Hema Leukemia Ajfb

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Abnormal, uncontrolled proliferation and accumulation of one or more hematopoietic cells

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Leukemia

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Leukemia that is rapidly progressive

Lasts for several days to 6 Mos

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Acute Leukemia

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Duration of Subacute Leukemia

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2-6 Mos

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Variable Duration, depending on the age of the px and type of cell involved

Most pxs lived a minimum of 1-2 Years or more

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Chronic Leukemia

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WBC Count >15,000/uL

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Leukemic Leukemia

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WBC Count <15,000/uL

With Immature or Abnormal forms of WBCs in peripheral blood

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Subleukemic Leukemia

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WBC Count of <15,000/uL

With No Immature or Abnormal WBCs in peripheral blood

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Aleukemic Leukemia

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Leukemia w/ predominance of immature cell types

(blasts & pro stages)

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Acute Leukemia

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Abrupt Onset of Symptoms

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Leukemia w/ predominance of Mature Cells

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Chronic Leukemia

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Gradual Onset of Symptoms

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FAB Criteria for Leukemia Include:

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1. Morphology

2. Cytochemistry

3. Immunophenotyping

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WHO Criteria for Leukemia

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1. Morphology

2. Cytochemistry

3. Immunophenotyping

4. Cytogenetics

5. Clinical Features

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FAB Diagnostic Criteria of AML

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>/= 30% Blasts

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WHO Diagnostic Criteria for AML

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>/= 20% Blasts

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*Widely Used Criteria

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Acute Lymphoblastic Leukemia type in which Lymphoblasts are small & homogenous

Most common type of

Childhood ALL

Best prognosis

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L1

Hint 14

Cytoplasm: Scanty

Nuclei: Inconspicuous

Nucleus: Round & Irregular

*Lymphoblasts have a very high N/C Ratio

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ALL Type in which Lymphoblasts are larger and variable in size (Large Heterogenous)

Adult Type ALL

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L2

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Cytoplasm: Abundant, Basophilic

Nuclei: Clefted, W/ nucleoli

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ALL Type in which Lymphoblasts are Large but varies little in size (Large Homogenous)

Burkitt Type

Rarest

Prognosis is Poor

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L3

Hint 16

W/ Prominent Vacuoles

Cytoplasm: Moderate in quantity, deeply basophilic

Nucleus: Round w/ fine chromatin structure, 1-3 nucleoli

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M7

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Acute Megakaryocytic Leukemia

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1. Large & Small Megakaryoblasts

2. High N/C Ratio

3. Pale Agranular Cytoplasm

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Acute Myelomonocytic Leukemia

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M4

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1. Both Myeloid & Monocytic Cells are present (20% of Total Leukocytes)

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M0

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Acute Myeloid Leukemia, minimally differentiated

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Undiffetentiated Blasts

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Acute Monocytic Leukemia,

poorly differentiated

(w/out maturation)

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M5a

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M2

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Acute Myeloid Leukemia

w/ Maturation

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Myeloid cells demonstrate maturation beyond blast and promyelocyte stage

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M6

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Acute Erythroleukemia

Erythremic Myelosis

Di Guglielmo's Syndrome

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1. Abnormal prolif of both erythroid & granulocytic precursors

2. May also include abnormal megakaryocytic, and monocytic prolif

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Acute Monocytic Leukemia

Well differentiated

(w/ maturation)

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M5B

Hint 23

>80% Monocytic Cells

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M1

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Acute Myeloid Leukemia

w/out Maturation

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Acute Promyelocytic Leukemia

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M3

Hint 25

1. Translocation in 15 & 17

2. DIC may occur

3. Presence of Faggot Cells w/mass of Auer Rods

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Myelodysplastic Syndrome in which there is

1. <1% of blasts in the Peripheral Blood

2. <5% of blasts in BM

3. May or may have ringed sideroblasts

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Refractory Anemia or Refractory Cytopenia

(RA/RC)

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Myelodysplastic Syndrome in which there is

1. <1 % of blasts in Peripheral Bld

2. <5% of blasts in BM

3. >15% RINGED SIDEROBLAST

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Refractory Anemia w/ Ringed Sideroblasts

(RARS)

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Myelodysplastic Syndrome in which there is

1. <5% of Blasts in Peripheral Bld

2. 5-20% of blasts in BM

3. May or may have Ringed Sideroblast

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Refractory Anemia w/ Excess Blasts (RAEB)

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Myelodysplastic Syndrome in which there is

1. >5% of Blasts in Peripheral Bld

2. 20-30% Blasts in BM

3. May or may have Ringed Sideroblast

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Refractory Anemia w/ Excess Blasts in Transformation (RAEBIT)

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Myelodysplastic Syndrome in which there is

1. <5% Blasts in peripheral bld

2. 5-20% Blasts in BM

3. No ringed sideroblast

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Chronic Myelomonocytic Leukemia

(CMML)

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Clonal Abnormality in Hematopoietic tissues

Pre-leukemia that can progess to ALL

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Myelodysplastic

Syndrome (MDS)/

Dysmyelopoietic

Syndrome

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Majority of cases appear to involve B Lymphocytes (Fragile than normal)

Large number of Smudge Cells

More than twice as common in men than in women

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Chronic Lymphocytic Leumemia (CLL)

Hint 32

A Lymphoproliferative Disorder

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Group of malignant tumors of lymphoid tissue (lymph nodes & spleen)

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Lymphoma

Hint 33

Types:

1. Non-Hodgkin's

2. Hodgkin's

3. Burkitt's

4. Sezary Syndrome

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Proliferations of malignant lymphocytes (primarily B cells) that are arrested at certain ages of maturation

Classified by Rappaport System

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Non-Hodgkin's Lymphoma

Hint 34

Frequent in middle & older age

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Presence of Reed-Sternberg Cells (Large Cell w/ large nucleoli)

The cells reacting to neoplasm is predominant rather than the neoplastic cells

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Hodgkin's Disease/Lymphoma

Hint 35

Frequent in young & middle aged

Classified According to:

1. Rye Classification

- Histolologic Apperance of Lymphoid Tissue

2. Ann Arbor Classification

- Most widely used staging

-Histoligic type & extent of tissue involvement

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Found most often in children of Africa & New Guinea commonly affects jaw & facial bones

EBV isolated from tumor cells plays a role in transforming the B lymphocytes by binding to surface receptors

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Burkitt's Lymphoma

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Leukemic Phase of

Mycosis Fungoides

affecting predominantly older males

Malignant lymphoma, affecting the skin, involves primarily T lymphocytes

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Sezary Syndrome

Hint 37

T Lymphocytes (+) monoclonal abs for CD2, 3, 4

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Resemble a medium-sized lymphocyte w/ convoluted ceribriform (brain-like) nucleus, resembling monocyte nucleus

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Sezary Cells

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(+) Acid Phosphatase Stain using

L Tartaric Acid

Cells are resistant to Inhibition by Tartaric Acid

Characteristic B lymphocytes

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Hairy Cell Leukemia

Hint 39

Leukemic Reticuloendotheliosis

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Group of acquired malignant disorders that develop from the proliferation of an abnormal pluripotential stem cell

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Myeloproliferative Disorders

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Stem cell disorder affecting the granulocytic, monocytic, erythrocytic, megakaryocytic cell lines

One arm of Chromosome 22 is

translocated to Chromosome 9 (Philadelphia Chromosome)

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Chronic Myelogenous Leukemia/Chronic Granulocytic Leukemia

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Indication of

(-)

Philadelphia Chromosome

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1. Poorer Prognosis

2. Do not respond Well to therapy

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Difference b/w CML & Leukemoid Reaction

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CML:

1. High WBC Count

2. Shift to the left w/ blasts, eosinophilia, & basophilia

3. LOW LAP

4. (+) Philadelphia Chromosome

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Leukemoid Reaction:

1. High WBC

2. Shift to the left (blasts rare), toxic granulation, dohle bodies

3. HIGH LAP

4. (-) Philadelphia Chromosome

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Fibrosis and granulocytic hyperplasia of the BM, w/ granulocytic and megakaryocytic proliferation in the liver & spleen

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Myelofibrosis w/ Myeloid Metaplasia

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Chronic MPD w/ thrombocytosis in excess of 1000 x 10^9/L w/ spontaneous aggregation of functionally abnormal platelets

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Essential

Thrombocythemia

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Characterized by an

Inc in RBC, WBC, PLTS

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Polycythemia Vera

Hint 46

Panhyperplasia

BM: Panmyelosis

BLD: Pancytosis

Dec EPO, Inc HCT

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Above normal Hgb, RBC,

Hct >50%

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Polycythemia/Erythrocytosis

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Chronic Myeloproliferative Dse

Inc RBCs, WBCs, Plts

D/t Increased RBC Conc, Inc Blood viscosity the px exhibits Ruddy Skin Coloration

Dec EPO

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Primary Absolute Polycythemia/

Polycythemia Vera

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Appropriate response to Hypoxia

Caused by:

1. Residence @ high altitude

2. Pulmonary & Cardiac Dse

Inc RBCs, WBCs, Plts

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Secondary Polycythemia w/ appropriate pxn of EPO

Hint 49

Secondary Polycythemia w/ Inappropriate Pxn of EPO

- Seen in certain tumors

- Normal RBCs, WBCs, Plts

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Caused by a dec in the fluid (plasma) portion of the blood

Actual # of RBC not Inc, but the # of cells per unit volume is Inc

Acute Dehy, Stress, Anxiety

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Relative Polycythemia

Hint 50

Spurious Polycythemia

- Gaisbock's Syndrome

- Occurs primarily in middle-aged males & may be associated w/ smoking, cardiovascular problems, hypertension & diuretic therapy

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Marker for primary granules and Auer rods

Deteriorates, should be done only on fresh specimens

Dark Brown Granules in cytoplasm of granulocytes and monocytes

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Myeloperoxidase (MPO)

Hint 51

(+) AML

(-) ALL

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Marker for phospholipids & lipids

Can be done on stored specimens

Dark Purple Granules in neutrophil precursors

Lymphoblasts (-)

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Sudan Black B (SBB)

Hint 52

(+) AML

(-) ALL

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Marker for immature lymphocytes

DNA Polymerase, Immunoperoxidase

Present in 90% of ALL

Differentiate ALL from AML

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Terminal

Deoxyribonucleotidyl

Transferase (TdT)

Hint 53

(+) ALL

(-) AML

Front 54

Marker for glycogen, glycoproteins mucoproteins, & high molecular weight carbohydrates

Activity results in

Bright Fuchsia Pink

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Periodic Acid-Schiff

Hint 54

Normally All Blood Cells are (+) exc:

Erythrocytes

L1 & L2:

BLOCK or Chunky PATTERN

L3:

NEGATIVE

Erythroblasts in M6 Leukemia:

POSITIVE

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Marker for mature & immature neutrophils & mast cells

Bright Red Granules in Cytoplasm

Specific Esterase

Stable Enzyme, lasts for months

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Naphthol AS-D Chloroacetate Esterase

Hint 55

Differentiates

Granulo (+) from Mono (-)

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Marker for monocytes, megakaryocytes, & plasma cells

Monocytes are Red-brown

Non-specific Esterase

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A-Naphthyl Acetate Esterase

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For id of monocytes, promonocytes, monoblasts

Dark Red precipitates in cytoplasm

Non-specific Esterase

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A-Naphthyl Butyrate Esterase

Hint 57

Specific = Mono (+) Granulo(-)

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Present in all hematopoietic cells & lysosomes

Purple to Red Granules

Can't be stored

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Acid Phosphatase

Hint 58

(+) Hairy Cell Leukemia using L (+) Tartaric Acid

(+) Trap

(-) Non T Cell Leukocytes

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Excellent marker for

Hairy Cell Leukemia

Purple to Dark Red granules in cytoplasm

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Tartrate Resistant Acid Phosphatase

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Only Neutrophils contains this activity, stains its ALP

Used to differentiate CML (Low) w/ Leukemoid Reaction (High)

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Leukocyte Alkaline Phosphatase

Hint 60

Specimen:

1. Fresh Capillary Bld (Heparin)

EDTA = Falsely Low

100 Cell Count

Scoring:

0 no activity

4 w/ large amount of activity

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Binds w/ acid mucopolysaccharides in blood cells

Useful for recognition of mast cells & tissue basophils

Strongly Metachromatic

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Toluidine Blue

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Color Reactions:

1. MPO

2. Sudan Black B

3. PAS

4. Naphthol AS-D Chloroacetate Esterase

5. A-Naphthyl Acetate Esterase

6. A-Naphthyl Butyrate Esterase

7. ACP

8. Tartrate Resistant ACP

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1. MPO

- Dark Brown

2. Sudan Black B

- Dark Purple

3. PAS

- Bright Fuschia Pink

4. Naphthol AS-D Chloroacetate Esterase

- Bright Red

5. A-Naphthyl Acetate Esterase

- Red Brown

6. A-Naphthyl Butyrate Esterase

-Dark Red

7. ACP

-Purple to Red

8. Tartrate Resistant ACP

-Dark Purple to Red

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Most common type of microscope used in Hema

Examine Blood Films

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Brightfield Microscopy

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Ensures an optimum contrast and resolution for the maximum definition of the specimen details by precisely focusing and centering light path and spreading the light uniformly over the field view

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KOEHLER/KOHLER ILLUMINATION

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Type of Brightfield Microscopy that gives a 3D Image

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Interfernce Contrast Microscope

Hint 65

Hoffman:

MODULATION CONTRAST

Nomarski:

DIFF INTERFERENCE CONTRAST

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Microscope used for ANA &

T & B-Cell studies

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Fluorescence Microscopy

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Used for observation of fine ultrastructure of cells w/ magnification of approx.

X100, 000

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Electron Microscopy

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Used for Manual Platelet Counts

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Phase-Contrast Microscopy

Hint 68

Halo

Front 69

Used for observation of erythrocyte morphology, & differentiates leukocytes

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Oil Immersion Microscopy

Hint 69

Solution for Cleaning:

1. Lens Cleaner

2. Alcohol