UNIT 2: Hemo Onc practice exam Flashcards
What is missing in CGD
NAPD Oxidase
What kind of microbes are people with CGD susceptible to?
Catalase positive
ex. Staph aureus and aspergillus
Which disease results in extramedullary hematopoiesis with leukoerythroblastic blood smear and why?
Primary myelofibrosis
megakaryocytes produce excess PDFG which leads to marrow fibrosis
the other organs now need to help make RBC bc theres no space in the bone marrow
What cells are proliferating predominantly in primary myelofibrosis
megakaryocytes in bone marrow
What is aplastic anemia indicated by
Lack of cell production in the marrow and low level of marrow derived cells
Do you see extramedullary hematopoiesis in iron deficency anemia
NO
What is characterizes Hodgkins lymphoma
Proliferation of reed sternberg cells
What are the common symptoms of Hodgkins lymphoma
fever, chills, unintentional weight loss, and night sweats
what are some things that are seen in pts with multiple myloma
Bone pain with “punched out” lesions
M-spike on electrophoresis (monoclonality)
Bence Jones proteinuria
Rouleaux formation (inc serum proteins)
What antigen is seen in ovarian or other gyn cancers?
CA-125
What does high WBCs in UA mean?
Infection of urinary tract
What is messed up in Xlinked-Agamma…
Brutons tyrine kinase (BtK)- involved in B-cell maturation
B cell cant go bro progenitor B cell to pre Bcell,
therefore low B cell count and NOOOOO antibodies
X-linked Agammaglobulinemia presentation and treatment
Presentation: Male with recurrent infections, pneumonia, otitis media, GI problems (giardia)
Treatment: IVIG, prophylactic Abx
What in question can lead to us to Brutons agammaglobinemia?
Boy with disease, has boy fam member with same disease (X-linked)
Low B count, normal T cell count
Low serum Ig’s (all types)
Hyper IgE syndrome (Job’s) defect
STAT3 gene–> impaired Th17 differentiation–> dec. neutrophil activity to infection site
Job’s syndrome presentation
-High IgE
-Cold staph aureus abscesses
-Eczema
-Retained baby teeth “two rows”
-
What is messed up in wiskott aldrich syndrome? and what type of inheritance?
WAS gene mutations—> defective WASP—> affects immune cell cytoskeleton
X-linked
Wiskott aldrich syndrome presentation
-thrombocytopenia–>inc risk of bleeding
-Eczema
-petechiae
-Recurrent infection
-IgE and IgA inc.
-IgM dec.
What is messed up in Ataxia Telangiectasia
ATM gene defect–> defective DNA repair
Presentation of AT
Ataxia- involuntary movements
Telangiectasia- small dilated vessels visible on skin
-recurrent infections
What disease is associated with ADA
SCID
SCID presentations
-persistant muco-cutaneous candidiasis
- recurrent viral infections
-opportunistic pathogen infections (esp. pneumo jiro)
-live vaccines can kill these pt’s
-recurrent diarrhea and failure to thrive
-low T cell count
-Absence of thymic shadow
What CD markers are on NK cells
CD56 and CD16
What CD markers are on B cells
CD 19, CD 20, CD 21
What CD markers on follicular dendritic cells?
CD21
What cells have CD35
B cells, RBCs, monocytes
What is the role of CD35
help clear antigen:antibody complexes and foreign material from body
what Ig is used for primary antibody response?
IgM
What two important molecules are present in breast milk?
Vit K and IgA
what is IgG used for?
Opsonization and neutralization of bacterial toxins
What kind of drug is Doxorubicin
Anthracycline
MOA of Doxorubicin
Intercalates between G-C base pairs
What drugs inhibit thymidylate synthase (dUMP –> dTMP)
5-flurouracil, pemetrexed, and capecitabine
What drugs inhibit topoisomerase I
Topotecan and irinotecan
What does colchicine do?
Arrest cells in M phase by interfering with mitotic spindle formation
What drugs inhibit topoisomerase II
etoposide and teniposide
What is the most common type of leukemia?
Chronic Lymphocytic leukemia
What CD markers are expressed in CLL
CD20 and CD 5
-CD5 typically on T cells
What disease contains smudge cells
CLL
Leiomyoma =
benign neoplasm of smooth muscle
Fibroma =
benign neoplasm of fibrous tissue (connective)
Osteosarcoma =
Malignant growth of bone
Rhabdomyoma =
non-malignant growth of striated muscle
Adenoma =
Benign growth of gland type tissue
what does endogenously vs exogenously synthesized mean in terms of antigens
Endo means antigen originated from inside cell
Exo means antigen originated from outside cell (bacteria toxin type)
what does methotrexate inhibit?
DHFR
which can lead to megaloblastic anemia with hypersegmented neutrophils due to disruption of DNA synthesis
what is AIRE’s function
Induce thymic expression of peripheral antigens
What does ADA do
Degrades adenosine to deoxyadenosine
What does Checkpoint kinase 1 do
detects DNA damage and regulates cell cycle progression
What does WASP do?
link signaling pathways to actin cytoskeleton reorganization
What is the triad of symptoms in wiskott aldrich syndrome
Thrombocytopenia
Eczema
Recurrent infections
What is the difference between Mycosis fungoides and sezary syndrome
Mycosis fungoides has sezary cells in skin rash/nodules/plaques, but DOESNT have sezary cells in bloodstream
Sezary syndrome is when there is sezary cells in the bloodstream
Why kind of cells are proliferation in mycosis fungoides
Atypical mononuclear mature CD4 T cells
what disease is associated with t15;17
Acute promyelocytic leukemia
What is defective in APL
t15;17 leads to defective RAR
leads to blockage of myeloid cell maturation
which causes accumulation on blasts
What lab finding automatically rules out chronic leukemia?
BLAST proliferation
What basic principle is seen in multiple myeloma
Monoclonal expansion of plasma cells
ALL is proliferation of lymphoblasts or myeloblasts
lymphoblasts
what are some lab findings to differentiated ALL and AML
ALL- TdT positive
- t12;21
-t9;22 (both translocations associated with B-ALL)
Previous infection of EBV leads to what kinds of cancers?
Hodgkins lymphoma, nasopharyngeal carcinomas, burkitts lymphoma
What kind of cancers are associated with HPV infection
cervical, penile, anal, esophageal cancer
Hep C virus can lead to what kind of cancer
liver adenocarcinoma
what kind of cancer is HHV-8 associated with
Kaposis sarcoma
What disease is associated with Human T- Lymphoblastic Virus infection (HTVL-1)
Adult T cell Leukemia/lymphoma
What co-stimulatory signal proteins are needed for T-cell activation
B7:CD28 (T cells carry CD28)
What cell markers needed to activate B cell differentiation
CD40; CD40L (Ligand on T cell)
What ENHANCES T-cell activation
CD2:CD58 (CD58- on APCs)
What does LFA-1 do?
Surface protein on leukocytes that binds to ICAM on endothelial cells for adhesion
What are common presentation of Hemophilia A?
Deep tissue, joint and post surgery bleeding
Increased PTT
Bruising easily
What is used to treat hemophilia A/B
Factor concentrate and desmopressin
What does desmopressin do
vasoconstrictor that stimulates vWF
What cytokines contribute to fever? (pyrogens)
IL-1
IL-6
TNF-a
What kind of drug is Imatinib?
Tyrosine kinase inhibitor
What causes CML
t9;22 translocation (Philadelphia chromosome)
BCR-ABL fusion, tyrosine kinase activity increased
What drug is used to treat CML and why?
Imatinib. Because its a tyrosine kinase inhibitor. therefore inhibits BCL-ABL
Common presentations of sickle cell anemia
Pain crisis (left flank pain, spleen)
Dactylitis
gross hematuria
What MPD’s are associated with JAK2 kinase mutation
Polycythemia vera
Essential thrombocythemia
Myelofibrosis
Common presentation of PV
Increased platelets, granulocytes, RBCs (major)
ruddy (reddish) complexion
elevated hemoglobin and hematocrit
Headaches and dizziness from inc. blood viscosity
Itching after bathing
What drug is used for anaphylactic reaction? (nut allergy)
Epinephrine
What does Epi do?
Rapid acting vasoconstrictor and bronchodilator
What drug is an anti-histamine
Diphenhydramine (Benadryl)
What does Albuterol do?
Bronchodilator used for ASTHMA
Recurrent ear infections and pneumonia suggests what? and why is it important?
pt is susceptible to encapsulated bacteria, which means theres most likely a defect is opsonization (B cell problem)
What is commonly seen with CGD pt’s?
granule formation due to immune system trying to contain catalase positive organisms
What causes increased susceptibility to candida infections?
MPO defects
What would be missing in leukocyte adhesion defeciency?
Purulent effusions (No neutrophils at infection site)
What should cue me to low platelet count disorders in lab findings?
Low platelet count, but normal PT/PTT
(ITP)
What would help me CONFIRM ITP diagnoses
Blood smear with low platelets BUT with megakaryocytes
What would a Platelet factor 4 assay test?
Heparin Induced thrombocytopenia
What would a ristocetin cofactor assay test?
Diagnosis of vWF disease(positive) or abnormal platelet adhesion/aggregation (negative test)
What complement proteins are anaphylatoxin (promotes inflammation) that attracts neutrophils
C5a and C3a
What complement protein acts as an opsonin
C3b
What complement protein activates classical cascade
C1
Elevated homocysteine, normal methylmalonic acid =
folate deficiency
Elevated homocysteine, elevated methylmalonic acid =
Vit B12 deficiency
Why would there be low serum ferritin
low iron storage
Why would there be high TIBC
to attempt to recuperate stored iron
Why would there be high transferrin
to try and promote iron absorption
Why does somatic hypermutation occur
to reach affinity maturation; to process antibodies with even higher affinity of the original antigen
What B cell antibody formation processes occur in the bone marrow PRIOR to antigen exposure
Random recombination of heavy and light chains
Allelic exclusion
VDL recombination
What is alternative splicing in B cells
Process that changes the constant region (Isotype switching) of antibody AFTER the B cell is presented an antigen and activated
DOES NOT CHANGE VARIABLE REGION
What type of inheritance is Hyper-IgM syndrome
X-linked
What is messed up in Hyper-IgM syndrome
Defective CD40L on T cells—> absence of B cell class switching
What lab findings is indicative of Hyper-IgM syndrome
Defective CD40L (obvi)
Normal B and T cells
Increased IgM and low IgA,G,E
Normal complement and neutrophils
What is CD55
DAF
What molecule does CD55 (DAF) interact with
C3b to inhibit activation/opsonization of host cells
What is CD16:Fc region of IgG called
antibody dependent cytotoxicity
What is CCR5
Co receptor on macrophages and CD4 cells for HIV infection (bings Gp120 of virus)
Hyperplasia=
increase in cell number
Hypertrophy=
increase in cell size (rather than number)
Metaplasia =
conversion of one adult cell type to another (squamous –> cuboidal)
Dysplasia=
abnormal cell growth. can lead to formation of cancer
Atrophy
decrease in cell size
IL-2 receptor gene mutation is associated with what diease
SCID
What is the type of inheritance of IL-2 receptor gene mutation SCID
X-linked recessive
What type of inheritance is ADA deficiency
Autosomal recessive
What type of inheritance is RAG1/2 mutation (Omenn syndrome)
Autosomal recessive
What do RAG 1/2 do?
Recombination Activating Gene
-helps with VDJ recombination
What cells are positive for CD14
Neutrophils and Macrophages
Why is CD14 important
CD14 and TLR4 bind to LPS of gram neg bacteria inducing inflammatory response (IL-1,6 and TNF-a)
TGF-B main role
cytokine involved cell growth, differentiation and apoptosis
Terminal deoxynucleotidyl Transferase function
TdT.
DNA polymerase that adds random nucleosides to DNA during VDJ recombination
Present in B and T cells
IL-10 function
anti-inflammatory and regulation cytokine
Recombination activating gene 1 function (repeat)
-helps with VDJ recombination
What mutation is seen in PNH
(phosphatidylinositol glycan class A)PIGA gene mutation—> GPI defect—> DAF cant bind–> complement binds to host cells and destroys them
What does P-glycoprotein 1 do?
A membrane transporter unregulated in cancer cells that pump out chemotherapy drugs
Why is expression of E-Cadherin important?
It mediated contact inhibition which inhibits cell proliferation
What kind of drug is cyclophosphamide
Alkylating cancer drug (cross-links DNA strands)
What drug can cause hemorrhagic cystitis? and what drug is co-adminstered to make sure this doesnt happen
cyclophosphamide
Co-admin: MESNA
What platin causes nephrotoxicity the most
Cisplatin
What drug is co-adminestered to combat nephrotoxicity of cisplatin
Amifostine
What drug is used to treat heavy metal poisining
Dimercaprol
What drug is used to prevent thickening of striated muscle due to doxorubicin
Dexrazoxane
What is contact inhibition
Halting cell growth when cell comes into contact with neighboring cell
What can bleomycin cause?
-skin toxicity (rash, hyperpigmentation)
-pulmonary toxicity (dyspnea, pneumonitis)
-mucositis and stomatitis (inflammation of mouth and lips)
-alopecia
Allopurinol MOA
Inhibits Xanthine oxidase (no uric acid formation)
Why is allopurinol given to prevent kidney damage with chemo treatments
Chemo breaks down tumors and they release their contents into bloodstream which can lead to hyperuricemia (plus other things). Allopurinol stops uric acid production and saves the kidney
What can cue me to vWF disease
Increased bleeding time with NO EASY BRUISING or thrombocytopenia
Lab findings for vWF disease
Increased PTT, normal PT, Increased Bleeding time due to decreased platelet adhesion/aggre.
Explain T0-4 in TNM staging
T0= no tumor
T1= small tumor confined in tissue
T2=Larger tumor confined in tissue
T3 = Tumor extends beyond the organ but not into major structures
T4 = Tumor invades nearby structures
Explain N0-3 in TNM staging
N0 = No lymph node involvement
N1 = A few nearby lymph nodes involved
N2 = More lymph nodes involved, further from primary tumor
N3 = Extensive lymph node involvement
Explain M0-1 in TNM staging
M0 = No distant metastasis
M1 = Distant metastasis present (e.g., lung cancer spreading to the brain)
What causes Beta thalassemia major
mutations in both B globin chain genes which leads to hypochromic, microcytic hemolytic anemia
What does Beta thalassemia major present with
Children demonstrate failure to thrive early in life.
Bone marrow biopsy shows frontal bossing (crewcut appearance) due to RBC production expanding to head bones
Hepatosplenomegaly
dark urine
Target cells
What does G6PD deficiency present with
Hemolytic anemia
jaundice
dark urine
fatigue and anemia
Hienz bodies
What causes hereditary spherocytosis
defects in cytoskeletal anchoring proteins such as ankylin and spectrin
Results in hemolytic anemia due to increased RBC fragility
What does hereditary spherocytosis present with
Pale skin
Jaundice and splenomeg.
What type of hypersensitivity reactive involves T cells and macrophages with NOOO antibodies
Type 4 (delayed)
What type of hypersensitivity reaction is is post streptococcal glomerulonephritis
Type 3
What are codocytes
Target cells
What type of hypersensitivity rxn is atopic dermatitis (Eczema)
Type 1- dry, red, itchy skin
what cells are predominantly found in medullary chords of lymph nodes
Plasma cells and macrophages
what cells are predominantly found in medullary sinuses of lymph nodes
reticulocytes and macrophages
IL-8 function
Proinflammatory cytokine that acts as a chemoattractant for neutrophils
What is cachexia
EXTREME muscle fatigue weakness (muscle wasting)
What cytokine mediates cachexia
TNF-a
What state is bone marrow in during aplastic anemia
Hypocellularity
Mitotic inhibitor drugs
Vincristine
Vinblastine
Taxanes (-taxal ending)
-pactitaxel
-docetaxel
-cabazitaxel
Side effects of paclitaxel
Anemia, hair loss, numbness and tingling of hands and feet (peripheral neuropathy)
What kind of drugs are associated with hand foot syndrome
Pain,redness and swelling in palms of hands and feet.
Thymidylate synthase inhibitors
(5 fluorouracil)
What long does anti HIV antibodies to form after infection
3-4 wks
What NRTI is used to treat neonates and pregnant mothers
Zidovudine
Side effects of Zidovudine
Anemia, Agranulocytosis (dec.), myelosupression
What does chikungunya virus cause
-maculopapular rash (flat and raised skin rash)
-Arthralgia;multiple joint pain
What does Zika cause
Mild symptoms in adults who are asymptomatic
Symptomatic pts
-conjunctivitis
-Microcephaly in neonates with vertical transmission
-Guillain Barre syndrome
What does Yellow fever virus cause
-jaundice
-bloody diarrhea
-hemorrhagic fever leading to bleeding diathesis
What does west nile fever cause
Encephalitis
Meningitis
Acute flaccid paralysis
Neurological dysfunctions (seizures, parkinson’s like symptoms
Does sudden onset (acute) fever, chills, headache and chills come from bacterial or viral infections
Bacterial
Viral usually causes slower onset
Does PAINFUL LAD compared to tender LAD make a difference characterizing the type of infection?
Yes. Typically tender LAD is viral infection
Painful LAD (bubos) is caused by yersinia pestis (bubonic plague). A gram - bacillus
Which bacteria has bipolar coloration on wayson stain
Yersinia spp.
Which bacteria has “ghost cells” on gram stain
Mycobac. Turburcolosis
What bacteria has round encapsulated yeast organisms on india ink stain
Cryptococcus spp
MOA of amphotericin B and nyastatin
Binds ergosterol and pokes holes in fungal cell membrane
MOA of flucytosine
Halts RNA and DNA synthesis by acting as a Uracil analog
What do amphotericin and flucytosine combo treat
Cryptococcus infections
What major side effect is seen with amphotericin B medication
Kidney toxicity
Common side affects of NNRTI’s
GI upset, rash, and liver toxicity
What does echinocandins treat and how does it work
Candidiasis and aspergillus infections by inhibiting glucan synthesis of fungal cell walls
what pathogen usually infects pt with AIDS, pneumonia with ground glass appearance
Pneumo jiro
pt with pneumonia that has ground class appearance that is immonocomprimised
Aspergillus fumigatus
If question stem is talking about chemo therapy, bone marrow transplant.. what are they trying go tell you about the pt
They are immunocompromised and can develop infections easily. espicially from opportunistic pathogens
What are the opportunistic fungi
Candida albicans
Aspergillus fumigatus
Pneumocystis jirovecii
Cryptococcus neoformans
Mucor and Rhizopus
What does a mucor and rhizopus infection present with
headache
vision changes
sinus congestion
necrotic lesions on nose or mouth
What do cryptococcus infections present as
Lung infections (bubble appearance) that disseminate and cause meningitis
What does sputum mean?
Mucus or phlegm from pulmonary tract
What does aspergillus infections usually present with
Pulmonary problems(mainly)
-pneumonia (cough)
-hemoptysis
Kidney problems
heart problems
Brain abscesses
What is Langerhans cell histiocytosis
Proliferation of skin dendritic cells that causes a rash
What cell markers are present in langerhans cell histiocytosis
CD1a+ and S-100+
What are birbeck granules
“Tenis-racket” granules present in langerhans cell histiocytosis
What does burkitts lymphoma present with
Extranodal swelling usually seen in haw region due to B cell proliferation linked to EBV infection
What is osteomyelitis
Infection of the bone. INFECTION
What lab values are elevated during an infection
CRP—> inflammation
ESR due to inc. fibrinogen
Both APR’s produced during infection
t8;14
Burkitts
c-myc (cell growth)
t11;14
MANTLE CELL LYMPHOMA
cyclin D1
t14;18
follicular lymphoma
Bcl2
t12;21
B-ALL
t9:22
CML - BCR ABL (inc. mature granulocytes)
What does painful LAD mean
Infection is occurring. either viral or bacterial
What does painless LAD mean
Lymphoma occuring
What type of antibody attacks RBCs in transfusions when blood types are different
IgM. anti A and/or B antibodies
What type of antibody attacks RBCs in transfusions when Rh is different
IgG. anti-Rh antibodies
If pt with syphilis is allergic to penicillin what drugs are given
Doxycycline or Tetracycline
What is Factor V Leiden deficiency
Mutated Factor V that lacks binding site for Protein C (anti-coag)
Leads to hypercoagulable state
What is the most important prognostic value in tumor staging (TNM)
METASTASIS
either M0 or M1, very important
What is leucovorin
A drug that serves as a folate analog
When is leucovorin used and why?
During chemo therapy with methotrexate
Methotrexate can cause folate deficiency and lead to megaloblastic anemia. Leucovorin bypasses this which fixes megaloblastic anemia
Rewatch babesia sketchy
UGHHHHH
What bug has the maltese cross on blood smear
Babesia spp
What is the vector for babesia
Tick
What diseases do mosquitos transmit
Malaria
Dengue
Yellow fever
Chikungunya
Zike
West nile fever
Hep C
What diseases do fleas transmit
Yersinia pestis (infected rodent–>flea—>human)
-bubonic plague
What symptoms occur during a transfusion acute intravascular hemolytic anemia and how fast does it occur
Fever
Hypotension
Flushing
Wheezing
Anxiety
Red colored urine and plasma (hemolysis)
Within minutes to hours
What symptoms occur during a transfusion delayed extravascular hemolytic anemia and how fast does it occur
Milder symptoms compared to acute
Occurs 3-10 days after tranfusion
What is a febrile reaction due to blood transfusion present as
Same symptoms as acute but no hypotension or blood in urine or plasma (no hemolysis)
What is an allergic reaction due to blood transfusion present as
Manifests as urticaria; rarely as anaphylaxis
what marker is essential to know if a tumor is benign or malignant
Monoclonality= Malignant
Polyclonality=Benign
If symptoms of malaria are severe then which strain caused it
Falciparum
Are lymphomas acute
NOOOO they happen overtime, but can be aggresive
What cells are infected by HIV and what are the differences
Macrophages- carry virus to lymph nodes, across blood brain barrier and can live silently within tissues for decades
Dendritic cells- carry virus to nodes but doesnt leave nodes
CD4 cells- Cells undergo lytic infection and have short lifespans
What do hypochronic RBCs in iron deficiency anemia look like
Cells with HUGE white cytoplasm
Go over in house HIV lectures
BRUHHHHHHHHHHHHHHHH
What kind of drug is Raltegravir
Integrase inhibitor
What kind of drug is Maraviroc
HIV gp120 inhibtior (viral attachment)
What kind of drug is Enfuvirtide
HIV gp41 inhibitor (viral fusion)
What is a typical presentation of someone with parvovirus B19 infection
Erythema infectiosum- cheeks look as if someone slapped them
what is the most common target for genetic alterations in human neoplasms
p53 gene (dna damage response)
-mutation can be inherited
What cancers are seen with APC gene mutations (tumor supressor)
Colon cancer and familial polyposis coli
what is TACO
Transfusion associated circulatory overload
What symptoms are seen in TACO
Pulmonary dyspnea, hypertension, pulmonary edema
What symptoms does Dengue fever cause
Myalgia
Fever
Erythematous maculopapular rash
Bone pain
(No joint pain)
GO OVER HIV GRAPH AND SCREENINGS
fuck med school
What does elevated serum LDH indicate
Cell breakdown
