Test 3 Flashcards
What is the most common age and gender for a myeloproliferative disorder to present?
70 yr old male
What is the prognosis for a person with MDS that transforms into a leukemia?
very poor
What can a MDS progress into?
acute leukemia
What blood problem can topoisomerase inhibitors create?
MDS
What are the three common presenting symptoms in a patient with 5q syndrome?
macrocytic anemia
leukopenia
low/normal platelets
How is 5q syndrome treated?
lenalidomide
What is CMML?
chronic myelomonocytic leukemia
What two drugs can be used to treat CMML?
azacytidine or imatinib
What two cell lines are almost always seen during CML?
basophils
eosinophils
What two labs are commonly high during CML?
LDH
uric acid
How can one tell that CML is transitioning into AML?
splenomegaly
What two drugs can be used in CML patients that have become resistant to imatinib?
dasatanib
nilotinib
What is the major side effect of nilotinib?
prolong QT
What is the most common mechanism of resistance to imatinib?
ABL mutations
What is the Hb diagnostic level for PV in men?
greater than 18.5
What is the Hb diagnostic level for PV in women?
greater than 16.5
Would serum EPO be high or low in PV?
low
What three drugs are used to treat PV?
hydroxyurea
interferon alpha
Anagrelide
What age and sex is most commonly presenting with Essential Thrombocytosis?
60 yr old, female
What two myeloproliferative diseases have very similar presenting symptoms?
essential thrombocytosis
polycythemia vera
What three drugs are used to treat Essential Thrombocytosis?
Hydroxyurea
Anagrelide
Interferon-alpha
What are the two common hallmarks of Primary Myelofibrosis?
marrow fibrosis
extramedullary hematopoiesis
What modalities are used to prolong life during primary myelofibrosis?
none
Does multiple myeloma effect men or women more often?
men
Does multiple myeloma effect AAs or caucasians more often?
AAs
What is the average age of mutliple myeloma Dx?
65
What are the two most common immunoglobulins to be produced during Multiple Myeloma? Which is more common?
IgA and IgG
IgG
What is the goal for stem cell transplants during Multiple Myeloma?
prolong life
What three drugs are used to treat multiple myeloma?
thalidomide
lenalidomide
carfilzomib
What is a plasmacytoma?
isolated malignant plasma cell tumor growing within axial skeleton or soft tissue
What is the most common site for a plasmacytoma?
upper respiratory tract
What is the mainstay of treatment for a plasmacytoma?
radiation
What is the stain for amyloidosis? What color does amyloidosis look like under fluorescent microscopy ?
congo red
red-green
What organ has the worst prognosis for the development of amyloidosis?
heart
Other than immunoglobulin, what is the main difference between mutliple myeloma and Waldenstroms Macroglobulinemia?
WM has greater than 10% lymphocytic involvement
What is the most common form of cancer in children under 14?
ALL
What is the most common form of cancer in children between 14 and 18?
Hodgkins Lymphoma
How hot and for how long does a fever indicative of Leukemia have to last for a child?
greater than 101F
longer than seven days
What are the two most common presentations of a childhood CNS tumor? Why?
vomiting and headaches
compression of CSF = increase ICP
How large must a lymph node from the epitrochlear region be to considered large in the epitrochlear region?
greater then 5 mm
How large must a lymph node from the inguinal region be to considered large?
greater than 15 mm
Adenopahy in what three regions is almost always considered abnormal?
epitrochlear, cervical, posterior auricular
Is ALL more common in AA children or white children?
caucasian
Is ALL more common in males or female children?
male
What is the peak age for ALL?
2-3 years old
In what child population is ALL most common?
hispanic
What are the two most common risk factors for the development of a child with ALL?
pre-natal Xrays
post-natal radiation
What two characteristics are the standard risk for ALL?
1-10 years old
WBCs below 50K
What two characteristics are the high risk for ALL?
below one or greater than ten
WBCs above 50K
By day 28, what level of lymphoblasts is wanted?
below 5%
What is the most common childhood brain tumor?
astrocytoma
What are astrocytomas associated with?
NF1
What is the etiology of LiFraumeni Syndrome?
p53 mutations
What are the four associated diseases that accompany Wilms Tumors?
Hemihypertrophy
Aniridia
Denys-Drash
Beckwith-Wiedemann
What type of tissue does a neuroblastoma develop from?
any neural crest tissue
How could a neuroblastoma in the cervical region develop?
Horner Sydrome
What protein is mutated in a neuroblastoma?
N-myc
What is the most common mesenchymal tissue in children?
Rhabdomyosarcoma
Are Ewing’s Sarcomas more common in male or females? What ages?
males
11-15
What are TH2 reactions?
antibody mediated
What are TH1 reactions?
cell mediated
What is the sex and age for AML presentation?
65 year old male
Is CML more common in males or females?
males
What is the age for the average onset of AML?
65 years old
Which form of myelegnous leukemia is known for infiltrating the skin?
AMML
Is organomegaly common in AML?
no
What two forms of AML have Auer rods?
M2 and M3
Which AML cellular subtype is known for invading the gingiva?
monocytic
What sex is more affected by mantle cell lymphoma? What age?
male
50-60
What GI organ does mantle cell lymphoma commonly effect?
small BOWEL
Does Mantle Cell lymphoma possess CD23?
no
What is the median age of deveopment for the sporadic form of Burkitts Lymphoma?
30
In the endemic form of Burkitts lymphoma, what are three common locations other than the jaw for cancer to develop?
ovaries, breast, kidneys
Does HTLV1 infect CD4 or CD8 cells?
CD4
What is the new antibody for HTLV1? What CD molecule does it target?
Alemtuzumab
CD52
What are two characteristics of the nucleus of cells infected with HTLV1?
folded
cerebriform
What is the difference between Sezary Syndrome and Mycoises Fungoides?
Sezary syndrome involves leukemia as well
What are two common presentation sites for T-cell leukemia?
Mediastinal mass
CNS issues
According to Fang, what are the three markers for T-cell leukemia?
CD34, TdT, CD1a
What is ALCL?
anaplastic large cell lymphoma
Which marker has a poor prognosis in ALCL? Good Prognosis?
good = ALK1 positive
bad = ALK1 negative
What is the shape of cells during ALCL?
kidney shaped
What CD molecule can ALCL stain for?
CD30
What can produce pain in lymph nodes with a B-cell lymphoma?
alcohol
Beta 2 microglobulin is normally apart of what molecule?
MHC one
In what two conditions can Beta 2 microglobulin be elevated?
multiple myoloma
lymphoma
According to Heddinger, what is stage one?
one nodal region
According to Heddinger, what is stage two?
two nodal regions on the same side of the diaphragm
According to Heddinger, what is stage three?
both sides of diaphragm
According to Heddinger, what is stage four?
extra-nodal site
What is the most common type of Hodgkins Lymphoma?
nodular sclerosing
What is the adverse prognostic number for albumin regarding Hodgkins Disease?
less than four
What is the adverse prognostic number for age regarding Hodgkins Disease?
greater than 45
What is the adverse prognostic number for hemoglobin regarding Hodgkins Disease?
less than 10.5
What is the adverse prognostic number for WBC regarding Hodgkins Disease?
greater than 15,000
What is the most common treatment for Hodgkins?
radiation
What is more aggressive, B-cell or T-cell?
T-cell
Which BCL molecule is present is Small lymphocytic leukemia?
BCL-3
Which BCL molecule is present in diffuse large b-cell lymphoma?
Bcl-6
Which BCL molecule is present during Mantle Cell Pymphoma?
bcl-1
What are the three prognostic factors for DLCL?
stage III/IV
over 60
elevated LDH
What is Richters Transformation?
Follicular lymphoma transforming into DLCL
What two viruses are known to cause lymphoma in HIV patients?
HHV8
EBV
Which leukemia present most like a lymphoma?
CLL
What are the two most common signs of CLL on physical exam?
lymphoma
splenomegaly
Which three immunoglobulins are known to be low during the late stages of CLL?
IgA/IgG/IgM
CLL will be positive for which three CD molecules?
CD5/CD20/CD23
Is CLL curable?
no
What neutrophil anamoly can present during MDS?
pseudo-pelger huet
What is Sweet’s Syndrome?
febrile neutrophilic dermatosis
What type of patient most often presents with a 5q syndrome?
elderly female
Other than RBCs, what two cell lines also increase in their numbers during PV?
WBCs and platelets
What amino acids are mutated during a JAK2 mutation?
V617F
What disease does a plasmacytoma often progress to?
multiple myeloma
What organ is most commonly effected by amyloidosis?
kidney
What organ gives the worst prognosis for amyloidosis?
heart
What percent of lymphocytes have to be monoclonal to Dx Waldenstroms?
greater than 10%
What type of bone pain is indicative of cancer in kids?
wakes them from sleep
What is the most common solid tumor of childhood?
brain tumor
What type of molecules do neuroblastomas secrete?
catecholamines
Do osteosarcomas effect long bones or all bones?
long bones
Does Ewing’s Sarcoma effect long bones or all bones?
all bones
When are antibiotics used during AML therapy?
ablating bone marrow
Which form of AML is most likely to produce DIC?
M3
One will almost always find a large spleen during what malignancy?
CML
How does a T-cell leukemia spread in the CNS?
via meninges
What is considered higher risk, B-ALL or T-ALL?
T-ALL
What two types of malignancies is EBV associated with?
Burkitts
Nasopharyngeal carcinoma
What two proteins undergo fibrosis in the bone marrow during MDS?
collagen and fibrillin
What drug can cause an MDS?
ethanol
What is the MOA of anagrelide?
inhibits platelet production
Does T-cell ALL effect adolescents or children more often?
adolescent
Does T-cell ALL effect males or females more often?
males
Is an astrocytoma a low-grade or high-grade tumor? What type of tumor?
low grade
glioma
What type of brain tumor does not respond to radiation?
choroid plexus tumors
Which ethnicity has a higher prevalence of Wilms Tumor?
AAs
Which ethnicity has a lower prevalence of Wilms Tumor?
Asians
What is the most common extra-cranial solid tumor in children?
neuroblastoma
Are Ewings sarcomas amenable to radiation?
no
Over the age of what is a poor prognosis for ALL?
35
Longer than how many weeks for remission of ALL is a poor prognostic factor?
4 weeks
Which lymphoma is known to involve the liver?
mantle cell
According to Fang, where is the most common site for a sporadic Burkitt lymphoma to arise?
ileo-cecal region
Where is the plaque phase of Sezary/Mycosis Fungoides located?
epidermis
Where is the tumor phase of Sezary/Mycosis Fungoides located?
dermis
Which leukemia or lymphoma displays a bimodal distribution of the ages it effects?
Hodgkins lymphoma
What is the typical lymphocyte count of CLL?
greater than 5K
What is seen during Stage 1 CLL?
lymphadenopathy
What is seen during Stage 2 CLL?
splenomegaly
What is seen during Stage 3 CLL?
Hb under 11
What is seen during Stage 4 CLL?
platelets under 100K
Which fungus can cause SVC syndrome?
Histoplasma capsulatum
A total neutrophil count of less than what raises suspicion for neutropenic fever?
500
What is the most common source of bacteria during neutropenic fever?
endogenous gut flora
What type of organism is rarely the primary causative agent of neutropenic fever?
fungus
Which two fungi are the most common to cause complications from neutropenic fever?
candida and aspergillus
What organism is the most common cause of venous catheter infection?
Candida
What is Lhermitte’s Sign?
pain down spine during neck flexion
What are the three metabolic abnormalities seen during TLS?
hyperkalemia
hyperphosphatemia
hypocalcemia
Would TLS produce metabolic acidosis or metabolic alkalosis?
acidosis
Deposition of what electrolyte can cause arrhythmia problems?
phosphate
Which two metabolites precipitate causing renal stones during TLS?
uric acid
calcium phosphate
What are the three treatments for TLS?
hydration
allopurinol
Rasburicase
What drug is used before the onset of TLS?
allopurinol
What drug is used after the onset of TLS?
Rasburicase
What rxn does Rasburicase catalyze?
uric acid to allantoin
What is the best test for a PE?
CT Pulmonary Angiography
What is the Tx for a hemodynamically stable patient having a PE?
warfarin
What is the Tx for a hemodynamically unstable patient having a PE?
fibrinolytic
What is the treatment of Waldenstroms Macroglobulinemia?
plasmapharesis
What two types of drugs are known to cause a myelodysplastic syndrome?
alkylating agents
topoisomerase inhibitors
According to Hughes, how do alkylating agents progress through a MDS?
MDS and then leukemia
According to Hughes, how do topoisomerase inhibitors produce an MDS?
present as leukemia (AML)
How are hypocellular MDS treated?
immunosuppressives
What is the only chance at cure for an MDS?
bone marrow transplant
In which Myeloproliferative Disorder is it common to see leukocytosis (> 100,000)?
CML
When is anagrelide contraindicated ?
pregnancy
In patients with a JAK2 mutated Essential Thrombocytosis, what molecule and cell type are increased?
Hb and neutrophils
Which leukemia can present with arthralgia?
AML
Does Burkitts respond will to therapy?
yes
What are the three complications of radiation during Hodgkins Lymphoma?
lung cancer
breast cancer
heart disease
Is NHL more common in men or women?
men
What is the most causative agent of gram positive infections during neutropenic fever?
S. epidermidis
Which gram-negaives are most likely to cause severe neutropenic fever?
E. coli and pseudomonas
What virus is most likely to arise during neutropenia?
herpes
Is the prognosis for 5q syndrome good or bad?
good
Whick leukemia presents like a lymphoma?
CML
What are the two associated viruses of Hodgkins Lympoma ?
EBV and HIV
Can DLCL be cured? What therapy?
yes
CHOP
A lymphocyte count greater than 5K should have a patient worked up for what?
CLL
Which viral infection can produce an MDS?
HIV
Which has a shorter latency period, topoisomerase inhibitor or alkylating agent?
topoisomerase inhibitor
Does 5q Syndrome have a good or bad prognosis?
good
A hypocellular MDS resembles what other disease?
aplastic anemia
