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79 Cards in this Set

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  • Back
what is neutropenia?
low number of neutrophils
what causes neutropenia
bone marrow suppression: tumors, granulomoatous infection of the marrow, infeefective hematopoiesis, genetics

destruction of neutrophils: immune disorders SLE, reheumatodi arthritis drug reaction, splenomegaly(sequesters blood cells causing pancytopenia

increased peripheral utilization: overwhelming infection
what is reactive leukocytosis?
high white count
what causes reactive leukocytosis
infections, tissue damage, allergic disorders, malignancy, autoimmune disorders, inflammatory bowel disease
leukemoid reaction
what is a a leukemoid reaction
a type of reactive leukocytosis in which there is a very high white count with circultatin immatrue white cells, simulateing a myeloid leukemia
how can you tell the difference btw a leukemoid reaction and a leukemia?
mostly matrue neutrophils in leukemoid reaction no blasts or nucleated RBCs
what causes a leukomoid reaction
perforrated abdominal viscera(peptic ulcer)
infarcted abdominal organs
what 3 things found on peripheral smear are indicative of leukemoid reaction?
metamyelocytes
dohle bodies
cytoplasmic vacuoles.
atypical lymphocites on CBC in a young adult with sore throat and swollen lymph nodes
infectious mononucleosis
where are B cells usually
in the lymphnodes
where are T cells usually
circulating in peripheral blood
what are the nature of atypica lymphocites in a person with mononucleosis
reactive t cells
what are the two types of leukemias?
myeloid-contains all but the white blood cells:granulocytes, megakaryocytes, and erythroid cells

lympoid: neoplastic proliferation of lymphocytes.
what does the white WBC count have to be in leukemia?
it can be anything high, normal, or even low.
what is the cause of neutrophilic leukocytosis
bacterial infection, or tissue necrosis
what is the cause of eosinophilic leukocytosis
allergy, drug reaction, parasitic infection, vascular disorder
what is the cause of basophilic leukocytosis
rare usually indicates a myloproliferative disorder
what is the cuase of a lymphocyctic luekocytosis
viral, fungal infections or bordetella pertussis (whooping cough)
what does the marker CD34 indicate
a hematopoetic stem cell
what does IL 5 stimulate a myloid progenitor to become?
eosinophil
what does thrombopoiten stimulate teh myloid progenitor to become?
basophil, megakaryocyte, erythroid.
what does GM CSF stimulate a myloid progenitor to become?
neutrophil or monocyte.
what is the clinical presentration of acute leukemia
short duration of symptoms
aggressive clinical course
what is the clincial presentation of chronic leukemia
insidious onset may be discovered by cbc

hepatosplenomegally
what are the characteristics of acute myeloid leukemia
mainly in adults
originates from stem cells
this means there are several subtypes based on which stem cell is proliferating
cytopenia can result from bonemarrow loss

examples promelocytic, myelocytice, monocytic
what are the clinical presentation of AML
ulceration of mucous membranes
fever
DIC
myeloid sarcomas:soft tissue masses of imature myeloid cells
anemia, thrombocytopenia
blasts in peripheral blood
what is gum hypertrophy a symptom of
AML monoblastic type
what can be found in peripheral blood smear for AML
myeloblasts
auer rods
nucleated RBCs
what does auer rods do?
confirms blasts as myeloid
what is the percent myeloblasts in the the bone marrow that indicates leukemia
greater than 20%
what is cytochemistry used for?
to separate AML and ALL

positive in AML negative in ALL
what does a myeloperoxidase(MPO) stain indicate
neutrophilic lineage
what does a sudan black B(SBB) stain indicate
neutrophilic liineage
what does a nonspecific esterase (NSE) stain indicate
monocytic lineage
what are the three main genetic findings found in AML with recurrent cytogentetic abnormality
t(8:21) good response to treatment
t(15;17)all trans retinoic acids works
inv(16) better remmision with cytarabine
what does myeloid sarcoma indicate
AML
what are some characteristics of acute promyelocytic leukemia
numerous Auer rods
best treated with all trans retinoic acid
strong association with DIC

associated with t(15;17) and (q22;q21)
what are the characteristics of Acute lymphoblastic leukemia
more common in children
more common in whites
pallor, weakness, anemia
petechiae, mucosal bleeding
bone pain
fever(50%)
lymphadenopathy or hepatosplenomegaly
anterior mediastinal mass
blasts in the periperal blood.
what is the diagnostic percentage of blasts in ALL
25%
what is an easy and accurate way to diagnose ALL
the use of TdT assay and panel of monoclonal antibodies for T and B cell antigens
what are the catagoies in ALL
T cell
B cell
what is the differecne btw leukemia and lymphoma
leukemia has malignacy circulating
lympha no blasts in peripheral blood
what are the characteristics of chronic lymphocycitc leukemia
most common leukemia
mainly in older people
20% asymptomatic
50% splenomegaly
lymphadenopathy
Elevated WBC
how is CLL staged
absence of anemia and thrombocytopenia is stage 1 or 2

presence of anemia only is stage 3

presence of both is stage 4
what is an implication of B cell neoplasms in CLL
anemai can be brought on by hemolysis caused by antibodies.

B cell neoplasms can make a variety of antibodies that could cause conditions that bring the patient in looking like an autoimmune issue.
what does CLL look like
cracked earth or soccer ball
hyperdense nuclear pattern
how is CLL diagnosed
lymphocytes express both CD5(tcell marker) and CD 20 and 23 (bcell marker) along with low level expression of surface IG(usually IgM)
when sequenced what does unmutated CLL mean for prognosis
unmuated CLL is more aggressive
what are the markers in CLL
ZAP-70:positive in unmuated CLL
CD 38: if greater than 30% poor prognosis
Del 17p or 11q22.3: poorer survival
del 13q: good prognosis
trisomy 12: responsive to anti-CD20 medications
what are the two most common transformation in CLL that make it more aggressive
prolymphcytic
Richter syndrome
what is the presentation of prolymphocitic transformation in CLL
gradual increase in circulating prolymphocytes seen in peripheral blood
what is the presentatino of richter syndrome
development of rapidly progressive lymphoma
what are the characteristics of hairy cell leukemia
rare
memory b cell neoplasm
middle aged male pancytopenia, enlarged spleen, dry tap on bone marrow aspiration

hairy cells seen on periperal blood smear.
what is the marker for hairy cell
pos CD19, CD20, 11c

red cell lakes
what are some key differences btw myelodysplasia and myeloproliferative
myelodysplasia:hypercellular bone marrow, periperal cytropenia, ineffective hematopoiesis, dysplasia, no splenomegaly

myeloproliferative: hyerpcellular bone marrow with peripheral hypercellularity., effective hematopoiesis, no dyspolasia, splenomegally may be massive
what are some characteristics of myelodysplasia
idiopthic or primary: age 50+ develps insidiously
therapy related MDS(secondary): complication of previous drug or radiation therapy, arising 2-8 years after treatment.

all can progress to AML
what is refractory anemia?
does not respond to iron suppliments
what is the diagnositc percentage of myeloblasts in the bonemarrow for MDS
less than 20% because greater than 20% would be AML
what is the worst MDS other than therapy related
Refactory anemia with excess blasts

RAEB type II
what abnormalites can be used to help diagnose MDS
clonal chromosomal abnormaliteis:
monosomy 5, monosomy 7, deletions 5q and 7q, trisomy 8 and deletions of 20q
what are the worst prognostic indicators in MDS
RAEB II
2-3 cytopenias or pancytopenia
cytogenetics with three or more abnormalities or a single abnormality on chromosome 7.
what are the various classifications that fall under myeloproliferative neoplasms
chronic myelogenous leukemia(CM), BCR-ABL1 positive

chronic neutrophilic leukemia
polycythemia vera
primary myelofibrosis
essential thrombocytosis
chronic eosinophilic leukemia
mastocytosis
what is the definition of MPN
clonal stem cell disorder

proliferation in the BM of nonlympohid lineages

all the following the in the peripheral blood:
myelocytes
metamyelocytes
bands
promyelocytes
nucleated reds


less than 20% masts

classified by periperal blood findings
what is characteristic of chronic myelgenous leukemia
bone marrow in the blood(excess cells in the periperal blood)
demonstration of philadelphia chromosome t(9:22), (q34;q11) if found can be treated with gleeveck

hepatosplenomegally
what is the philidelphia chromosome
translocation between chromosome 9 and 22 which creates BCR-ABL which is a fusion protien with tyrosine kinase acitvitiy which activates cell division and inhibits apoptosos
what is polycythemia vera
marked increase of red blood cells
which causes intense itching espeacially after bathing
high risk for bleeding or thrombosis
headache diziness, HTN, GI distress
red face red hands
can lead to splenomegaly
can lead to AML but less common
what is a normal amount of RBCs in the peripheral blood of a person with Polycemia vers
6 million
what is found on CBC of a person with polycemia vera
high WBC
high RBCs
high platelets
high hemoglobin and hematocrit
anemia with low MCV due to iron deficiency secondary to chronic GI blood loss
how is polycemia vera treated
phlebotomy
what is primary myelofibrosis
megakaryocyte proliferation
marrow is obliterated and fibrosed
how is primary myelofibrosis diagnosed
CBC-anemia with elevated WBC and platelet count
leukoerythroblastosis seen on peripheral blood
imature RBC and granulocytes
tear drop shaped RBCs
white bonemarrow
what is leukoerythroblastosis?
indicates space occupying lesion:fibrosis, metastaic carcinoma, TB granulomas,

aka myelophthisic anemia

most commin in primary myelofibrosis
what is essential thrombocytosis
neplastic proliferation of megakaryocyts and platelets

very high platelt counts 600,000

excess platelets leads to bleeding and thrombosis

erythromelalgia=throbbinga nd burning hands and feed due to small vessel occlusion by platelets.
how is essential thrombocytosis diagnosed
peripheral blood shows a lot of platelets and those platelets are abnormal
what is the most vital thing to know about diagnosis for CML
must rule our reactive leukocytosis first and verify philadelphia chromosome
what is the most vital thing to konw about diagnossis for Polycythemia vera
rule out secndonary polycythemia

can be caused by smoking, chronic tissue hyposia, high O2, affinity hemoglobinopahty, high erythropoiten level
what is needed to know about diagnosis of essential thrombocytosis?
must rule out reactive thrombocytosis
what is the jak2 study
lab test that detects myeloproliferative disorders
what myeloproliferative disorder is most likely to progress to AML if untreated
CML