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159 Cards in this Set

  • Front
  • Back
what is a first generation stem cell able to do?
ability to differentiate into ANY type of blood cell
what can second gen stem cells do?
differentiate into myeloid or lymphoid precursor only
in the first 8 weeks of gestation, the blood cells are maintained in the __ __
yolk sac
in the mesoblastic period, the inner region is __ __ cells
primitive blood
in the mesoblastic period, the outer region of the yolk sac will become the __ __
blood vessels
when do blood cells begin to migrate to other body tissue?
by 6th week of gestation in the mesoblastic period
what is the second phase of early cell development called>
hepatic period
when does the hepatic period of cell development occur?
week 8 to 7 months
From weeks 8-7months, where are blood cells formed?
in the liver
during the hepatic phase, the spleen will begin to generate ___ and at this time there is rampant destruction and ___ ___ will occur
erythrocytes; erythroblastosis fetalis
what occurs from the 2nd month ot the 2nd trimester in terms of blood production?
activation of monocytes and lymphocytes by spleen; this will only every again occur in hemolytic crisis
when does the thymus, lymph nodes and bone marrow become seeded with stem cells?
by the 4th month
___ in the thymus ceases in early childhood, but continues in ___ tissue for life
leukopoiesis; lymphatic
what is the final period of blood cell production?
3rd trimester (7months on): blood produced in the bone marrow
what is the 3rd period of blood cell production called?
marrow or myeloid period
in early childhood the RBCs are made in the long shafts of the __ and __
tibia and femur; ribs; and sternum/vertebrae
where are RBCs made in adulthood?
bones of the axial skeleton. Pelvis
what are the characteristics of RBCs?
anucleate; highly flexible; biconcave discs; 80-100 femotoliters in volume
what is the major role of RBCs?
carry O2 to and CO2 away from cells
erythropoietin is a ___ produced by the __-
cytokine; kidney
what is erythropoeitin needed for?
necessary for erythroid proliferation and differentiation
What occurs in the absence of erythropoeitin?
apoptosis of erythroid committed cells
ToF: kidney failure can cause anemia?
true! No erythropoeitin production
what is the name for the total volume count of platelets seen on labwork?
MPV
which is larger, platelets or their precursor?
precusor: megakaryocytes
how long do RBCs live?
120 days
RBCs are recycled by the __ and __
liver and spleen
during RBC destruction, __ and ___ are stored and ___ is released.
iron and protein; bilirubin
what do RBCs lack?
nucleus, mitochondria, and cytoplasmic RNA
what triggers the kidneys to produce erythropoeitin?
decrease in blood O2 causes decreased O2 tension in tissues signalling to the kidneys
ToF: people who live at higher altiutdes have more O2 available and thus less RBCs
false opposite
what RBC count would warrant a bleeding?
>60
the first microscopically recognizable RBC precursor is the ____
pronormoblast
what is a pronormoblast?
RBC precursor; big cell filled with nuclei; has deep blue cytoplasm;
how long does it take for a pronormoblast to lose nuclei and cytoplasmic RNA?
7 days
HgB will fill ___ the diameter of the pronormoblast.
30%
what is a normal reticulocyte count?
0.5-1.5%
what is a measurable precursor of RBC development?
reticulocyte
ToF: reticulocytes should be microscopically visible
TRUE
With normal Hgb A; reticulocytes should live in circulation for ___ __
1 day
the retic count can increase as high as ___ % after 1-2 weeks of iron therapy?
20%
what is the equation for the reticulocyte index?
[retic count x (Hct/Normal Hct)]
why must a retic calculation be done?
to correct for anemia to get a true retic count
the reticulocyte count is presented as a __
percentage
why is the reticulocyte production index needed?
in anemia, the patient's RBCs are depleted, creating an erroneously elevated retic count
An RPI > 3 indicates
increased production of retics suggesting either hemolysis or blood loss
an RPI < 2 means
reduced or ineffective production of retics in the bone marrow for the degree of anemia
what do target cells have?
increased surface to volume ratio
where are target cells seen most often?
severe IDA; thalassemia; liver disease
what are spherocytes?
hyper dense with decreased surface to volume ration
where are spherocytes seen?
hereditarty spherocytosis; ABO incompatability; autoimmune hemolytic anemia
what is another name for acanthocytes?
spur cells
what are acanthocytes?
cells with 5-10 spicules of varying length and apear smaller than normal cells because of spheroid shape
where are acanthocytes seen?
liver disease; malabsorbtive disease; hypothyroidism
what are echinocytes?
Burr cells: 10-30 spicules of equal size distrubted evenly over the cell
when are burr cells seen?
artifact; dehydration; after RBC transfusion
what are schistocytes?
helmet, triangular shapes or small fragments caused by contact with abnormal vascular surfaces
when are schistocytes seen?
severe hemolytic anema (G6PD); HUS; DIC; connective tissue disorders; prosthetic valves (ie Blender syndrome)
what are eliptocytes?
elliptical; normochromic cells which normally comprise less than 1% of RBCs
when are elliptocytes seen?
SS disease/Thalassemia; severe IDA; severe bacterial disease; malaria
what does it mean if normoblasts or nucleated RBCs are seen in a peripheral blood smear?
the spleen is not working
when does splenic function go down leading to normoblasts in the peripheral smear?
in myeloproliferative blood disorders causing cellular overload
when are normobloasts common?
in the newborn period
what are the causes of normoblasts seen in the peripheral blood smear?
hypoxia; hemolytic anemia; anemia under treatment; megaloblastic anemia; ineffective erythropoeisis; collagen vascular disease; malignant neoplasms; chemotherapy; caucher's and other storage diseases
ToF: nucleated RBCs may be prematurely released into the peripheral blood without reticulocytosis
TRUE
With NRBCs, dysplastic RBC changes may occur, as shown by the appearance of ___ and ___ cells in the peripheral blood
macroovalocytes and teardrop
what does a smear show with NRBCs?
spherocytes and reticulocytes
what is the treatment for NRBCs?
splenectomy if Hgb is greater than 10 and retics are less than 10; folic acid daily due to increased demand for erythrocytes
ToF: the osmolotic fragility test is specific for HS?
false; it is normal in 10-20 percent of patients with disease
what are the characteristics of teardrop cells?
usually microcytic and hypochromic
when are teardrop cells seen?
newborns; thalassemia; myloproliferative disorders
what are stomacytes?
rare: slit like central pallor and considered normal in small numbers
when are stomacytes seen?
artifact; actue alcoholism; malignancies
when are NRBCs seen?
hypoxia; acute bleeding; severe hemolytic anemia; post splenectomy; congenital infections
what are blister cells?
area under membrane that is Hgb free. Looks like a blister
when are blister cells seen?
G6PD (during acute hemolysis); S disease; Pulmonary emboli
what are basophillic stippling?
coarse or fine punctate inclusion that represent areas of visible RNA
when are basophilic stippling seen?
Lead poisoning; IDA; hemolytic anemia
what are Howell Jolly bodies?
small, well definined, round densely stained inclusions with eccentric locations
when are howell jolly bodies seen?
post splenectomy or hyposplenic states; newborn period; megaloblastic anemia
___% of childhood cancers are ___
hematologic malignancies
what are the most common childhood cancer?
leukemia
what are the demographics of leukemia in children?
ALL has highest rate in 2-5 years old; males are higher than females; Hispanics > Whites > Blacks
what are the common leukemias of childhood?
ALL; ANLL; CML
what are the common CNS cancers of childhood?
Glioma; medulloblastoma
What are the solid tumors of childhood?
neuroblastoma; wilms tumor; rhabdomyosarcoma; osteosarcoma
which childhood cancer is caused by heredity
retinoblastoma
what aer the environmental causes of childhood cancers?
organic solvents; pesticides; HIV; parental smoking
what are the drugs thought to cause cancer?
diethylstibestrol; chemo; benzone
ToF: the japanese survivors of the atomic bomb have a low risk of cancer
false: high risk of leukemia
what are the genetic abnormalities that are associated with an increased risk for childhood cancer?
downs; NF1; li-fraumeni syndrome; basal cell nevus cancer syndrom
children with down syndrome have a ___ times incidence of leukemia greater than the population at large with _-% of children with Down having leukemia by at 5
15; 1
of the newborns with transient myeloproliferatice disease, 25% will develop ___ _____
acute megakaryoblastic leukemia
what children have an increased risk of ALL?
Downs; ataxia telangiectasia; Bloom syndrome; schwachman syndrome; fanconi anemia; klinefelter; and NF1
ToF: in ALL, children will present with massive HSM and abnormalities in LFTs
false: no changes in LFTs
what are important sites of infiltrations of ALL?
CNS and testes
what are the clinical presentations of ALL related to expansion of the bone marrow?
bone pain; limp; lytic lesions on xray or abnormalities on bone scan; pathologic fractures are uncommon
why might atypicals be present in the peripheral smear of ALL?
viruses, and therefore a biopsy is needed
coagulopathies result from a rare subtyep of ANLL: ___ __ ___
acute promyeloctyic leukemia
neutropenia of ALL can result in __, whereas bleeding results from ___
sepsis; thrombocytopenia
why can airway obstruction occur in ALL?
large mediatstinal lymphadenopathy (seen in T-cell ALL) adolesecent boys
___ damages vital organs in ANLL
hyperleukoctyosis
what is thought to be classically elevelated in ALL?
uric acid do to breakdown of leukemic cells
A rare B-cell subtype of ALL can present in __ __
renal failure
ToF; Hypercalcemia appears with ALL
true, but also can have hypocalcemia
what are the symptoms of CNS involvement with leukemia?
increased ICP; headache; vomiting; papilledema
what are the signs of testicular infiltration of leukemia?
painless, firm enlargement of one or both testes
what are the common presenting symptoms of leukemia?
fever; fatigue; cutaenous or mucosal bleeding; bone pain; pallor; anorexia; weight loss
what are the favorable factors of ALL?
dx between 1-9; WBC < 50,000; no chromosomal abnormalities; pre-B cell;
What do infants (<6months) who are dx with ALL often have?
MLL gene rearrangements, CD10 negative/FLT3 positive
__ __ ALL accounts for 80-85%
precursor B-cell
which groups often have T-cell ALL?
older males with higher WBC presenting
what are the other malignancies than leukemia that have bone marrow involvement?
neuroblastoma; retinoblastoma; rhabdomyosarcoma; non-hodgekins
what are the non-malignant conditions that are differentials for ALL?
JRA; mono; ITP; pertussis; parapertussis; aplastic anemia; acute infectious lymphocytosis
what are the characteristics of very high risk ALL?
t(9,22), hypodiploidy, MLL translocation with slow response; induction failure
ToF: AML is common in childhood
FALSE
what is the mortality rate of AML?
50% with standard risk
what is the treatment like for AML?
most toxicity of all childhood cancers with multiple chemotherapies
what is the ultimate treatment for AML?
HSCT
what are the congenital syndroms associated with increased risk of AML?
polycythemia vera; thrombocytopenia; idiopathic myelofibrosis and myelodysplastic syndrome
what is myelodysplastic syndrome?
hypercellularity with dysplastic changes in meyloid precursor cells
___ have a higher risk of NHL?
males
ToF: most cases of NHL in children are high grade
TRUE
what are the types of NHL?
mature B-cell; lymphoblastic; anaplastic
lymphoproliferative disease in NHL is associated with __
immunodeficiency
what is Stage I NHL?
single tumor or nodal area (excluding mediastinum, abdomen)
if a child has NHL with regional involvement, multiple primaries on one side of the diaphragm, they are stage__
II
what is stage III NHL?
tumors on both sides of the diaphragm, primary intrathoracis or extensive abndominal disease
what is Stage IV NHL?
bone marrow and or cns involvement
what is the cure rate of HL?
90-95%
what pathogen is associated with HL?
EBV
what are the classifcations of classic HL?
lymphocyte-rich; nodular sclerosis; mixed-cellularity; lymphocyte depleted
what is the most common pediatric solid tumor of the head and neck?
rhabdo
how do primary nasopharyngeal tumors of rhabdo present?
nasal congestion, mouth breathing and unilateral otitis
what is rhabdo?
malignant tumor of skeletal muscle origin
what are the most common sites of rhabdo?
head, neck, GU tract, and extremities
what are the syndromes associated with Rhabdo?
Li Fraumeni (p53); costello (HRAS mutation); beckwith-wiedemann; NF1; Noonan
what age group has the best prognosis with rhabdo?
1/9/2013
what are the favorable sites for rhabdo?
orbit; nonparameningeal head and neck; paratestis; vulva; vagina; uterus; bilary tract
what size tumors of rhabdo have best survival?
<5cm
in rhabdo, ___ histology is favorable and the ___ histology is unfavorable
embryonal; alveolar
what is the most common malignancy in the first month after birth?
neuroblastoma
where can neuroblastoma occur?
anywhere along the sympathetic chanin including cervical ganglia
what syndrome can occur with neuroblastoma?
horner's syndrome, sweat on 1/2 of face
what is secreted in neuroblastoma?
catecholamines (VMA, HVA)
what does the secretion of cathecholamines in Neuroblastoma cause?
diaphoresis and hypertension
what is a nonspecific marker of cellular turnover in Neuroblastoma?
LDH
what is part of the diagnosis of neuroblastoma?
LDH; serum phosphorus and CMP; chest xray; US for neck masses; CT with contrast
what is the survival of Wilms?
>90%
what are the malformations associated with Wilms?
beckwith-weidemann; Anirida (PAX6); Aniridia/GU anomalies/MR -del(11p13); Denys-Drash syndrome- germline WT1 and somatic CTNNB1 mutations
what is stage 1 Wilms?
limited to the kidney, GTR
what stage of wilms has regional extension, GTR?
II
what stage of wilms has residual tumor in abdomen?
III
what stage is Metastatic Wilms?
IV
what is stage V wilms?
bilateral
what are the prognostic factors for wilms?
anaplastic (focal) or (diffuse); nephroblastomatosis; loss of heterozygosity for 1p and 16q
what are the common brain tumors of the infratentorium?
astrocytoma; ependymoma; medulloblastoma; brain stem glioma; ATRT
what are the common brain tumors of the supratentorium?
astrocytoma; PNET, CPC, ependymoma; germinoma; ganglioglioma
what are the common brain tumors of the sellar/suprasellar region?
craniopharyngioma; astrocytoma; germinoma