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41 Cards in this Set

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1) What is this


2) Morphology


3) Gene mutation? Causes?


4) Clinical?


5) What is accelerated phase?

1) Chediak Higashi Syndrome


2) large blue to pink granules (about 10 per cell), made of primary and secondary granules


3) Auto recessive CHS1 (LYST) gene mutation causes defect in vesical (lysosome) formation/fusion/trafficking, leading to decreased phagocytosis


4) Defects in cells with granules


- WBC (bacterial and viral infections d/t PMN and LGLs)


- Melanosomes (albinism)


-Neurons (Neuropathy)


Platelets (bleeding)


5) triggered by EBV causing hemophagocytic syndrome (months to years after birth in 85% of patients)

1) What is this?


2) Morphology


3) Gene mutation


4) Inclusion made of


5) Compare with regular dohle body

1) May Hegglin Anomaly


2) Dohle body (more irregular located, not peripheral) and giant platelets


3) Auto dominant MYH9 mutation


4) RNA (d/t prob in endoplasmic reticulum)

1) What is this?


2) Morphology


3) D/t?


4) Inclusions made of?


5) Problems?


6) Compare with toxic granulation

1) Alder Reilly anomaly


2) Increased density of PAS+ basophilic (Azurophilic) granules in cytoplasm


3) lack lysozomal enzymes to break down mucopolysaccharides in Tay-sachs/hunters/hurlers


4) Mucopolysaccharides


5) No problem, neutrophils work fine!

1) What is this?


2) Morphology?


3) d/t?


4) Clinical problems?


5) Compare with MDS

1) Pelger-Huet Anomaly


2) HYPO-lobated nuclei


3) Auto dominant lamin B receptor mutation causing nuclei to fail to separate making "peanuts" and pince-nez eyeglasses


4) No clinical significance (functionally normal)


5) In Pelger-huet, all cells will look like this. In MDS (pseudo-pelgerization), only few WBCs look like this.

1) What is this?


2) Morphology?


3) D/t?


4) See when else?

1) Gaucher cell


2) Tissue paper cytoplasm with wrinkles and striations


3) Auto recessive Gaucher's disease in ashkenazi jews who have glucocerebrosidase deficiency causing accumulation of glucocerebrosidase


4) CML (pseudo-gaucher cell)

1) what is this?


2) Morphology (histology, polarized light, UV)


3) D/t?


1) Niemann Pick Cell


2) foamy vaculated cytoplasm that is birefringent on polarized light and yellow-green on UV


3) Niemann pick disease with sphingomyelinase deficiency causing accumulation of sphingomyelin

Chronic Granulomatous Gisease



1) D/t?


2) Presents?


3) Diagnose

1) X-linked or Auto recessive NADPH oxidase deficiency


2) Recurrent catalase positive bacterial and fungal infections (staph aureus, salmonella, serratia, aspergillus, nocardia)


3) nitoblue tetrazolium (NBT) reductase negative


(cant reduce NBT to insoluble purple formazan granules)

Myeloperoxidase Deficiency



1) D/t?


2) Presents?


3) Diagnose

1) Auto recessive cant catalyze H2O2 to HClO-


2) Usually no problems, unless diabetic, then get candida infections


3) Cytochemistry for MPO or Flow for MPO

1) What is it?


2) Morphology


3) d/t?

1) Activated atypical lymphocyte (Downey Cell)


2) low N:C ratio with scalloped indented edge adjacent to RBC with cytoplasmic basophilia


3) EBV/CMV/HIV/toxoplasmosis causing reactive CD8 cytotoxic T-cells

What is it and Cytopenia or Cytosis?



1) MDS


2) MYeloproliferative disorders


3) Acute leukemia

1) Ineffective hematopoiesis causing CYTOPENIA



2) prolif of mature cells causing CYTOSIS



3) prolif of immature cells causing CYTOSIS and CYTOPENIA

Markers



1) Immature (2)


2) Myeloid (3)


3) Monocytic (5)


4) Erythroid (2)


5) Megakaryocyte (2)


6) B-cell (2)


7) T-cell (3)

1) CD34, tdt


2) CD13, CD33, CD117


3) CD4, CD14, Cd11b, CD64, CD68


4) Glycophorin, Hemoglobin A


5) CD61, CD41 (gp2b/3a)


6) CD19, CD20


7) CD2, CD3, CD7

Lymphoblast vs myeloblast

Lymphoblast is smaller, less cytoplasm, let prom nucleoli and NO GRANULES



Myeloblast is larger, mroe cytoplasm, more prob nucleoli and GRANULES

1) Lymphoblast stain pos for (1)



2) Myeloblast stain pos for (3)



3) Monoblast stain pos for (1) (myelocytes become what color, monocytes become what color?_

1) PAS positive (chunks of blocks of positivity) (stains glycogen)


2) PAS positive (weak, but diffuse positivity)


MPO +


Sudan black B + (stains lipid in granule membrane) (less specific than MPO)



3) Nonspecific esterase (alpha naphthyl acetate/butyrate) (myeolid cells become red, and monocytic cells become blue)(acetate also stains megakaryocytes)

Classify AML based on WHO 2008 (4)

- AML w/ recurrent genetic abnormalities


- AML w/ MDS related changes


- Therapy related Myeloid Neoplasm


- AML, NOS (M1, M2, M3...)

1) Diagnose AML (definition) (3)



2) Recurrent genetic abnormalities in AML (3)

1) >20% blasts in marrow OR have any recurrent genetic abnormality (3) OR acute erythroblastic leukemia



2) t(15;17)(q22,q12)


t(8;21)(q22,q22)


inv(16)(p13,q12)

1) If normal karyotype in AML, look for what mutations? (3)


2) Good prognosis with which mutations? (2)


3) Bad prognosis with which mutations? (1)



4)Chromosomal alterations with good prognosis in AML (3)


5) Chromosomal alterations with bad prognosis in AML (4)

1) FLT3, NPM1, CEBPA



2) NPM1 mutation AND FLT3 wild type (normal)


Or


CEBPA mutation



3) NPM1 mutation AND FLT3 mutation



4) t(15;17), t(8;21), inv(16)


5) del5q, del7q, t(9;22), or complicated cytogenetics

1) What is this?


2) Definition?


3) Can cause?


4) Treat


5) Genetics? creates what?

1) AML -M3


2) >20% blasts and promyelocytes


3) DIC


4) ATRA


5) t(15;17). PML/RARalpha fusion protein

1) What is AML M4?


2) Define


3) Clinical

1) Acute myelomonocytic leukemia



2) >20% blasts, >20% PMN, >20% monocytes



3) Gingival hyperplasia

1) What is AML M5?


2) Define. Define M5a and M5b


3) Monoblast morphology

1) Acute monoblastic and monocytic leukemia



2) >80% are monocytic cells



M5a = >80% of monocytic cells are monoblasts



M5b = <80% of monocytic cells are monoblasts



3) Cleaved (not rounded) nuclei and cytoplasmic vacuoli

1) What is AML M6?


2) Define M6a


3) Define M6b


4) eg: if >80% erythroid cells, and 10% myeloblasts, is it M6a?

1)Erythroleukemia (M6a), Pure Erythroid leukemia (M6b)



2) >50% of all nucleated cells are erythroid AND >20% of nonerythroid elements are myeloblasts



3) >80% of all nucleated cells are immature erythroids



4) Yes, becuase only have 20% nonerythroid cells, so 10/20 = 50% of nonerythroid cells are myeloblasts

1) What is AML M7


2) Define


3) Dont call M7 if?

1) Acute megakaryoblastic leukemia


2) >20% blasts and >50% blasts are of megakaryocytic lineage (CD61+, CD41 +)


3) Patient has Down's syndrome

1) Define AML M0


2) Define AML M1


3) Define AML M2

1) AML w/ minimal differentiation (>20% blasts, w/ <3% blasts SBB or MPO +)



2) AML w/out maturation (>20% blasts w/ >3% blasts SBB or MPO+)



3) AML with maturation (>20% blasts w/ >3% blasts SBB or MPO + AND >10% maturation

Therapy related AML



1) D/t what (2)


2) Which one assoc w/ MDS and poor prognosis?


3) Which one takes longer to develop after treatment?

1) Alkylating agents (busulfan, cyclophosphamide) and Topisomerase 2 inhibitors



2) Alkylating agents



3) Alkylating agents

1) What is transient myeloproliferative disordeR? At risk for anything later?



2) Solid growth of myeloblasts called?



3) Chromosomal alter

1)in first week of life in 10% of Down's children get increased WBC count with increased blasts. At risk for AML down the line!!!



2) Chloroma or Granulocytic Sarcoma or Nonleukemic Myeloid Sarcoma

1) B-ALL WHO 2008 classification for B ALL(2)



2) Survival better in kids or adults for B-ALL?

1) B lymphoblastic leukemia/lymphoma, NOS


or B lymphoblastic leukemia/lymphoma, w/ recurrent genetic translocations



2) kids, 80% cure

1) Good prognosis in B-ALL?


2) Bad prognosis in B-ALL

1) t(12;21) or ETV6-RUNX6


- Hyperdiploid (>50 chromosomes)


- Age 1-10



2) Hypodiploid


-t(9;22)


-11q23


-RUNX1 amplification

Translocation most likely to have in ALL patient


1) Infant


2) Child


3) Adult

1)t(4;11)


2) t(12;21)


3 t(9;22)

1) Diff acute leukemia from myeloproliferative neoplasms



What goes out of whack?


2) CML


3) PV


4)ET


5)PMF

1) Acute leukemia has >20% blasts, and myeloprolif has <20% blasts



2) Myeloid cells


3) Erythroid cells


4) Platelets


5) Megakaryocytes

Genetic abnormalities


1) CML


2)PV


3)ET


4)PMF


5)Myeloid neoplasms with eosinophilia


6) Mastocytosis

1) ABL1


2) JAK2V617F (95%), JAK2 exon 12


3) JAK2V617F (50%), MPLW151 (<5%)


4) JAK2V617F (50%), MPLW151 (10%)


5) PDGFRA, PDGFRB, FGFR1


6) KIT D816V

1) JAK2 located on what chromosome?


2) JAK2 does what?


3) What happens to JAK2 in myeloproliferative disorders?


4) Is finding the mutation prognostic?


5) What other diseases do you see it in?

1) 9p


2) mediated intracellular signaling for growth factors like erythropoietin, thrombopoietin, GCSF


3) single point mutation causing Activation!! (valine=V replaced by phenylalanine=F) (so downstream receptors active INDEPENDENT of growth factors)


4) No!


%) MDS and AML (may signify preceding myeloprolif disorder)

1) Most common myeloproliferative neoplasm?



2)MYeloproliferative neoplasm with worst prognosis?

1) Essential thrombocythemia



2) Primary Myelofibrosis

Essential Thrombocythemia



1) Define


2) Presents


3) PB Morphology


4) BM Morphology


5) Prognosis

1) Sustained platelets >450 x 10^9 (mostly megakaryocytes) and no teardrop cells



2) Hemorrhage and thrombosis, splenomegaly or hyposplenism



3) Platelets >450,000; no teardrops



4) Normocellular, large clusters hyperlobulated megakaryocytes, and NO FIBROSIS



5) 80% 5 year survival, 5% transform to AML

Polycythemia vera



1) Define


2) Need to do what?


3) PB morphology


4) BM morphology

1) Hb > 18.5 g/dL (>16.5 in women) AND presence of JAK-2 mutation



2) exclude everything else that inc HB (RCC, HCC, inc EPO)



3) erythrocytosis


4) Hypercellular with PANmyelosis

Primary myelofibrosis



1) PB morphology


2) BM morphology


3) Compare with ET (3)

1) leukoerythroblastic rxn (nucleated RBC and precursor myeloid) and teardrop cells



2) Fibrosis, osteosclerosis, dysplastic megakaryocytes



3) PMF has worse prognosis, dysplastic megakaryocytes, and leukoerythroblastic rxn

Chronic Myelogenous leukemia



1) Define


2) Presents and LAP score


3) PB morphology


4) BM morphology


5) 3 Stages


6) Which stage gives diff genetic abnormality (besides BCR)? Which ones (4)

1) must have BCR-ABL (+)


2) around 50 with splenomegaly and high WBC; LOW LAP Score (high in leukemoid rxn and other MPNs)



3) WBC at all stages and >50,000



4) Not necessary to make diagnosis (just need PB and BCR-ABL), but need to determine blast count to stage



5) Chronic (<10% blasts), Accelerated (<20% blasts), Blast phase (>20% blasts)



6) Blast phase


+PH, i(17q), +8, +19

1) Philly chromosome CML is what?



2) CML in kids is what? Prognosis

1) Not CML, is atypical CML (aCML) or Chronic Myelomonocytic leukemia (CMML)



2) Juvenile myelomonocytic leukemia (JMML). Poor prognosis

BCR-ABL



1) Seen in which diseases (3)



BCR-AB: Fusion protein created in :



2) B-ALL


3) CML


4) Chronic neutrophilic leukemia

1) CML, ALL, Chronic neutrophilic leukemia



2) p190 (190KD)


3) p210 (210KD)


4) p230 (230KD)

MDS



1) Define


2) Prognosis

1) PB pancytopenia despite hypercellular BM



2) Die from cytopenia, or become AML in 25%

MDS Blood blasts/BM blasts/features



1) RCUD (Refractory Cytopenia with unilineage dysplasia)



2) RARS (Refractory anemia with ringed sideroblasts)



3) RCMD (Refractory cytopenia with multilineage dysplasia)



5) MDS w/ Isodel5q

1) <1%/<5%/<15% ringed sideroblasts



2) <1%/<5%/>15% ringed sideroblasts



3)<1%/<5%/dysplasia in 2 or more cell lines



4)<1%/<5%

MDS Blood blasts/BM blasts/features



5) RAEB-1 (refractory anemia with excess blasts 1)



6) RAEB-2 (refractory anemia with excess blats 2)



7) Chronic myelomonocytic

5) 2-4%/ 5-9%



6) 5-19%/10-19%/if auer rods advances to RAEB-2



7) Monocytosis > 1x10^9/ 5-19%/ increased WBC from monocytosis



1) Good prognosis for MDS



2) Bad prognosis for MDS

1) -Y, del(5q), del(20q), TET2 mutation



2) Complex (>3 abnormalities), Chrom 7 problems