Term
| What cells are stained by myeloperoxidase stain and how do they stain? |
|
Definition
| MYELOID cells stain primary granules a dark brown-black precipitate...also LIGHTLY stains MPO found in monocytes |
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Term
| Purpose: myeloperoxidase stain |
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Definition
| differentiates myeloid from lymphoid blasts..is the most sensitive and specific stain for granulocytes |
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Term
| What cells are stained by the sudan black stain and how do they stain? |
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Definition
Sudan Black stains the phospholipid membrane of primary granules a BLACK color
It also lightly stains the primary granules of monocytoid cells a black color |
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Term
| What is the purpose of the Sudan Black stain? |
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Definition
| The results parallel the myeloperoxidase stain |
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Term
| When would you want to use the Sudan black stain over the MPO stain? |
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Definition
| Use sudan black over MPO when fresh specimens are not avaliable |
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Term
| What cells stain with specific esterase? How do they stain? |
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Definition
| Myeloid cells...they stain dark blue with naphthol AS-D chloroacetate |
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Term
| What cells stain with non-specific esterase? How do they stain? |
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Definition
Monocytoid cells...they stain brown **NSE is a specific marker for early nomos and macrophages
ALSO with NSE, see brown focal positivity in ALL lymphoblasts, dot-like + pattern in T-lymphs, also + are plasma cells |
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Term
| Purpose: Combined esterase stain |
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Definition
Differentiates M4 from M5
M4: Blue AND Brown blasts...
M5: All Brown Blasts; segs stain BLUE |
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Term
| PURPOSE: Fluoiride inhibition test |
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Definition
| addition of NaFl inhibits staining of monocytes with non-specific esterase stain..sooo a check for AML M4 |
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Term
| What cells stain with the PAS stain and how do they stain? |
|
Definition
MATURE granulocytes stain strongly, and they increase in intensity with increased maturity
monocytes are positive
lymphoBLASTS are positive with block-like staining
Lymphocytes are positive and granular with coarse staining
megakaryocytes stain positive with varying intensity anf pattern
ABNORMAL ERYTHROBLASTS STAIN ++ |
|
|
Term
| What is the purpose of the PAS Stain? |
|
Definition
| This stain is useful for leukemic blasts in Erythroleukemia, Acute lymphoblastic leukemia, and acute megakaryocytic leukemia |
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|
Term
| What cells stain with the LAP stain? |
|
Definition
| mature neutrophils and bands are positive..activity increases with neutrophil maturity |
|
|
Term
|
Definition
To differentiate between CML and a leukemoid reaction
CML=LOW score
Leukemoid rxn=HIGH score |
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Term
| In what disease state do we see a "leukemic histus" (arrest in maturation) |
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Definition
|
|
Term
| What is the diagnostic characteristic of the BONE MARROW in AML? |
|
Definition
| It has >20% (WHO) or >30% (FAB) BLASTS! |
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|
Term
| What aspect of the cell does the PAS stain? |
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Definition
|
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Term
| Describe the pathophysiology of acute leukemia |
|
Definition
There is a malignant transformation of stem cell.
Clonal amplification of that cell occurs but cell maturation arrests at the primative, blast stage of differentiation
As the neoplastic population increases...normal hematopoiesis decreases to inevitable and life threatening cytopenias
patients have problems with INFECTIONS and BLEEDING |
|
|
Term
| What are the PBS findings characteristic of AML? |
|
Definition
Normocytic/normochromic anemia
nRBCs on PBS
AUER RODS!!!!!!!!!!!!!!!!!!
WBC is variable...sometimes low, sometimes normal, sometimes high |
|
|
Term
| What is the DIAGNOSTIC test for AML? |
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Definition
|
|
Term
| The absence of auer rods means.. |
|
Definition
| The leukemia may be either lymphoid OR myeloid |
|
|
Term
|
Definition
| CD34, CD33, CD13, CD14, CD15 |
|
|
Term
|
Definition
| Acute Myeloblastic Leukemia without Maturation |
|
|
Term
|
Definition
| Acute Myeloblastic Leukemia WITH maturation |
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|
Term
|
Definition
| Acute Promyelocytic Leukemia |
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|
Term
| What are the 3 common chromosome aberrations found in AML-M1? |
|
Definition
| trisomy 8, t(9;22), t(6;9) |
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|
Term
| What do we see in the PBS of someone with AML-M1? |
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Definition
|
|
Term
| What do we see in the PBS of someone with AML-M2? |
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Definition
| Promyelocytes and occasionally more mature cells of the neutrophilic line, in addition to blasts, are present, plus other leukocytes |
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|
Term
| What is the chromosome abnormality seen in AML-M2? |
|
Definition
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|
Term
| What do we see on the PBS of someone with AML-M3? |
|
Definition
Abnormal promyelocytes with a folded nucleus.."butterfly promyelocytes"
Also see bundles of auer rods..."faggot bundles" |
|
|
Term
| What translocation is specific for AML-M3? What does this translocation involve? |
|
Definition
t(15;17) is diagnostic for AML-M3
involves teh RAR-alpha and PML genes and results in an abnormal retinoid receptor |
|
|
Term
| Why do patients with AML-M3 have coagulation problems? |
|
Definition
| Inside the promyelocytes is a tissue-factor-like substance.. when released it causes coagulation problems |
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|
Term
| What is a treatment for patients with AML-M3? |
|
Definition
| To give these patients TONS of retinoic acid, which forces the cells into maturation |
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|
Term
|
Definition
|
|
Term
| What is seen on the PBS of a patient with AML-M4? |
|
Definition
myeloblasts, promyelocytes, monoblasts, and monocytes are present
Auer rods may be present |
|
|
Term
| What is the diagnostic BONE MARROW criteria for AML-M4? |
|
Definition
| Monocytes make up >20% of the non-erythroid cells of the bone marrow but NOT more than 80% |
|
|
Term
| What is the PBS monocyte count in AML-M4? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| What is the diagnostic criteria for AML-M5? |
|
Definition
| monocytic cells exceed 80% of the bone marrow cells |
|
|
Term
| What are the chromosome abberations seen in AML-M5? |
|
Definition
| Chromosome translocations or deletions involveing the long arm of chromosome 11 |
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|
Term
| What is the difference between AML-M5a and AML-M5b? |
|
Definition
| AML-M5a is an undifferentiated form, and AML-M5b is a differentiated form |
|
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Term
|
Definition
| Erythroleukemia (Di Guglielmo's Syndrome) |
|
|
Term
| What is the diagnostic Bone marrow finding in AML-M6? |
|
Definition
| Abnormal nucleated erythroid cells exceed 50% of the bone marrow cell |
|
|
Term
| What word can you use to describe the abnormal erythroid cells in AML-M6? |
|
Definition
|
|
Term
| What are some of the unusual RBC morphologies seen in AML-M6? |
|
Definition
Giant cell forms
Multiple lobes
Variations in the size of the nuclear lobes
Vacuoles in the cytoplasm
megaloblastic changes
Howell-Jolly bodies
IN BM: ringed sideroblasts |
|
|
Term
| Descrobe how the prominent cells in erythroleukemia, ALL, and AML stain with the PAS stain |
|
Definition
Erythroleukemia: intense granular staining in abnormal RBC precursors
ALL: PAS variable. Positivity shows bright fuchsia block-like granules
AML: Usually negative, occasional faint-diffuse stain is observed |
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|
Term
|
Definition
| Megakaryoblastic Leukemia |
|
|
Term
|
Definition
| Cytoplasmic extensions of megakeryoblastic blasts may resemble platelet formation |
|
|
Term
| What immunologic markers do the blasts of AML-M7 reacti with? |
|
Definition
|
|
Term
|
Definition
| Minimally differentiated Myelocytic leukemia |
|
|
Term
| What is the normal LAP range, and what range describes CML and reactive neutrophilia? |
|
Definition
Normal LAP range: 13-130
CML: LAP <13
Reactive neutrophilia: LAP >160 |
|
|
Term
|
Definition
| Philadelphia Chromosome--CML |
|
|
Term
|
Definition
| Burkitt's Lymphoma--ALL L3 |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| retinoic acid receptor alpha AML-M3 |
|
|
Term
| t(14;18) what does it create? |
|
Definition
| Follicular lymphoma--BCL-2 and IGH |
|
|
Term
| t(11;14) what are these genes |
|
Definition
| mantle cell lymphoma IGH and cyclin D1 |
|
|
Term
|
Definition
| diffuse large B-Cell lymphoma..this translocation makes the cells resistent to H. Pylori therapy |
|
|
Term
| What is myeloid metaplasia? |
|
Definition
Myeloid metaplaisa refers to the extramedullary hematopoiesis of the myeloid cell line
This often occurs secondary to |
|
|
Term
| What are the two stages of CIMF/PMF? |
|
Definition
| Prefibrotic stage and Fibrotic stage |
|
|
Term
| Where do we see the JAK-2 mutation? |
|
Definition
| The JAK-2 mutation is found in almost all cases of PV and in approximately 50% of patients with CIMF (and ET) |
|
|
Term
| Describe how the JAK-2 mutation relates to the pathogenesis of disease |
|
Definition
| The mutation causes JAK2 enzyme to be constituatively active causing cells to be permenently active, and proliferate without EPO signal (EPO no longer acts as regulation mechanism) |
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|
Term
| Comment on how each cell line looks on a PBS of someone with CML |
|
Definition
Mature granulocytes: predominantly monocytic cellular pattern, DYSPLASTIC cells: hyposeg neutrophils, small and wierd looking monocytes
Plateplets: thrombocytopenia...large platelets and micromegakaryocytes
RBCs: poik and iunclusions |
|
|
Term
| What is the component of the Non-specific esterase stain? |
|
Definition
|
|
Term
| What is the component of the specific esterase stain? |
|
Definition
|
|
Term
| What lab findings would help differentiate CMML from CML? |
|
Definition
1.) PBS/BM: For CMML a noticeable monocytosis and tri-lineage dysplasia will be noticed but absent in CML (no dysplasia)
2.) Philadelphia Chromosome: Absent in CMMl and present in CML
3.) Lysozyme: elevated in both serum and urine levels in CMML but not in CML |
|
|
Term
| What test helps differentiate AML M0 from AML M1? |
|
Definition
| The blasts in AML-M0 display negative reactions with all cytochemical stains. On the other hand, the myeloperoxidase or Sudan Black B is positive in more than 3% of the blasts of patients with AML-M1 |
|
|
Term
| How are AML-M5a and AML-M5b differentiated? |
|
Definition
| In M5a, monoblasts account for 80% or more of all monocytic cells in the bone marrow, while in M5b, monoblasts account for <80% of the monocytic component. Such classification is important as it provides information on the aggresiveness of the disease and the response to therapeutic regimens |
|
|
Term
| Describe the appearance of the bone marrow in patients with AML M6 |
|
Definition
| More than 50% of the bone marrow cells in a patient with AML M6 are erythroid and 30% or more of the remaining cells are blasts. Additionally, the normoblasts found in the bone marrow may be giant and multinuclear or multilobular. The normoblasts may exhibit nuclear budding and fragmentation, cytoplasmic vacuoles, Howell-Jolley bodies, ringed sideroblasts, and megaloblastic changes. Additionally, erythrophagocytosis, auer rods within the myeloblasts, hypogranular neutrophils and pseudo-Pelger-Huet anomaly in neutrophils |
|
|
Term
| Explain why patients with AML M3 might have abnormal coagulation test |
|
Definition
| AML-M3 is characterized by a predominance of promyelocytes. The granules of promyelocytes contain a procoagulant which is readily introduced into the bloodstream of patients with AML-M3 when their promyelocytes release their granules or are lysed |
|
|
Term
| What purpose does the retinoic acid receptor serve? |
|
Definition
| Helps in cellular differentiation and maturation |
|
|
Term
| Compare G:E ratio in CML vs. in AML |
|
Definition
|
|
Term
| Describe blast crisis in CML |
|
Definition
Patients in this final phase of CML have more than 30% blasts in their PBS and BM samples. The blasts frequently invade other tissues and organs outside the bone marrow. During this phase the disease transforms into an aggressive, acute leukemia (70% AML, 30% ALL)) If untreated, CML is fatal in roughly 20% of all patients each year
In BM: FIBROSIS!!! |
|
|
Term
| What two cytochemical stains are usually positive in ALL but negative in AML |
|
Definition
|
|
Term
| How do we calculate the G:E ratio? |
|
Definition
Granulocytes:
Segs, bands, metas, myelos, promyelocytes, blasts, eos and basos
NOT Lymphs, NOT Monos
Erythrocytes: ALL erythroid precursors |
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