Hematology Lec Midterm

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HEMATOLOGY 2, LEC 3.

Can be classified by stem cell involved and


Prof. Ruby Garcia-Meim, RMT, MSPH length of clinical course
a. Lymphoproliferative disorders –
MALIGNANT WBC DISORDERS acute or chronic
- Common lymphoid
Characteristics progenitor
1. A malignant clone of cells proliferate that do b. Myeloproliferative disorders –
not respond to normal regulatory mechanisms acute or chronic
a. Leukemia originates in the bone marrow - Common myeloid
and is initially systemic progenitor
b. Lymphoma originates in lymphoid tissue
and is initially localized Bone marrow examination used to aid in diagnosis
2. Etiology remains unclear. Multiple theories Indication included:
exist about oncogene activation, which likely 1. Investigation of peripheral blood
include multiple factors: abnormalities, such as unexplained
a. Viral – viruses can suppress cytopenias
immune function or activate 2. Staging and management of patients with
oncogenes (HTLV-I, II, V) and HIV1, certain lymphomas or solid tumors
EBV – burkitt’s lymphoma 3. Ongoing monitoring of response to therapy in
b. Bone marrow damage – radiation, patients with malignancy
chemicals (benzene), and 4. Optimal sample for examination includes both
malignancies secondary to cancer the aspirate and core biopsy specimen
treatments 5. Posterior superior iliac crest commonly
c. Chromosome defects – some used; less commonly used anterior iliac crest
chromosomal abnormalities are and sternum
diagnostic for leukemic subtypes; t  Children – tibia
(15;17) is diagnostic for acute 6. Routinely assessed for cellularity
promyelocytic leukemia (hypocellular, hyper), M:E ratio (3:1),
- Translocated megakaryocyte evaluation, iron stored,
chromosomes differential
7. Assessment may also include flow cytometry,
cytogenetics, molecular, and microbiology
testing

Clinical effects of leukemia


 The neoplastic cells replace the normal bone
marrow
o Deficiency of red cells – anemia
o Deficiency of platelets --- thrombocytopenia –
bleeding
o Deficiency of white cells --- infection
o Increased cell turnover --- increase DNA
breakdown --- increase uric acid --- gout
d. Genetic factors – increase
incidence in down syndrome, Comparison of Acute and Chronic Leukemia
Fanconi, and others 1. Duration
e. Immune dysfunctions – heredity a. Acute – survival is week to months
and acquired defects in the immune without treatment; due to infection
system and bleesing
b. Chronic – survival is years without
treatment
2. Predominant cell type
a. Acute – immature/blast cells
predominant
1. Acute myelocytic leukemia –
has myeloblasta
2. Acute lymphoblastic leukemia –
has lymphoblasts
b. Chronic – maturing or mature cells
predominate
1. Chronic Myelocytic Leukemia –
has granuocytes
2. Chronic Lymphoblastic
Leukemia – has lymphocytes

Acute vs Chronic
Clinical manifestations and laboratory findings
a. acute – sudden onset; affects all ages form acute lymphoblastic leukemia
1. weakness and fatigue due to anemia (ALLs) (-)
2. petechiae and bruising due to (+) granules and monocytes
thrombocytopenia
3. fever and infection due to neutropenia
4. variable leukocyte count
5. marrow blasts > 20% based on WHO
classification or >30% based on fench-
american-british classification with cellularity
>70%
b. Chronic – frequently asymptomatic initially; affects
adults
1. anemia mild or absent
2. normal to slightly increase platelet count
3. WBC count usually high
4. Marrow cellularity is >70%

2. Sudan Black B
a. Stains phospholipids and lipoproteins
b. Granulocytic cells and Auer rods stains
Treatment positive (blue-black granulation);
a) Chemotherapy used is dependent on type of lymphocytic cells are negative for sudan
leukemia. Proper diagnosis is crucial black B (reaction parallels MPO)
b) Radiation c. Used to differentiate blasts of AML(+)
c) Bone marrow/stem cell transplant and ALL(-)
d) Supportive with transfusions of red blood cells
and platelets, antibiotics, growth factors

French-American-British (FAB) and WHO


1. Hematopoietic malignancy classifications
2. FAB classifications is based on cellular
morphology and cytochemical stain results,
FAB defines acute leukemia as >30% bone
marrow blasts Black – because high phospholipids and lipoproteins
3. WHO classification is based on cellular
morphology and cytochemical stains, but also
utilizes information obtained from
immunologic probes of cell markers,
cytogenetics,molecular abnormalities, and
clinical syndrome
4. WHO classification is now the standard for
diagnosis
5. FAB classification is easier to use and is still
widely taught.
3. Esterases
Cytochemical Stains a. Specific esterase stain )naphthol AS-D
1. Myeloperoxidase (MPO) chloroacetate esterase stain)
a. Cells of the granulocytic series and - Detects esterase enzyme present in
to a lesser degree the monocytic primary granules of granulocytic
series contain the enzyme cells; monocytic cells negative for
peroxidase in their granules that is this stain
detected by this stain. Auer rod also - Granulocyte (+)
stain positive; lymphocytic cells are - Monocyte (-)
negative to this stain
b. Used to differentiate blast of acute
myelogenous leukemias (AMLS) (+)
b. Nonspecific esterase enzyme stains (alpha-
napthyl acetate and alpha-naphthyl butyrate
- monocytes (+)
- granulocytes (-)
- detects esterase enzyme present in monocytic
cells; granulocytic cells negative for these
stains LAP score
- the esterase stains may be useful in 1. 100 neutrophils are graded on a scale from 0
distinguishing acute leukemias that are of to 4+ based on stain intensity and size of
myeloid origin (FAB M1, M2, M3, M4) from granules. Results are added together
those leukemias that are primarily cells of 2. Reference range is 13-130
monocytic origin (FAB M5)

4. Periodic acid—Schiff (PAS)


a. PAS stain intracellular glycogen
bright pink
b. Immature lymphoid cells, malignant
erythroblasts, and megakaryocytic
cells stain (+) ; myeloblasts and
normal erythrocytic cells are (-) with
this stain
c. Useful in diagnosis of 10 – 0 = 0
erythroleukemia (FAB M6) and acute 40 - +2 = 80
lymphoblastic leukemia 20 - +3 = 60
30 - +4 = 120
Lap score = 260

LAP Score clinical significance


1. Decrease LAP score: CML, paroxysmal
nocturnal hemoglobinuria (PNH)
2. Normal LAP score: CML is remission or with
infection, Hodgkin lymphoma in remission,
secondary lymphoma
3. Increased LAP score: neutrophilic keukomoid
reaction (NLR), polycythemia vera, CML in
blast crisis, late trimester of pregnancy

5. Leukocyte alkaline phosphatase (LAP)


a. Detects alkaline phosphatase
enzyme activity in primary granules
of neutrophils
b. A positive stain will show dark 6. Tartrate-resistance acid phosphatase stain
precipitate when alkaline (TRAP)
phosphatase activity is present; color a. Almost all blood cells contain the
is dependent on dye used acid phosphatase enzyme and show
c. Used to differentiate chronic positive with acid phosphatase
myelogenous leukemia (CML) from a stain. Once tartrate is added,
neutrophilic leukemois reaction staining is inhibited in most cells
(NLR) b. Only hairy cells from hairy cell
leukemia is resistant to inhibition
with tartrate and continue to stain
positive; all other cells stain
negative.
2. Small lymphoblasts, homogeneous
appearance
3. Best prognosis
4. Most T cell ALLs are FAB L1

b. FAB L2
1. Most common in adults
2. Large lymphoblasts, heterogenous
appearance
7. Perl’s Prussian blue stain
- Hemosiderin c. FAB L3
a. Free iron precipitates into small blue/green 1. Leukemic phase of Burkitt lymphoma
granules in mature erythrocytes; cells are 2. Seen in both adults and children
called siderocyte (retics w/ iron). Iron 3. Lymphoblasts are large and uniform with
inclusions are called siderotic granules or prominent nucleoli; cytoplasm stains deeply
Pappenheimer bodies when visible with basophilic and may show vacuoles
Wright’s stain. 4. Poor prognosis
5. ALL FAB L3 are B cell lineage
b. Sideroblasts are nucleated RBCs in bone
marrow that contain iron that encircles the d. Burkitt lymphoma
nucleus (blast w/ iron depositis). These are 1. High-grade non-Hodgkin lymphoma phase of
abnormal. FAB L3 leukemia
c. Increased percentage of siderocytosis seen in 2. Endemic in East Africa with high association
severe hemolytic anemias, and post- with Epstein-Barr virus; children present with
splenectomy; ringed sideroblasts are seen in jaw/facial bone tumors
bone marrow of myelodysplastic syndrome 3. U.S. variant seen in children and young
(refractory anemia with ringed sideroblasts adults; present with abdominal mass
[RARS] and sideroblastic anemias.
Immunophenotyping of ALL

CD marker characteristics of B cell lineage


1. Expressed by specific cell lines at different
maturation stages; as cell matures, loses
some antigens and expresses new ones
2. Progenitor B cells are CD19, CD34, and
TdT (terminal deoxynucleotidyl transferase)
positive; CD10 (CALLA), negative. This is
the least mature B cell.
3. Early-pre-B cells ALL are CD10 (CALLA),
CD19, CD34, and TdT positive. This is the
most common subtype
4. Pre-B cells ALL are CD10 (CALLA; common
acute lymphoblastic leukemia antigen), CD19,
CD20, and TdT positive. This is the second
Wright’s stain - can’t detect enzymes most common subtype.
5. B cells ALL (early B) are CD19, CD20
ACUTE LYMPHOPROLIFERATIVE DISORDERS positive; TdT negative. This is the most
1. Unregulated proliferation of the lymphoid stem mature B cell and least common subtype.
cell; classified morphologically using FAB criteria, 6. CD19 is the only marker expressed through
or immunologically using CD markers to all stages of B cells
determine cell lineage (T or B cell)
2. Clinical symptoms: Fever, bone/joint pain, CD marker characteristic of T cell lineage
1. Differentiated from B cells using markers
bleeding, hepatosplenomegaly
present on all T cells, including CD2, CD3,
3. Laboratory: Neutropenia, anemia, and CD5, and CD7 (pan T cell markers).
thrombocytopenia; variable WBC count, Immature T cells are TdT positive.
hypercellular marrow with bone marrow blasts 2. Immature T cells can have both or neither
≥20% (WHO)or >30% (FAB) CD4 and CD8. Mature T cells have one or the
4. Lymphoblasts stain PAS positive; Sudan black B other, but not both.
and myeloperoxidase negative 3. T cell ALL occurs most often in males;
mediastinal mass is a common finding.
FAB classification of acute lymphoblastic leukemia
(ALL) Genetic translocations are helpful in diagnosis.
a. FAB L1 Common ones include:
1. Most common childhood leukemia (2-to 10- a. FAB L3/Burkitt lymphoma – t(8;14) with a
year peak); also found in young adults rearrangement of the MYC oncogene
b. Pre-B cell ALL associated with t(9;22); B cell 1. Plasma cell neoplasms
ALL associated with t(4;11) a. Multiple myeloma
c. T cell ALL associated with t(7;11)  Monoclonal gammopathy causes
FAB Classification of ALL B cell production of excessive IgG
(most common) or IgA, with
decreasd production of the other
immunoglobulins
 found in adults over 60 years old;
incidence higher in males
 multiple skeletal system tumors of
plasma cells (myeloma cells) cause
lytic bone lesions and
hypercalcemia
 identified on serum protein
electrophoresis by an “M” – spike in
the gamma-globulin region;
immunoglobulin class determined
using an immunoassay method

CHRONIC LYMPHOPROLIFERATIVE DISORDERS


1. Chronic lymphocytic leukemia (CLL)
a. Found in adults over 60 years old; more
common in males (2:1); survival rate of 5-
10 years
b. B cell malignancy (CD19, CD20 positive)
c. Often asymptomatic and diagnosed
secondary to other conditions
d. Laboratory: bone marrow hypercellular;
blood shows absolute lymphocytosis of  Excessive IgG or IgA production by
>5.0x109/L; homogenous, small, myeloma cells causes increased
hyperclumped lymphocytes and smudge blood viscosity.
cells  Abnormal immunoglobulin binds to
e. Anemia is not usually present unless platelets, blocking receptor sites for
secondary to warn autoimmune coagulation factor binding; this result
haemolytic anemia (frequent in prolonged bleeding.
complications)  Laboratory: Bone marrow plasma
f. Small lymphocyte lymphoma (SLL) is the cells>30%,marked rouleaux,
lymphoma phase of CLL increased erythrocyte sedimentation
rate (ESR), blue background to
2. Hairy cell leukemia (HCL) blood smear, plasma cells and
a. Found in adults over 50 years old; more lymphocytes on blood smear
common in males (7:1)  Bence Jones proteins (free light
b. B cell malignancy (CD19, CD20 chains—kappa or lambda) found in
positive) th urine; toxic to tubular epithelial
c. Massive splenomegaly; extensive bone cells; cause kidney damage
marrow involvement results in dry tap on b. Waldenstrom macroglobulinemia
bone marrow aspiration  Monoclonal gammopathy causes B
d. Laboratory: pancytopenia, cytoplasm of cell production of excessive IgM
lymphocytes shows hair-like projections; (macroglobulin) and decreased
hairy cells are tartrate-resistant acid production of the other
phosphatase (TRAP) stain positive immunoglobulins.
 Found in adults over 60 years old
3. Prolymphocytic Leukemia (PLL)  Lymphadenopathy and
a. Found in adults; more common in males hepatosplenomegaly; no bone
b. Can be either B cell (most common) or tumors
T cell malignancy  Identified on serum protein
c. Marked splenomegaly electrphoresis by an “M” –spike in
d. Laboratory: characterized by the gamma-globulin region;
lymphocytosis (>100x109/L) with immunoglobulin class determined
prolymphocytes; anemia and using immunoelectrophoresis and
thrombocytopenia quantified using an immunoassay
e. both B and T cell types are aggressive method
and respond poorly to treatment  Excessive IgM production causes
increased blood viscosity
Other Lymphoid Malignancies
 Abnormal immunoglobulin may Burkitt leukemia), mantle cell, follicular,
interface with platelet function, fibrin and the other lymphomas
polymerization, and the function of - Cells can be small and mature (e.g.,
other coagulation proteins. small lymphocytic lymphoma) or large
 Laboratory: Marked rouleaux, and primitive (e.g., Precursor B cell
increased ESR, blue background to lymphoblastic lymphoma)
blood smear; plasmacytoid - Can be slow growing or very aggressive
lymphocytes, plasma cells, and
lymphocytes on blood smear c. Mycosis fungoides (cutaneous T cell
lymphoma)
2. Lymphoma - Classified by WHO as T/NK cell
 Proliferation of malignant cells in solid neoplasm (non-hodgkin lymphoma)
lymphatic tissue - Seen in patients over 50 years of age
 Initially localized; may spread to bone - Cutaneous lymphoma causes skin
marrow and blood itching, leading to ulcerative tumors
 Clinical symptom: Lymphadenopathy - Sezary syndrome , a variant mycosis
 Diagnosis: Tissue biopsy, CD surface fungoides, presents as a disseminated
markers, cytogenetics, DNA disease with widespread skin
analysis/PCR involvement and circulating lymphoma
cells
 World Health Organization (WHO)
- CD2, CD3, and CD4 positive
groups the lymphomas into Hodgkins, B
cell,and T/NK cell (non-Hodgkin)
ACUTE MYELOPROLIFERATIVE DISORDERS
neoplasms.
1. Unregulated proliferation of the myeloid stem
a. Hodgkin lymphoma (classical)
cell; classified using morphology, cytochemical
- 40% of lymphomas; seen in patients
stains, CD markers, cytogenetics; WHO
between 15 and 35 years of age and over
classified standard of diagnosis; FAB
55 years of age; seen more frequently in
classification still widely taught
males; certain subtypes have an Epstein-
2. Platelets, erythrocytes, granulocytes, and/or
Barr virus (EBV) association
monocytes can be affected.
- Reed-Sternberg (RS) cells found in
3. Found mainly in middle-aged adults, also
lymph node biopsy are large, multi-
children <1 year old
nucleated cells each with prominent, large
4. Clinical symptoms: fever, malaise, weight loss,
nucleoli; B cell lineage
petechiae, bruises, mild hepatosplenomegaly
5. Laboratory: neutropenia, anemia, and
thrombocytopenia; variable WBC count;
hypercellular marrow with bone marrow blasts >
20% (WHO) or >30% (FAB)

1. Acute Myelogenous Leukemia (AML)

Acute myelogenous leukemia


FAB M0—Blasts exhibit myeloid markers CD13,
- Hodgkin lymphoma subtypes using WHO
CD33, and CD34 but stain negatively with the usual
classification:
cytochemical stains, myeloperoxidase (MPO), and
a. Nodular sclerosis—70% are this
Sudan black B (SBB). Constitutes
subtype; lowest EBV association
b. Mixed cellularity—20% are this
FAB M1 (AML without maturation)
subtype; highest EBV association
- shows 90% or more marrow myeloblasts;
c. Lymphocyte rich
may have Auer rods (fused primary granules)
d. Lymphocyte depleted—uncommon
- Laboratory: : Mild anemia, eosinophilia,
FAB M2 (AML with maturation)
and monocytosis; increased LAP score
- shows <90% marrow myeloblasts; may have
and ESR during active desease
Auer rods; chromosome abnormality t(8;21)
b. Non-Hodgkin lymphoma
- WHO separates B cell and T/NK cell  Both FAB M1 and FAB M2 are SBB, MPO,
neoplasms into conditions with precursor and specific esterase positive
cell of mature cells.  FAB M1 and FAB M2 account for 50% of the
- 60% of lymphomas; seen in patients AMLs
over 50 years of age; seen more  CD13 and CD33 positive (pan myeloid
frequently in males markers)
- Enlarge lymph nodes or gastrointestinal
(GI) tumors Acute promyelocytic leukemia (APL; FAB M3)
- B cell neoplasms are more common;  Characterized by >30 marrow
include Burkitt (lymphoma phase of promyelocytes with bundles of Auer rods
(faggot cells); heavy azurophilic granulation
 Clinical symptoms: severe bleeding,  Biphenotypic leukemias occur when myeloid
hepatomegaly, and disseminated and lymphoid antigens are expresses on the
intravascular coagulation (promyelocytes same cell; poor prognosis
have procoagulant activity)  The WHO classification of acute myeloid
 Account for 5% AMLs leukemias has more than 20 subtypes; all
 SBB, MPO and specific esterase positive have ≥20% marrows blast.
CD13 and CD33 positive; diagnostic
chromosome abnormality t(15;17);
PML/RARA oncogene involved

Acute myelomonocytic leukemia (AMML; FAB M4)


 Characterized by > 20% (WHO) or >30%
(FAB) marrow blasts; NAEGELI’s type of
leukemia
 Myeloblasts with >20% cells of monocytic
origin; may have Auer rods
 Proliferation of unipotential stem cell CFU-GM
that gives rise to both granulocytes and
monocytes
 Account for 30% of AMLs
 Increase urine/serum lysosome
 SBB, MPO, and specific and nonspecific
esterase positive
 CD13 and CD33 positive (myloid) and CD14
positive (monocytes)

Acute monocytic leukemia (AMoL; FAB M5)


 Characterize by ≥20% (WHO) or >30%
(FAB) marrow monoblasts
 Account for 10% of the AMLs
 Nonspecific esterase positive; CD14 positive
 Contains two variants:
o M5a is seen in children with >80%
monoblasts in the bone marrow.
Also known as Schilling’s Leukemia
o M5b is seen in middle-aged adult
with <80% monoblasts in the bone
marrow

Acute erythroleukemia (AEL, Di Guglielmo


syndrome; FAB M6)
 Characteristic by ≥20% (WHO) or >30%
(FAB) marrow myeloblasts and >50%
dysplastic marrow normoblasts
 Account for 5% of the AMLs •
 Malignant normoblasts are PAS positive. The
myeloblasts are SBB and MPO positive

Acute megakaryocytic leukemia (AMegL; FAB M7)


 Characterize by a proliferation of
megakaryoblasts and atypical
megakaryocytes in the bone marrow; blasts
may have cytoplasmatic blebs
 Accounts for <1% of the AMLs
 Marrow aspiration results in dry tap; blood CHRONIC MYELOPROLIFERATIVE DISORDER
shows pancytopenia  Characterized by hypercellular marrow,
 Difficult to diagnose with cytochemical stains erythrocytosis, granulocytosis, and
 CD41, CD42 and CD61 (platelet markers) thrombocytosis
positive  Defect of the myeloid stem cell
 Named for the cell line most greatly affected
AMLs  All may terminate in acute leukemia
 Bilineage leukemias contain two cell  Molecular diagnostic studies are helpful in
populations. One population expresses identifying oncogenes.
myeloid antigens; the other population  JAK2 oncogene is implicated in the
expresses lymphoid antigens. polycythemia vera (80%), chronic idiopathic
myelofibrosis (50%), and essential  increase in all cell lines (polycythemia);
thrombocythemia (40%). erythrocytes most greatly increased
 The BCR/ABL oncogene is associated with despite decreased erythropoietin (EPO);
chronic myelogenous leukemia. inappropriate erythropoiesis
Chronic myelogenous leukemia (CML)  High blood viscosity can cause high
Chronic myelogenous leukemia (CML) presents with blood pressure, stroke, and hearth
proliferation of granulocytes. attack.
 Found mainly in adults 45 years of age and  Found in adults 50 years of age and
older; often diagnosed secondary to other older
conditions  Laboratory: Increased RBC (7-10 x
 Clinical symptoms: Weight loss, 1012/L), hemoglobin (>20g/dL), and
splenomegaly, fever, night sweats, and hematocrit (>60%) along with increased
malaise
leukocytes and platelets indicate
 Bone marrow has an increased M:E ratio
polycythemia. RBC mass is increased
 Laboratory: Blood findings include mild
anemia and WBC between 50 and 500 X with a normal plasma volume.
109/L, with all stages of granulocytes  Treatment is therapeutic phlebotomy,
production (shift to the left), including early splenectomy, and chemotherapy. PV is
forms of eosinophils and basophils. a chronic with a life expectancy after
Myelocytes predominate; may have a few diagnosis of up to 20 years
circulating blasts  Must differentiate from other form of
 CML can mimic a neutrophilic leukemoid polycythemia
reaction (NLR). LAP score is used to
-Secondary polycythemia
differentiate; LAP is low in CML and high in
NLR.  Increased in RBC mass is an
 Philadelphia chromosome, t(9;22), is present appropriate response to increased EPO
in virtually all patients. All cell lines are or tissue hypoxia. Plasma volume,
affected except lymphocytes. The few who leukocyte count, and platelet count are
lack the chromosome have a worse normal.
prognosis.  Can be caused by smoking,
 Chronic phase can last up to 5 years; emphysema, or high altitude
accelerated phase (blast crisis)  Must differentiate from other form of
 ultimately leads to acute leukemia in most
polycythemia
patients. Recent therapies are improving the
prognosis. -Relative (pseudo-) polycythemia
 Decreased plasma volume with normal
RBC mass caused by dehydration
(diarrhea, diuretics, or burns)
 Increased hemoglobin, normal leukocyte
and platelet count, normal EPO

Chronic idiopathic myelofibrosis


 Myeloid stem cell disorder characteristic
by proliferation of erythroid, granulocytic,
and megakaryocytic precursors in
marrow with dyspoiesis
 Progressive marrow fibrosis
 Found in adults 50 years of age and
older
Essential thrombocythemia (ET)  Clinical symptoms: bleeding due to
 Characterized by proliferation of
abnormal platelet function;
megakaryocytes
extramedullary hematopoiesis causes
 Found mainly in adults 60 years of age
splenomegaly and hepatomegaly
and older
 Laboratory: Anisocytosis, poikilocytosis
 Laboratory: platelets commonly greater
with teardrop cells, leukoerythroblastic
than 1000 x 109/L, giant forms, platelet
anemia (immature neutrophils and
function abnormalities, leukocytosis
nucleated RBCs in circulation);
 Must differentiate from reactive abnormal morphology associated with
thrombocytosis and polycythemia vera all cell lines
Polycythemia vera (PV) Myelodysplastic Syndrome (MDS)
 Malignant hyperplasia of the
multipotential myeloid stem cell causes
a. Group of acquired clonal disorders
affecting the pluripotential stem cell;
characterized by progressive blood
cytopenias despite bone marrow
hyperplasia
b. Dyspoiesis affects erythroid, myeloid,
and megakaryocytic cell lines. High
incidence of terminating in acute
myelogenous leukemia occurs.
c. MDS development can be triggered by
chemotherapy, radiation, and chemicals. 3) Chronic myelomonocytic leukemia
d. Found in older adults; rarely found in (CMML)
children and young adults - The one MDS that usually present
e. Hematologic evidence of dyspoiesis: with leukocytosis
1) Erythroid: Variable anemia; - Laboratory: Bone marrow blasts 5-
erythrocytes can be macrocytic 20% and peripheral blood blasts
(with oval macrocytes) or <5%; absolute monocytosis greater
microcyte and hypochromic; than 1.0X109/L
dimorphic erythrocytes,
poikilocytosis, Howell-Jolly
bodies, basophilic stippling,
Cabot rings, nucleated RBCs
2) Myeloid: neutropenia,
hypogranulation,
hyposegmentation of neutrophils,
shift to the left
3) Thrombocytes: Variable platelet
count, giant platelets,
hypogranulation, 4) Refractory anemia with excess blasts
micromegakaryocytes (RAEB)
- Trilineage cytopenias common
5 subgroups of MDS using the FAB - Laboratory: Bone marrow and
classification in scheme; up to 30% blasts in peripheral blood blasts are the
the bone marrow same as with CMML, but there is
1) Refractory anemia (RA) no absolute monocytosis.
- Anemia that is refractory (not - The higher the blast percent, the
responsive) to therapy worse the prognosis
- Laboratory: Oval macrocytes,
reticulocytopenia, dyserythropoiesis; 5) Refractory anemia with excess blasts in
bone marrow blasts transformation (RAEB-t)
2) Refractory anemia with ringed - Laboratory: bone marrow blasts
sideroblasts (RARS) >20% but less than 30% peripheral
- Ringed sideroblasts comprise more blood blasts >5%
than 15% of bone marrow nucleated - WHO classification reassigns
cells. Signs of dyserythropoiesis, RAEB-t as an acute leukemia
neutropenia instead of a myelodysplastic
- Laboratory: Similar to RA; dimorphic syndrome because of the bone
erythrocytes marrow blast percent
- This is the primary/idiopathic
sideroblastic anemia discussed with WHO classification of MDS has
the anemias additional groups (e.g., refractory
cytopenia with multilineage dysplasia,
5q deletion syndrome).
WHO created the new category of
myelodysplastic/myeloproliferative
disease, which the FAB’s CMML

Features of PBS and BM in MDS

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