range, symptoms (6)
Definition
Older folks
HTN, dizziness, cyanosis, ab pain
Thrombosis, hemorrhage
Term
PV labs: WBC, RBC, plt, BM
Definition
WBC mildly elevated
Hct >55%
Plt nl/elevated
BM: panmyelosis
Term
PV: EPO, cytogenetics, prognosis, tx
Definition
EPO decreased from negative feedback
JAK2 mutation
10-15 years, most enter "spent phase"
Some will have blast crisis
Tx: decrease RBC mass
Term
Ddx erythrocytosis
Definition
Relative: dehydration, spurious
Absolute: primary (PV), secondary
Term
Ddx secondary absolute polycythemia
Definition
Appropriate: high altitude, Hb-opathies, cardio-pulm disease
Inappropriate: renal cyst, neoplasms, paraneoplastic syndrome
Term
EPO levels in secondary absolute polycythemia are...
Definition
Increased either appropriately or inappropriately
Term
Essential thrombocytopenia: age range, symptoms
Definition
Least common
Older folks
Thrombosis, hemorrhage
Term
Essential thrombocytopenia labs: WBC, RBC, plt, BM
Definition
WBC nl/elevated
RBC anemia
plt >450,000
BM hypercellular w/ inc. megakaryocytes
Term
ET morphology: plt, megakaryocytes
Definition
Plt nl or large and/or hypogranulalr
Megakaryocytes may be in clusters, may be large w/ immature nuclei
Term
Ddx thrombocytosis
Definition
ET, other MPD
Chronic inflamm
TB
Neoplasms
Term
Symptoms of MDSes (3)
Definition
Anemia, fatigue, DOE
Recurrent infections
Inc. bruising/bleeding
Hepatosplenomegaly rare!
Term
Kinds of MPDs (4)
Definition
CML
CIM
PV
ET
Term
Kinds of MDSes (6)
Definition
RA
RARS
Refractory cytopenia w/ multilineage dysplasia
Refractory anemia w/ excess blasts 1
RAEB2
C-myelomonocytic-L
Term
Characteristics of MDSes (3)
Definition
Hypercellular marrow
Dyspoiesis
Intramedullary cell death, cytopenias
Term
Cellular origin of MDSes
Definition
Earliest myeloid progenitor
Term
MDS erythroid morphology: peripheral, BM
Definition
Macro-ovalocytic anemia, basophilic stippling
Megaloblastic appearance w/ nuclear-cytoplasmic dys-synchrony, multi-nucleation, nuclear fragmentation
Maybe ringed sideroblasts
Term
MDS leukocytic morphology: peripheral, BM
Definition
Pseudo-pelger-Huet neutrophils, abnl/immature monocytes, blasts
BM same + hypogranulation
Term
MDS megakaryocytic morphology: peripheral, BM
Definition
Large, hypogranular plt
Dec. #s, separate/individual nuclei or non-lobated, microkaryocytes
Term
MDS cytogenetics
Definition
Partial or total chromosomal deletions
Term
Refractory anemia characteristics
Definition
<15% ringed sideroblasts
Erythroid dysplasia only
<5% BM blasts
<1% peripheral blasts
Term
RARS characteristics
Definition
>15% ringed sideroblasts
Erythroid dysplasia only
<5% BM blasts
<1% peripheral blasts
Term
Refractory cytopenia w/ multilineage dysplasia characteristics
Definition
<15% ringed sideroblasts
Dysplasia in 2 or more cell lines
<5% BM blasts
<1% peripheral blasts
Term
RAEB1
Definition
5-9% BM blasts
<5% peripheral blasts
Term
RAEB2 characteristics
Definition
10-19% BM blasts
5-19% peripheral blasts
Can have Auer rods
Term
CMML characteristics
Definition
5-20% BM blasts
<5% peripheral blasts
>1000 monocytes
Term
MDS: prognosis, tx
Definition
RA/RARS have better prognosis
RAEB1/2 have the worst
Tx: none, will die due to complications of BM failure
Term
Characteristics of acute leukemias (2)
Definition
>20% blasts
Little/no maturation
Term
Acute leukemias: age range, symptoms
Definition
ALLs usually in kids
AMLs usually in older adults
Anemia, recurrent infections, bleeding issues, local symptoms/signs
Term
Acute leukemia general blast morphology
Definition
High nucleus:cytoplasm, fine chromatin
No granules
Auer rod indicates AML
Term
Acute leukemia cytochemical stains (4)
Definition
Sudan: my/mono primary granule membrane
Myeloperoxidase: my/mono primary granule content
Specific esterase: myeloid enzymes
Non-specific esterase: monocytic enzymes
Term
Acute leukemia myeloid cell markers (4)
Definition
11
13
33
34
Term
Acute leukemia pre-B cell markers (4)
Definition
19, 20
10
34
Term
Acute leukemia T-cell markers (3)
Definition
2
5
7
Term
Acute leukemia B-cell markers (4)
Definition
19, 20
10
Monoclonal surface Ig
Term
AML 4 major classifications
Definition
Recurrent genetic abnormalities
Multilineage dysplasia
Therapy related
Other
Term
AML t(8;21): age range, morphology, genes, prognosis
Definition
Younger
Maturation + Auer rods
AML1/ETO: inactivation of former stops WBC maturation
Good
Term
AML t(16;16) or inv(16): age range, morphology, genetics, prognosis
Definition
Younger
Maturation, abnormal eosinophils and precursors
CBFB/MYHII: inactivation of the former stops WBC maturation
Better
Term
AML t(15;17): age range, morphology, genetics, prognosis, tx
Definition
Middle-aged
Kidney-bean nucleus, hypergranular, multiple Auer rods
PML/RARa:latter can't receive vitA to stimulate WBC maturation
Prognosis: at risk for thrombosis/DIC
Tx: vitA
Term
AML t(9;11) or t(11;19): morphology, genetics, prognosis
Definition
Monocytic differentiation, can have neutrophilic as well
MLL rearrangement
Intermediate
Term
AML with multilineage dysplasia: age range, morphology, prognosis
Definition
Older folks
Dysplasia in >50% of BM in 2 or more cell lines
Poor
Term
AML, MDS tx related: etiology, morphology, prognosis
Definition
Topo w/in 12-130 mo, alkylating w/in 5-6 yrs
Dysplasia in all 3 cell lines
Poor
Term
AML minimally differentiated: morphology, cytochemistry, immunophenotyping
Definition
No Auer rods
Neg. cytochemistry stains
Pos. myeloid markers
Term
AML w/o maturation: morphology
Definition
<10% myeloid maturation, Auer rods
Term
AML w/ maturation: age range, morphology, prognosis
Definition
Younger folks
>10% myeloid maturation, Auer rods
Better
Term
AMML: morphology
Definition
>20% but <80% MM differentiation
Term
Acute monoblastic, acute monocytic leukemia: morphology, other findings
Definition
>80% monoblastic/monocytic differentiation
Tissue infiltration esp. gingival hypertrophy
Term
Acute erythroid leukemia: morphology, prognosis
Definition
Rare, erythroblasts
Poor
Term
Acute megakaryoblastic leukemia: morphology, other findings
Definition
Megakaryoblasts, abnormal megakaryocytes
Associated w/ fibrosis
Term
ALL B lymphoblastic: morphology, prognosis
Definition
Homogenous small blasts w/ minimal cytoplasm, immature chromatin, no nucleoli
OR heterogenous somewhat larger blasts w/ moderate cytoplasm, 1-2 nucleoli
Good if hyperdiploid, poor with hypodiploid and most translocations
Term
ALL T lymphoblastic: morphology, prognosis
Definition
Homogenous small blasts w/ minimal cytoplasm, immature chromatin, no nucleoli
OR heterogenous somewhat larger blasts w/ moderate cytoplasm, 1-2 nucleoli
High risk
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