Neoplastic disorders part 2 Flashcards
What conditions are included in Myeloproliferative Neoplasms?
- Chronic myelogenous Leukemia
- Polycythemia Vera
- Primary myelofibrosis
- Essential Thrombocytosis/Thrombocythemia
*an MPN that originates in an abnormal pluripotent bone marrow stem cell
*and is consistently associated with the BCR-ABL 1 fusion gene located in the Philadelphia chromosome
Chronic Myelogenous Leukemia (CML)
reciprocal translocation of DNA between chromosomes 9 and 22 (t9q:22q)
Philadelphia chromosome
*an absolute increase in red cell mass, leukocytosis, and thrombocytosis. *Panhyperplasia of the bone marrow
*Pancytosis in peripheral blood
Polycythemia vera
Polycythemia vera is characterized by what mutation?
JAK2 V617F
both are increased in PV
Serum and urine Erythropoietin
systemic bone marrow fibrosis and extramedullary hematopoiesis
Primary fibrosis
caused by infiltrative disorders, including malignancies and infections, or exposure to chemical toxins or
Secondary myelofibrosis
__________ - replaced by abnormal cells
__________ - fibrotic tissue
- myelopthisic
- myelofibrosis
a clonal myeloproliferative disorder primarily affecting the megakaryocytic lineage with the principal manifestation of sustained thrombocytosis
Essential thrombocythemia
the ‘millionaires’
Essential thrombocythemia
*disorders in which apoptosis predominates, hematopoiesis is ineffective, and cytopenias occur
*chronic myelomonocyticleukemia (CMML),
*atypical chronic myeloid leukemia (aCML),
Myelodysplastic syndromes
Dysgranulopoiesis
- Pseudo Pelger Huet
Dysmegakaryocytopoiesis
giant hypogranular platelets
cytochemicals in order to identify a particular or differentiate leukemias
cytochemistry
To differentiate a myelogenous or monocytic leukemia from acute lymphocytic leukemia
Myeloperoxidase Reaction
___________ is present in the primary azurophilic granules of neutrophil, eosinophil and monocyte
Peroxidase
interpretations for myeloperoxidase reaction
Red brown peroxidase:
Auer rods:
Monocytes:
Lymphoblast and lymphoid cells:
– present in neutrophil and eosinophil
- strongly positive
- negative to diffusely +
- negative
interpretations for myeloperoxidase reaction
Acute leukemia infection & myelodysplasia:
Basophils:
Peroxidase:
- show negative stain
- increase in CML
- may stain positive in granulocytic leukemia
- may show results similar to those of sudan black B
if Myeloperoxidase (MPO) or Sudan Black B (SBB) stain is used, which type of leukemia would show positive ad negative stain?
Positive:
AML
Negative:
M6, Mixed
Show positive stain if Chloroacetate Esterase (CAE) is used
Myelocytic:
M1, M2, M3, M4
Show positive stain if Non-specific Esterase is used (NSE)
Monocytic:
M4, M5
Show positive stain in Periodic Acid Schiff is used (PAS)
ALL, M6, M7
To distinguish acute myelogenous and monocytic leukemia from lymphocytic leukemia.
Sudan Black B (SBB)
Principle of Sudan Black B (SBB)
- Sudan black B dye is fat soluble,then it stains fat particles which present in the primary and secondary granules of myelocytic and monocytic cells.
interpretations for SBB
- Positive stain in patients with AML
- not black staining cytoplasmic granules in myeloblasts
- Myelogenous cells show coarse staining (faint staining pattern for myeloblast and increases staining with maturation)
- Auer rods = positively stained
- Monocytic cells show finely scattered granules
- negative lymphocytic stain
- L3 Burkitt type shows positive staining vacuoles
Differentiate myelocytic and monocytic leukemia
Non-specific Esterases (w/ fluoride inhibition)
principle of this test is WBCS contain esterases, a group of lysosomal enzymes
*show positive black red stain in megakaryocytes, platelets, histiosyte macrophage monocyte & lymphoblast of ALL
*negative for granulocytes
Non-specific Exterase
with the addition of this reagent to incubation solution, it inhibits the staining reaction in monocytes but not in granulocytes
Sodium fluoride
principle of this test is when naphthol AS-D chloroacetate is used as a substrate, the reaction is positive in the granulocytic cells and negative to weak in the monocytic cells
Specific Esterase or Chloroacetate
interpretations for Specific esterase Choloroacetate
Myeloid cells:
Monocyte and Basophils:
Other cells (lymph-plasma-megakaryocyte-nrbc):
Auer rods:
- positive
- negative to weakly positive
- negative
- positive
Cytochemical stains for differentiation of Acute leukemia for AML and ALL
Myeloperoxidase:
Sudan Black:
Napthol-AS-D Chloroactetate esterase (specific esterase):
Periodic Acid Schiff (PAS):
AML
- Pos
- Pos
- Pos
- Neg or diffusely pos
ALL
- Neg
- Neg
- Neg
- Pos (coarsely granular or block like)
*Diagnosis of some acute lymphocytic leukemia
*subtypes of AML
Periodic Acid Schiff (PAS) Reaction
principle for Periodic Acid Schiff
the stain indicates the presence of mucoproteins, glycoproteins and high molecular weight carbohydrates in blood cells.
Interpretations for PAS
Neutrophils:
Myeloblasts:
Eosinophils:
Monocytes:
Lymphocytes:
NRBC:
Megakaryocytes:
- positive at most stages of development (strongly in mature stage)
- weakly positive or negative
- positive in stages of development
- weakly positive (fine granules)
- may contain few positive granules
- negative (positive in abnormal erythroid precursors of erythroleukemia
- positive
which malignant cell has presence of increased number of PAS-positive granules in a focal or block-like positivity?
malignant lymphocytes in lymphoblastic leukemia
Distinguishing the cells of leukemoid reactions with increase activity from these of (CML)with decreased activity.
Leukocyte Alkaline phosphatase (LAP)
principle for Leukocyte Alkaline Phophatase
Alkaline phosphatase -
Activity is present in varying degrees in the neutrophil and band form of the granulocytes/sometimes in B lymphocytes
how to perform LAP score?
Count 100 neutrophils and score them (0/+4), then multiply the score by the number of cells with that score , then calculate the final score by adding the total scores.
Normal range for LAP score
30-185
LAP score grading
No stain:
Faint and diffuse staining:
Pale with a moderate amount of blue staining:
Strong blue precipitated stain:
deep blue or brilliant staining with no visible cytoplasm:
- 0
- +1
- +2
- +3
- +4
LAP is elevated in
- Leukemoid reaction
- Pregnancy (3rd trimester)
- Polycythemia vera
- Aplastic anemia
- Mutilple myeloma
- Obstructive jaundice
- Hodgkins’ disease
LAP decreased in
- CML
- Paroxysmal Nocturnal Hemoglobinuria
- Sickle Cell anemia
- Hypophosphatasia
- Sideroblastic anemia
- Myelodysplastic anemia
CML has a LAP score of?
<10
Will not affect LAP result:
1.Untreated hemolytic anemia.
2.Lymphosarcoma.
3.Viral hepatitis.
4.Secondary polycythemia.
Siderotic granules are found in the cytoplasm of developing cells in [BM] in the form of Ferric[Fe+3]
Prussian Blue Reaction
Siderotic granules are found in?
NRBCs and some Reticulocytes
___________ hydrolyzes the substrate napthol AS-BI phosphoric acid. When hydrolyzed,
*this substrate couples with a dye such as fast garnet GBC and produces red precipitate at the site of enzyme activity
Acid Phosphatase
