Heme/Onc Flashcards
name the important neutrophil chemotactic agents
C5a IL-8 LTB4 kvllikrein platelet-activating factor
lipid A from bacterial LPS binds what on macrophages to induce septic shock
CD 14
what causes eosinophilia
Neoplasm Asthma Allergic RXN Chronic adrenal insufficniey parasites
what lymphocyte mediate cell immunity
T cells
what lymphocyte mediates humoral immune response
b cells
cell wth clock face chromatin distribution and eccentric nucleus with well developed golgi
plasma cell
what makes oxidized vitamin k into reduced vitamin k so it can activate the coagulation factors
epoxide reductase
antithrombin inhibits wha factors
2 7 9 10 11 12
inhibit ADP induced expression of GpIIb/IIIa
Ticlopidine
clopidogrel
Prasugrel
inhibit GpIIb/IIIa directly
Abciximab, Eptifibatide, Tirofiban
what activates vWF to bind to GpIb
Ristocetin
spur cells are seen in
Abetalipoproteinemia and Liver disease
what test tests for autoimmune hemolytic anemia because the body produces AB to RBC
direct coombs test
a test that detects antibodies to RBC unbound in the patients serum. used in pregnant women and before blood transfusions
indirect coombs test
cis deletion of alpha globulin in alpha thalassemia means what
deletions are on the same chromosome
cis deletion of A thalamseima is seen in
Asians
trans deletion of A thalassemia is seen in
Black people
b thalesemia is present in what people
mediteraniean
what is protective against major beta thalassemia
HbF until 6 months
b thalassemia major patients have an increased chance of what problem
parvovirus B19 induced aplastic crisis
what is the chelation for lead poison used in children
Succimer
sideroblastic anemia has a defect in what
ALA synthase gene
treatment for sideroblastic anemia
ALA synthase or pyridoxine
historical test used to diagnose B12 def.
Schilling test ( tells if the problem is malabsorption or dietary insufficiency)
defect in UMP synthase that causes failure to thrive in children and megaloblastic anemia. No hyperammonemia is present
Orotic Aciduria
important physical symptom of Diamond Blackfan syndrome
triphalangeal thumb
haptoglobin are increased in what kind of hemolysis
extravascular hemolysis
haptoglobin are decreases in what kind of hemolysis
intravascular hemolysis ( paroxysmal nocturnal hemoglobunbria)
hemolytic anemia of the newborn because of decreased levels of ATP is seen because of
Pyruvate kinase deficiency
what is the terminal complement inhibitor that treats Paroxysmal Nocturnal Hemoglobinuria
Eculizumab
Cold IGM complement induced autoimmune hemolytic anemia can be caused by
Mycoplasma Pneumonia
Mono
Cll
acute intermittent porphyria is caused by a deficiency in what
porphobilinogen deaminase
porphyria cutanea tarde is a deficiency in what
uroprphyrinogen decarboxylase
what usually cause HUS in children
SHiga toxin producing E. Coli (O157:H7)
skin necrosis and hemorrhage after administration of warfarin is seen when
there is protein C and S deficiency
what should be given if you want to replace coagulation factors fibrinogen and factor 8
cryopercipitate
8:14t
burritos
14:18 t
follicular lymphoma
11:14 t
mantle cell lymphoma
starry sky appearance of lymphocytes associated with EBV and a jaw lesion in the african type
Burritos lymphoma
most common type of non hodgkin lymphoma in adults
diffuse large b cell lymphoma
waxing, winning lymphadenopathy lymphoma
follicular lymphoma
verry aggressive with patients mainly presenting in the alter stages lymphoma
mantel cell lymphoma
AIDS defining illness that needs to be differentiated from toxoplasma
primary central nervous system lymphoma
associated with IV drug abuse which presents with cutneoys lesions and lytic bone lesions and hypercalcemia
adult t cell lymphioma
skin patches are seen with atypical CD4 cells with cerebriform nuclei which may progress to Sezary Syndrome
Mycosis Fungicides
hyper viscosity syndrome associated with an Mspike and Raynaud phenomenon
Waldenstrom Macroglobulinemia
bone lytic lesions, anemia, renal involvement and hypercalcemia
multiple myeloma
monoclonal plasma cell expansions that is asymptomatic that could lead to multiple myeloma
MGUS
neutrophils with bilobed nuclei usually seen after chemo
Pseudo-Pelter Huet Anomaly
what is the ALL with a better prognosis
12:21
t cell All can present how
like a mediastinal mass like in SVC syndrome
when SLL/CLL progresses into aggressive diffuse large be cell lymphoma
Richter Transformation
marrow fibrosis that causes dry tap on aspiration and spleenomegaly
hairy cell leukemia
hairy cell leukemia stains with what
TRAP
tx for hair cell leukemia
pentostatin
cladribine
AML that responds t retinoid acid
15:17
philadelphia chromsome 9:22 BCR-ABL represent
CML
what is Imatinib
BCR-ABL tyrosine kinase inhibitor that treats CML
lytic bone lesions in kids with skin rash and recurrent otits media . a mass involving the mastoid bone
Langerhans cell histocytosis
what is characteristic on the EM of langerhans cell histocytosis
tennis rackets, Birbeck granules
when is there a decrease in EPO seen in a polycythemia
vera
innaprpriate absolute polycythemia its seen when
there is ectopic EPO secretion (renal cell carcinoma)
when is there appropriate absolute polycythemia
high altitude
when is there relative polycythemia
dehydration and burns
name the two LMW heparin
Enoxaparin
Dalteparin
What kind of virus is parvovirus b 19
Non envelopes single stranded DNA
What kind of virus is EBV
Envelopes double stranded Dna
What kind off virus is hep c
Envelopes single stranded positive sense rna
What kind of virus is hep e
Non envelopes single stranded positive sense RNA
What kind of virus is HIV
Enveloped single stranded positive sense RNA
What kind of virus is parvovirus b 19
Non envelopes single stranded DNA
What kind of virus is EBV
Envelopes double stranded Dna
What kind off virus is hep c
Envelopes single stranded positive sense rna
What kind of virus is hep e
Non envelopes single stranded positive sense RNA
What kind of virus is HIV
Enveloped single stranded positive sense RNA
In mono there is hyperplasia of what part of the lymph node
Paracortex (t cell)
Peri arterial lymphatic sheath is the part that is distended when
There is a T cell infection like mono ( this is the area of T cells)
What does mono spot test detect
IgM Heterophile antibodies
name the six protooncogenes
RAS ABL HER2 EGFR MYC BRAF
Name six tumor suppressor genes
WT1 VHL RB Tp53 APC BRCA
protooncogene associated with hairy cell leukemia
BRAF
what do the BRCA genes do
they repair breaks in DNA
zinc containing enzymes that are used in wound healing and remodeling to break down collagen, laminin, and fibronectin
Metalloprotineases
CD 55 is used for what? and is missing in what
it is used to inactivate complement and it is missing in Paroxysmal Nocturnal Hemoblobinguria
hemolytic anemia, low haptons, pancytopenia and thrombosis of things like the mesenteric vein are associated with what disease
Paroxysmal Nocturnal Hemoglobinuria
explain hemosiderosis seen in PNH
there is a continuous destruction of RBC which leads to free iron being deposited in the kidney
cast nephropathy is seen in what disease
multiple myeloma
what diseases cause osteolytic bone metastasis
Multiple myeloma non small cell lung cancer non hodgkin lymphoma renal cell carcinoma melanoma
what diseases cause osteoblastic bone metastasis
prostate cancer
small cell lung cancer
hodgkin lymphoma
cancer associated with high parathyroid hormone related peptide (PTH rP) and hypercalcemia as well as osteolytic bone metastasis
non small cell lung cancer
diffuse medium sized lymphocytes and high proliferation index represented by the high Ki-67 fraction approaching 100% is seen in what disease
Burritk’s Lymphoma
how can giving packed RBC to a patient who lost a lot of blood cause hypocalcemia
packed blood contains citrate which can chelate calcium and cause hypocalcemia (parathesias)
* usually seen after a massive transfusion
what infections induces heterophil antibodies that react with antigens from animal RBCs (sheep-PaulBunnell test or horse - Monospot test)
EBV infections
what are two malignancies that EBV is associated with
BurrKits lymphoma
Nasopharyngeal Carcinoma
the absence of factor 8 and 9 won’t allow for the activation of factor 10 and subsequently won’t allow for the activation of prothrombin to thrombin so clots won’t be formed. What can you give to someone with hemophilia who won’t quit bleeding
thrombin
what turns fibrinogen into fibrin, main part of a clot
thrombin
waxing and waning lymphadenopathy are common in what cancer
follicular lymphoma
what cells clonal expand because of EBV infected B cells
Cytotoxic T cells CD 8+
CD14 is a marker for what
macrophages, this is where LPS binds to activate them
decreased ferritin, increased transferrin and low MCV are seen in
IRON deficiency anemia
RECAM-1 is used for
transmigration
ICAM-1 is used for
tight adhesion
Leukocyte Adhesion Deficiency type 1 is what
when there is an absence of CD 18 so that interns Mac-1 and LFA1 aren’t synthesized so there is no tight adhesion, crawling or trasnmigration
symptoms of LAD 1
skin infections with no puss, delayed detachment of umbilical cord and poor wound healing
small rings called trophozoites found on RBC is common in what disease
malaria p. falciparum
main treatment for malaria
chloroquine
treatment for malaria in chloroquine resistant areas
mefloquine
ivermectin treats>
Onchocerciasis “River Blindness”
Nifurtimox treats
Chagas Disease
Pentamide is used for what
prophylaxis of Pneumocystis Jirovecii in HIV patients
- african sleeping sickness and leishmaniasis
what is needed to kill P vivid and P oval liver hypnozoites
Primaquine
most cases of HUS are preceded by what
diarrhea from Shiga toxin producing bacteria (Shigella and Ecoli 0157:H7)
systemic autoimmune disease characterized by proximal muscle weakness resembling polio but with the additional inflammatory features that involved to skin
Dermatomyositis
Gottronsq Papules and Heliotrope rash are seen in what disease
Dermatomyositis
non enveloped single stranded DNA virus that can cause arthralgia in the hands, knees, wrist and feet without a rash
Parvovirus
Why is haptoglobin decreased in hemolytic anemia due to prosthetic valves
Because haptoglobin binds to free hemoglobin and with the increased destruction of red blood cells, there is more free hemoglobin binding to the haptoglobin making the number of haptoglobin decease
Repeated splenic infections seen in sickle cell disease makes what happen to the spleen
Fibrosis and atrophy
Splenic sequestration can cause what to happen to the spleen
Splenic congestion
Patients with sickle cell disease are lily to develop what because of high RBC turnover
Folic acid deficiency
Parvovirus in a pregnant woman can cause what in the fetus
interruption of erythropoiesis causing profound anemia which causes CHF. This CHF causes pleural effusion, lung hypoplasia and ascites
what kind of viruses are VZV, CMV and HSv
enveloped dsDNA
what kind od virus is rubella
enveloped ss RNA
What can VZV in a pregnant woman do to a fetus
cause limb hypoplasia
indirect bilirubin is the same as
unconjugated billirubin
what is the most specific red cell index for spherocytosis
increased mean corpuscular hemoglobin concentration
what is caused by a defective binding of the red cell cytoskeleton to the plasma membrane due to mutations involving ankyrin, band 3 and spectrum proteins
hereditary spherocytosis
pappenheimer bodies in RBCS are associates with what
sideroblastic anemia
increased osmotic fragility on acidified glycerol lysis test ( hemoglobin is released when the patients RBC are incubated with glycerol) is seen in
hereditary sphereocytosis
pigmented gallstones are a complication of what
hemolytic anemia like in hereditary spherocytosis
two factors that drive angiogensesis
VEGF
FGF
what can interferon-y do
INDIRECTLY PROMOTE neovascularization by activating macrophages (these release VEGF)
benzene is found in what
gasoline and tobacco smoke
what disease affects erythrocyte precursors and causes red cell aplasia ( anapestic crisis) in patients who already have a preexisting blood disease like nickel cell, or hereditary spherocytosis
Parvovirus B19
neuroblastoma has an over expression of what marker
n-myc
what is the function of the c-myc gene
transcription activation which controls cell proliferation, differentiation and apoptosis
cyclin D1 promotes what
g1–> s phase
what gives the toughness and rigidity to keratin protein
the disulfide bonds between the cysteine molecules
what gives keratin its small structure and ability to coil tightly and form many hydrogen bonds
alanine and glycine molecules
what are the two markers used for neuroendocrine tumors
Chromogranin A and neuron specific enolase
what is the intermediate filament seen in mesenchymal tissue
vimentin
what pathology is associated with an inversion of the short arm of chromosome 2 that creates a fusion gene between EML4 ( echinoderm microtubule associated protein like 4) and ALK ( analytic lymphoma kinase) that causes active tyrosine kinase that causes malignancy
non small cell lung cancer
treatment for the EML4-ALK fusion protein that causes malignancy in non small cell lung cancer
Crizotinib (tyrosine kinase inhibitor)
EML4-ALK non small cell lung cancer pathophysiology is the most similar to what cancer
CML ( it is also a fusion protein)
hemolytic anemia that presents after the administration of TMP-SMX is usually due to what disease
G6PD deficiency
increased vulnerability od RBC to oxidative stress manifesting as hemolytic anemia that is induced by
- infection
- drugs ( bacterium, dapsone, antimalarials, nitrofurantoin)
- fava beans
G6PDD
x linked disorders mainly seen in men in which presides of oxidative stress cause extra/intravascualr hemolysis. Haptoglobin are low and urine is dark read. Fave bean, and sulfa drugs canc cause this
G6PD D
what should be checked before administering dapsone so that you do not cause hemolytic anemia in your patient
G6PD levels
how does peripheral tolerance occur
by T cell anergy ( inactivation of t cells that react to self)
central tolerance happens where
in the fetal thymus during negative selction
where does isotoype switching of b cell occurs ( when they switch from IgM to something else by making antibodies)
in the germinal centers in the lymph nodes
interaction of the CD40 receptor on B cells and the CD40 ligand on activated T cells cause what
isotope switching
where are heterophiles antibodies found
in patients with EBV mono
when are ringed sideroblast found
in myelodysplastic syndrome
the hEr2 oncogene encodes for what
185-kD transmembrane glycoprotein that has intracellular tyrosine kinase activity
toll like receptors release what
cytokines
what is clathrin
an intracellular cytosolic protein the mediated endocytosis
autoimmune destruction of platelets by anti platelet antibodies likely IgG agains GpIIb/IIIa
Immune thrombocytopenia purpura
main clue for immune thrombocytopenia purport
isolated thrombocytopenia
small lymphoid cells with increased nuclei to cytoplasmic ration and cleaved nuclei are seen in what cancer
follicular lymphoma
what is the disease that presents as a benign leukocytosis that happens in response to an underlying condition like infection, hemorrhage, malignancy or acute hemolyisi.
Leukemiod reaction
Dhole bodies are basophilic granules inside of neutrophils seen in
leukemiod reaction
where dose positive selection happen
in the cortex of the thymus
where does negative selection occur
in the medulla of the thymus
interaction of the developing t cell with thyme medullary epithelial and dendritic cells in seen in
negative selection
eczematous nipple lesion is associated with what kind of disease
pagets
what causes black gall stone
chronic hemolysis
what causes brown gall stones because of the release of microbial B-glucuronidases
biliary tract infection
what are two things that the splenic red pulp is important for
- destroying aged RBCs
2. Clearing out circulating bacteria
what are the three main encapsulated bacteria that can present as a problem for individuals with no spleen
Strep Pneumo
H. Influenza
N. Meningitides
what causes macrophages and t cells to be activated in viral infected cells
interferon a and b
pro carcinogens are metabolized by what
P450 monooxygenase
what makes bilirubin into soluble bilirubin
glucuronide transferase
PT prolongation seen in liver dysfunction is due to what
Factor 7 running out from the extrinsic pathway (shortest half life)
pure red cell aplasia is associated with what?
thymic tumor (thymoma)
MAIN MECHANISMS OF IONIZING RADIATION
double strand breaks in the DNA
free radical formation
hypophosphorylated RB is what
active
hyperphosphorylated RB is what
inactive
explain the pathophysiology of Thrombotic Thrombocytopenia Purpura
there is a decrease in ADAM13 activity which causes a larger vWF multiuser that causes microvascular platelet rich thrombi
name the clinic features of Thrombotic Thrombocytopneic Purpura
hemolytic anemia
low haptoglobin
thrombocytopenia
schistocytes
how to manage TTP
plasma exchange
what is the normal role of ADAM13
it cleaves vWF into smaller pieces so that clotting won’t occur
IgA immune complex deposition and palpable purpura renal disease and arthritis
Hence Schonlein Puprpura
frequent nose bleeds and HEAVY menstrual periods is a good sign of what disease
vWF deficiency
deep tissue blessing into muscle and joints, g.i. bleeding is usually a sign of what disease
Factor 8 deficiency
clot instability and recurrent bleeding after surgery is a sign of what disease
Factor 13 deficiency
vWF is a protective carrier for what
factor 8
vWF binds to what
collagen
prolonged PTT and bleeding time is seen in what problem
vWF deficiency