myeloid, WBC III, Putthoff, lec Flashcards by Amanda Beach

myeloid neoplasms come from what

hematopoietic progenitor cells

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how do you Dx a myeloid neoplasm

bone marrow, at least 20% blasts

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3 categories of myeloid neoplasms

acute myeloid leukemia
myelodysplastic syndromes
myeloproliferative disorders

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what type of cancer is from immature blasts in bone marrow that suppress normal hematopoiesis

acute myeloid leukemia

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defective maturation of myeloid progenitors leading to ineffective hematopoiesis and cytopenia

myelodysplastic syndrome

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what is characteristic of myeloproliferative disorders

increase production in 1+ types of blood cells

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what is CD34 on

multipotent stem cell

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what is CD64 indicative of

mature myeloid cell

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what is CD33

immature myeloid cell

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what genes are commonly affected in AML

RUNX1 and CBFB genes that are needed for hemtopoiesis

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what are the WHO categories of AML

I AML with genetic changes
II AML with MDS-like features
III AML therapy related
IV AML not otherwise specified

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what are the AML not otherwise specificed classifications

M0 minimally differentiated
M1 AML w/o maturation
M2 AML w/ myelocytic maturation
M3 Acute promyelocytic leukemia
M4 AML w/ myelomonocytic maturation
M5 AML w/ monocytic maturation
M6 AML w/ erythroid maturation
M7 AML w/ megakaryocyte maturation

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what are the common genetic mutations of AML

t 8;21
t 15;17
t 11q23;V
inv (16)

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majority of AML is what stage and what is assoc genetic mutation

M2 AML with myelocytic maturation

t8;21

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which AML is associated with DIC? stage? age of occurrence?

Acute promyelocytic leukemia stage M3

age 35-40

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What AML is peroxidase + and non specific esterase +

AML with myelomonocytic maturation class M4

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nonspecific esterase + in AML means what

from monoblast

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if auer rods are seen in AML then can tell arised from what lineage

myeloblast

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which AML has platelet specific Ag to vWF or GpIIb/IIIa

the megakaryocyte M7

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clincal features o fAML

anemia, neutropenia, thrombocytopenia, fatigue, infecitonm bleeding, mucosal bleeding and opportunistic infections

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what is leukemia cutis and when is it seen

skin infiltration of tumor on cutaneous skin

seen in monocytic AML

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what should you do is suspect AML

karyotyping because 90% associ with abberations

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Myelodysplastic syndrome patients are at high risk in develping what

AML

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what are types of myelodysplastic syndromes

primary idiotpathic and secondary genotoxic

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what are Sx of idiopathic myelodysplastic syndrome

weakness, infections, hemorrhages

how do you Dx myelodysplastic syndromes

bloos mear | bone marrow and cytologic studies

what type of myelodysplastic syndrome is more aggressive and what is onset timing and survival

genotoxic 2-8 yrs post exposure 4-8 mo survival

age onset myelodysplastic syndrome

around 70

what will histo look like in myelodysplastic syndromes

multilobated nuclei of RBC progenitors ringed sideroblasts, iron laden macrophages pseudo pelger huet cells (neutrophils with 2 lobes) megakaryocytes with multiple nuclei

what will you see in peripheral blood in myelodysplastic syndromes

pancytopenia

what is Tx for young patient with myelodysplastic syndrome? older?

young: hematopoietic stem cell transplant older: suppportive, thalidomide like drugs for 5q variants and DNa methylation inhibitors

what are mutations associated with myelodysplastic syndromes

monosomies 5 and 7 deletions: 5q 7q 20q trisomy 8-- MYC

what mutations occur in myeloproliferative disorders

mutated constitutively activates tyrosine kinase

What is mutation in CML

chimeric BCR-ABL, 9;22

how to Dx CML

fluorescence in situ hybridization or PCR based | leukocytosis >100,000, low LAP

CML preferentially drives what cell lines

granulocytic, megakaryocytic progenitors

what is distribution of CML

50 y.o M>F

clnical features CML

insidious onset: fatigue, weakness, weight loos, anorexia, abdmonial fullness or pain massive splenomegaly

what shoul dyou suspect in CML patient with severe LUQ pain

splenic infarct

histo of CML looks how

hypercellularity | Macrophages are wrinkled with blue/green cytoplasm "sea blue" histiocytes

what is prognosis CML

3 yr w/o Tx

What occurs within first year of CML

blast crisis and accelerated phase

what is Tx CML

BCR-ABL inhibitors | young- stem cell transplant 75% curable

If LAP measured and is high what do you suspect

reactive (infection

how come myeloproliferative disorders lead to cytopenias

the bone marrow is exhausted and gives up

What occurs in Polycythemia vera

JAK2 point mutation that is activated dec need for erythropoeitin increase RBC, granulocytes and platelets

what is the point mutation in PCV

JAK2 valine- phenlyalanine 600

what age group affected PCV and Sx

late middle age | insidious: plethora, cyanosis, intense pruritis, HA, HTN, GI ulceration, splenomegaly, hyperuricemia

What will CBC show for PCV

increase Hct increase RBC increase platelets | basophilia

what accounts for pruritis in PCV

basophilia

what is Tx for PCV

phlebotomy inc surivval 10 yrs

what is first sign of PCV in many patients

DVt or MI or stroke

what is spent stage of PCV

marrow fibrosis and splenomegaly because bone marrow exhausted

What is Essential thrombocytosis

thrombocytosis without PCV

mutaiton in ET

JAK2 or MPL increasing platelet count and megakaryocytes

how Dx ET

bone marrow biopsy and Dx of exclusion

what must you rule out for ET

reactive procress (inflammation or infeciton)

what are Sx and age onset ET

adults>60 insidious erthromelalgia

what is erythromelalgia

throbbing and burning of hands and feet from occlusion of small arterioles by platelet aggregates

What is Tx for ET

chemo | survival 12-15 yr

What markers are on Langerhans cell histiocytosis

S100+ CD1a+ HLA-DR+

histo of Langerhans cell histiocytosis

vesicular nuclei with linear grooves or folds & vacuolated cytoplasm birbeck granules in cytoplasm (langerin in granules)

what is mutation in Langerhans cell histiocytosis

valine to glutamate at 600 BRAF | also have TP53 RAS and MET

what are the categories of Langerhans cell histiocytosis

multifocal/multisystemic Langerhans cell histiocytosis unifocal and multifocal unisystem Langerhans cell histiocytosis pulmonary Langerhans cell histiocytosis

what are Sx of mutlifocal multisystemic Langerhans cell histiocytosis

cutaneous lesions like seborrheic eruption | HSM, lymphadenopathy, pulm lesions, bones lesions, anemia, throbocytopenia and recurrent infecitons

age Dx multifocal multisystem Langerhans cell histiocytosis

before 2 y.o | sometimes adult

what is Tx for multifocal mulisystemic Langerhans cell histiocytosis

50% survive 5 yrs

what do you see in blood fo unifocal and multifocal unisystem Langerhans cell histiocytosis

langerhans + eos+ plasma cells + lymphocytes + neutrophils

how does unifocal Langerhans cell histiocytosis present

skel system in older children like ribs, skull and femur

how does multifocal Langerhans cell histiocytosis present

multiple erosive bony masses in young children, 50% have DI because involvement of posterior pituitary stlak

What is Hans Schuller christian triad and assoc with?

calvarial defects + DI + exopthalmus | assoc with multifocal unisystem Langerhans cell histiocytosis

Tx for unisystem Langerhans cell histiocytosis

unifocal is resection or irradiation | multifocal is chemo

what is pulmonary Langerhans cell histiocytosis

b/l interstitial lung disease in middle and upper zones | smoking

What are normal markers on langerhans cells

CCR6

neoplastic markers on Langerhans cells

CCR6 AND CCR7!