AAW - Heme/Onc Flashcards
cancers assc with EBV
Associated with Hodgkin lymphoma, endemic Burkitt lymphoma (seen most commonly in kids and involves the jaw), nasopharyngeal carcinoma.
burkitt lymphoma general
c-myc gain of function (oncogene)
t(8;14)—translocation
of c-myc (8) and
heavy-chain Ig (14)
Starry sky appearance - sheets of lymphocytes with interspersed macrophages
jaw lesions in endemic form in Africa,; pelvis or abdomen in sporadic form
Diffuse large B-cell lymphoma general
usually in older adults
t(14;18)
most common type of non-Hodgkin lymphoma in adults
Mantle cell lymphoma general
I am an older male with 5 CDs on my Mantle which makes it Super LLegit/CooLL so don’t Smudge them (my mantle is above the TV where I am watching the movie “11:14” (google it))
Mantle cell lymphoma and Small Lymphocytic Lymphoma/Chronic Lymphoid Leukemia (with smudge cells) are CD5+
t(11;14)
follicular lymphoma general
adults
t(14;18) translocation of heavy chain Ig and BCL-2
BCL-2 inhibits apoptosis
all the translocations of non-hodgkin lympoma
burkitt, mantle, diffuse/follicular
8;14
11;14
14;18
kind of a pattern
Adult T-cell lymphoma general
caused by HTLV-1 (assc with IV drug abuse)
adults present with cutaneous lesions, lymphadenopathy, hepatosplenomegaly, lytic bone lesions, hypercalcemia
endemic in japan, west africa, caribbean
mycosis fungoides/sezary syndrome general
adults- cutaneous t-cell lymphoma
present with cutaneous crusty red patches
circulating malignant cells seen in sezary syndrome
indolent, CD4+
virus assc with cervical, anal, penile carcinoma
HPV
HPV 6 and 11 are low risk
consider everything else high risk for the high yield only
the warts that HPV causes are called verrucae: Soft, tan-colored, cauliflower-like papules
genital warts = condyloma acuminatum
hepatocellular carcinoma assc virus
chronic hep B/C virus
kaposi sarcoma assc virus
HHV8
burkitt lymphoma oncogene
c-myc
gene product is transcription factor
also t(8:14)
(mantle t(11;14), diffuse and follicular lymphoma t(14;18))
patient goes into starvation mode and they get:
painful abdomen, hallucinations, dark red colored urine
Acute intermittent prophyria
deficiency in porphybilinogen deaminase
accumulated prophobilinogen in the urine
Symptoms (5 P’s): Painful abdomen Port wine–colored urine Polyneuropathy Psychological disturbances Precipitated by drugs, alcohol, and starvation
very young patient presents with large mass in the inferior pole of the retina
what cancer
what other cancers go with it
what gene
retinoblastoma
osteosarcoma
Rb gene deletion (gene product inhibits E2F, blocking G1 ->S phase)
bleomycin
intercalates DNA during G2, causes scission by an oxidative process
pulmonary side-effects
cyclophosphamide
DNA alkylator - crosslinks DNA at any stage of cell cycle
activated by cytochrome P450
vincristine is mainly used for
lymphomas
most common metastatic tumors to the peritoneum
ovarian and pancreatic carcinoma
african american with who gets jaundice, fatigue, SOB after taking a drug
what is most likely enzyme deficiency
what are some possible drugs
g6pd
drugs that cause ROS (no G6PD means no HMP shunt which means no NADPH for glutathione which means oxygen radicals in RBCs reign supreme)
welder presents with microcytic anemia and basophillic stippling
what is affected
lead poisoning
Ferrochetalase (binds iron to heme) and delta-aminolevulinic acid dehydratase enzymes (ALA dehydratase)
accumulated protoporphyrin, delta-ALA in the blood
Can mimic a porphyria
factors involved in the extrinsic pathway
I, II, V, VII, X
hemophilia a,b,c
all have increased PTT and normal PT (intrinsic pathway disorder)
ABC
8,9,11
a: deficiency of factor VIII; x-linked recessive
b: deficiency of factor IX; x-linked recessive
c: deficiency of factor XI; autosomal recessive in ashkenazi jews
Sx: macrohemorrhage, hemarthroses (bleeding into joints), easy bruising.
retinoblastoma in a kid
what other cancer are they at risk for
osteosarcoma
both controlled by the RB gene
osteochondroma
most common benign bone tumor
males less that 25 years old
bony exostosis with cartilaginous cap
rarely transforms into chondrosarcoma
giant cell tumor
benign but locally aggressive
20-40 years old
epiphyseal end of long bones, often around knee
aka “osteoclastoma”
soap bubble on x-ray
osteosarcoma
2nd most common primary malignant bone tumor after MM
bimodal distribution, 10-20 years old, greater that 65
Predisposing factors: Paget disease of bone, bone
infarcts, radiation, familial retinoblastoma,
Li-Fraumeni syndrome (germline p53
mutation
metaphysis (between epiphysis and diaphysis) of long bones, often around knee
elevation of periosteum or sunburst pattern on x-ray
what chemotherapy’s side effects are made better by leucovorin?
which drug’s side effects are made worse
with MTX, myelosuppression is reversible with leucovorin “rescue”
with 5-FU, myelosuppression is worsened with leucovorin
leucovorin = folinic acid
nighttime bone pain, raised lesion in the diaphysis of a long bone
osteoid osteoma
“pseudopalisading” pleomorphic tumor cells that border central areas of necrosis and hemorrhage
what cancer
what cells stain for GFAP
glioblastoma multiforme
(grade IV astrocytoma)
astrocytes stain for GFAP
brain tumor of arachnoid cells
spindle cells concentrically arranged in a whorled pattern
Meningioma - spindle cells concentrically arranged in a whorled pattern is psammoma bodies
its external to brain parenchyma, may have dural attachment
cerebellar
closely arranged, thin-walled capillaries with minimal intervening parenchyma
what tumor
paraneoplastic effects?
assc with what disease?
hemangioblastoma
can produce erythropoietin leading to polycythemia
VHL syndrome with found with retinal angiomas
cancers that are S-100 positive
mesodermal origin cancers
langerhans cell histiocytosis
Melanoma
Schwannoma (classically at the cerebellopontine angle, but can be in any peripheral nerve. bilateral vestibular schwannomas found in NF-2)
cerebellopontine angle tumor
schwannoma, classically
S-100 +
frontal lobe calcification
lots of thin capillaries, round nuclei with clear cytoplasm,
oligodendroglioma
rare, slow growing
“fried egg cells”
trousseau sign
migratory thrombophlebitis - redness and tenderness on palpation of extremities
seen in pancreatic carcinoma (producing tissue factor)
treatment regimen for hodgkin’s lymphoma
DVBD
doxorubicin
vinblastine
bleomycin
dacarbazine
3 year old abdominal mass big tounge big organs one side of the body is bigger than the other
what chromosome is affected
what is changed on that chromosome
beckwith-wiedemann: wilms tumor, macroglossia, organomegaly, hemihypertrophy
deletion of the WT2 tumor suppressor gene on chromosome 11 (just wilms tumor is deletion of WT1 on chromosome 11)
recombinant IL-2 is used to treat what
RCC, metastatic melanoma