Pathology Flashcards
Where is vWF stored?
W-P bodies of endothelium
What is stored in W-P bodies?
1) vWF
2) p-selectins
What does vWF bind?
SEC and platelets
What receptor doe platelets use to connect to fibrinogen?
GpIIb/IIIa
Petechiae occurs with (quantitative/qualitative) platelet disorders
quantitative (thrombocytopenia)
Autoimmune production of IgG against platelet antigens is called ____
ITP
In ITP, autoimmune antibodies are made in the _____. Red cells are consumed in the _______
Antibodies made in spleen
Red cells consumed in the spleen
Dx: Child with ITP
Rx: ________
usually self-limiting, possible platelet transfusion
Dx: Adult with chronic ITP, low platelet crisis
Rx: ______
IVIG
Dx: Adult with chronic ITP, low platelet crisis
Surgical option?
Splenectomy
Pathologic platelet micro-thrombi in small vessels is called _____
Microangiopathic Hemolytic Anemia
TTP is caused by a deficient enzyme _____
ADAMTS13
ADAMTS13 normally cleaves _____ multimers into monomers
vWF
The lack of ADAMTS13 in TTP is usually due to an underlying ________
autoimmune Dz
Hemolytic Uremic Syndorme primarily affects the _______ (organ)
kidney
The usual causative agent for HUS is ______
E. coli O157:H7
E. coli O157:H7 has the virulence factor ______ that damages renal endothelial cells
verotoxin
Name 2 Microangiopathic Hemolytic Anemias:
- TTP (thrombotic thrombocytopenic purpura)
2. HUS (Hemolytic Uremic Syndrome)
In Microangiopathic Hemolytic Anemias, bleed time (increases/decreases) and PT/PTT is ______
increases
PT/PTT is normal
How do you treat HUS and TTP?
- plasmapheresis
2. corticosteroids
In Bernard-Soulier, there is a genetic deficiency in ______
GP1b
In Bernard-Soulier, what function is impaired?
platelet adhesion
Platelets without GP1b can’t bind ____
vWF
Bernard-Soulier Syndrome
Blood smear of Bernard-Soulier Syndrome patient shows _______
enlarged platelets
In Glanzmann’s Thrombasthenia there is a genetic deficiency in ________
GIIb/IIIa
ASA blocks cyclooxygenase preventing platelets from making ____
TXA2
In vWF disease (PT/PTT) is increased
PTT
Which test is abnormal in vWF disease?
- ristocetin test
2. and PTT
What enzyme does coumadin block?
epoxide reductase
where is vitamin K generated?
gut bacteria
You can follow the extent of liver failure by measuring (PT/PTT)
PT
The best screening test for DIC is _______
elevated D dimer
A serine protease that converts plasminogen to plasmin
tPA
Dissolves fibrin clots:
1) cleaves fibrin
2) clears serum fibrinogen
3. destroys clot factors
plasmin
Lab findings: increased PT and PTT, increased bleed time, increased fibrinogen splits, no D-dimer
Dx: ?
overactive plasmin
Lines of Zahn: their presence implies thrombosis at a site of rapid blood flow that happened with the patient (alive/dead)
alive!
Coagulation can be kicked off by either ____ or ___
SEC or TF
Blocks platelet aggregation (TXA2/ PGI2)
PGI2 blocks
Promotes platelet aggregation (TXA2/ PGI2)
TXA2 promotes
on the surface of endothelial cells, serves as a cofactor for thrombin
TM: thrombomodulin
Name 3 endothelial damages that increase clot risk
- atherosclerosis
- vasculitis
- elevated homocystine
Vitamin k is required for which blood protein factors to work?
2, 7, 9, 10, C and S
Which has the shortest half-life? (2, 7, 9, 10, C or S)
C and S! hence the need for HEP therapy along with coumadin
Heparin binds and activates _____
ATIII
What are the 4 types of micorcytic anemias?
- sideroblastic
- thalasemia
- iron deficient
- anemia of chronic Dz
anemia, dysphagia w/ esophageal web, red beefy tounge is ____ _____ disease
plummer-vinson
what acute phase reactant sequesters Iron?
hepcidin
what does TIBC stand for?
total iron binding capacity
Hx: bone marrow suppression w chemo and anemia
how can you treat the anemia?
erythropoietin
Protoporphyrin is attached to iron in what cell compartment?
mitochondria
iron laden mitochondria occurs in _______ anemia
sideroblastic
alcoholism can lead to a ________ anemia
sideroblastic
intracellular protein that stores iron and releases it in a controlled fashion, commonl measured in labs
ferritin
decreased synthesis of globin chains is called ______
thalassemia
which leads to a worse thalasemia, cis or trans deletion?
cis
Where is the cis deletion thalassemia more commonly seen? (asia/africa)
asia
4 beta chains in a thalassemia is called Hb__
HbH
a tetramer of gamma chains in hemoglobin is called Hb_____
Hb barts
Beta thalassemia leads to (microcytic/macrocytic) anemia
microcytic
Massive erythroid hyperplasia such as in SS and beta thal leads to what appearance on skull radiograph?
“crew cut”
due to massive erythroid hyperplasia
why is a person w/ beta thalassemia at risk for hemochromotosis?
frequent transfusion
Electrophoresis: no HbA, increased HbA2 and HbF
Dx: ________
beta thalassemia major
Macrocytic anemia is usually due to a deficiency in ______ of _______
- folate
2. vitamin B12
Three diseases that can lead to a macrocytic anemia are:
- alcoholism
- liver disease
- chemo drugs: 5-FU, methotrexate and folate def.
R binder is produced by the ______, then complexed to vit. B12 for absorption
salivary glads
intrinsic factor that binds B12 is produced by the _____ cells o the stomach
parietal
B12 is stored in large volume in the _______ (organ)
liver
Pernicious anemia is due to _________ destroying the parietal cells of the stomach
autoimmune disease
With low serum B12, serum homocystine and _______ build up, leading to spinal cord damage
methylmalonic acid, can damage the spinal cord
Hemoglobinemia with hemoglobinuria and hemosiderinuria (intravascular/extravascular) destruction of RBCs
intravascular destruction
brown urine”, with chronic intravascular hemolysis is called _________
hemosiderinuria
what 3 tethering molecules are deficient in Hereditary spherocytosis?
- spectrin
- ankrin
- band 3.1
what virus infects erythrocyte pre-cursors?
Parvovirus B19
Treatment for Hereditary spherocytosis is _______
splenectomy
Howell-Jolly bodies indicate _______
splenic dysfunction, (such as post splenectomy)
Three risk factors for RBC sickling are:
- acidosis
- dehydration
- hypoxemia
Swollen hands and feet due to vaso-occlusive infarts of bones is called _______
dactylitis
in kids with SS, there is increased risk of infection with _____ _____
encapsulated bacteria
What lab test can allow identification of HbS?
Metabisulfite
What two molecules linked by GPI prevent complement form destroying Red cells?
- DAF: decay accelerating factor
2. MIRL: Membrane inhibitor of reactive lysis
Nightime shallow breathing and acidosis lead to activation of ______
complement
Lack of CD55 indicates the disease ______
CD55 is DAF,
indicates PNH: paroxysmal nocturnal hemoglobinuria
Patients with PNH, paroxysmal nocturnal hemoglobinuria have an increased risk for which caner?
AML, acute myeloid leukemia
G6PD deficiency renders cells susceptible to _______ _______
oxidative stress
Red cells as Bite cells are seen with _________
G6PD deficiency
Warm agglutinin is seem with (IgG/IgM) mediated immune hemolytic anemia
IgG
The two autoimmune disease, 1. ITP and 2. immune hemolytic anemia can be treated with ____
IV IG
RBC are bound by antibodies in the colder extremities with (IgG/IgM) immune hemolytic anemia
IgM
deficiency of all types of blood cells due to lack of blood cell progenitors is called _____ _____
aplastic anemia
A pathologic process that replaces bone marrow with fibrosis, tumor, granuloma etc, is called a ________ proces
myelophthisic process
Where is most of the marginated pool of neutrophils?
the lung endothelium
A left shift with immature neutrophile means a decrease in the _____ receptor or CD ____
Fc receptor = CD 16
Basophelia is associated with (ALL/APL/CML)
CML
Which cells are enlarged ‘monocytes’ in Mononucleosis?
CD8+ T-cells
Mononucleosis is a misnomer
Mononucleosis increases the risk for ______(organ) rupture
splenic
acute leukemia is a neoplastic proliferation of ______, greater than 20% in Bone marrow
blasts
You can specifically identify a Lymphoblast by the presence of (TdT/ MPO) in the nucleus
TDT
You can specifically identify a myeloidblast by the presence of (TdT/ MPO) as crystals of auer rods
MPO: myeloperoxidase crystalizes as auer rods
What are the two types of acute leukemia?
AML: Acute myeloid leukemia
ALL: Acute lymphoblastic leukemia
ALL is common in children with ______ syndrome after the age of 5
downs
The more common B-ALL usually has the translocation t(__:__)
t(9:22)
T-ALL usually presents as a T_____ (organ) mass in a T___ aged person
Thymic mass in a Teenaged
Hairy Cell Leukemia is a proliferation of (B cells/ T cells)
B cells
In CML, the predominant cell is the (RBC/ basophil/ neutrophil/ eosinophil/ monocyte/ megakaryocyte)
Basophil
CML is driven by the translocation t(__:__)
t(9:22)
A patient with CML can transform to (AML/ ALL)
ALL or AML! the mutation is in the Hematopoietic stem cell
- Polycythemia Vera,
- Essential Thrombocythemia and
- Myelofibrosis are driven by a mutation in ______
JAK2 kinase
Budd-Chiari syndrome with thrombosis in the portal vein can be cause by this blood cell proliferation _______
Polycythemia vera
In Polycythemia vera there are increased red cells AND ____ cells, which can degranulate in heat causing itching after bathing
mast cells
low SaO2, increased EPO, high red count (lung Dz/ Renal cell carcinoma/ polycythemia vera)
lung Dz
normal SaO2, increased EPO, high red count (lung Dz/ Renal cell carcinoma/ polycythemia vera)
Renal cell carcinoma
normal SaO2, low EPO, high red count (lung Dz/ Renal cell carcinoma/ polycythemia vera)
polycythemia vera
In myelofibrosis, megakaryocytes overproduce _____ resulting in fibrosis of the marrow space
PDGF
follicular lymphoma is characterized by the translocation [ t(14:18)/ t(11:14)/ t(8:14) ]
t(14:18)
Ig heavy chain: 14
Bcl-2: 18
over-expression of Bcl-2 will (promote/ inhibit) apoptosis
inhibit
Tingible body macrophages that eat up apoptotic B cells are (present/ absent) in follicular lymphona
absent! lack of B- cell apoptosis
mantle cell lymphoma is drive by translocation [ t(14:18)/ t(11:14)/ t(8:14) ]
t(11:14)
Ig heavy chain: 14
Cyclin D1: 11
Which B cell proliferation is associated with chronic inflammatory states like hashimotos and sjogrens (Follicular/ Mantle cell/ Marginal zone) Lymphome
Marginal zone lymphoma
Burkitt lymphoma is driven by [ t(14:18)/ t(11:14)/ t(8:14) ]
t(8:14)
Ig heavy chain: 14
c-myc: 8
Reed–Sternberg cells are CD__ and CD__ positive and not CD20+
CD15+ and CD30+
In Multiple myeloma, neoplastic plasma cells can activate the RANK receptor leading to ____-___ lesions on x-ray
punched out lesions
An M-spike on Serum protein electrophoresis may indicate the neoplastic proliferative disorder ______ _______
Multiple myeloma
Free light chain excreted in the urine with multiple myeloma is called ____-_____ protiens
Bence-Jones
Lytic bone lesions, hypercalcemia, M-spike, AL-amyloid and Bence-Jones protiens all indicate ______ _____
Multiple myeloma
An M-spike with IgM, absent lytic bone lesions, generalized LAD and increased bleeding are all characterisitcs of _____ _____
Waldenstrom macroglobulinemia
A Neoplastic proliferation with Birckeck, tennis racket granules and CD1a+ and S100+ cells is _______ _______
Langerhans Histiocytosis
Benign proliferation of langerhans cells, presents with pathologic fracture, skin not involved.
Eosinophilic granuloma, will have inflammatory eosinophils
What are the 3 Langerhans cell histiocytosis?
- Eosinophilic Granuloma (benign)
- Letter Siwe disease (malignant, >2y.o.)
- Hand-Schuller-Christian disease (malignant >3y.o.)
Are there blood vessels in the epidermis?
no, they stop at the dermis
Which layer of the epidermis has desmosoms that look like spinous processes?
the stratum spinosum
vitamin A derivatives are used to treat acne by (killing P. acnes/ reducing keratin production)
reducing keratin production
Benzoyl peroxidase is used to treat acne by (killing P. acnes/ reducing keratin production)
antimicrobial, kill P. acnes
Caused by excessive keratinocyte proliferation (psoriasis/ eczema)
psoriasis
usually on flexor surfaces (psoriasis/ eczema)
eczema = atopic dermatitis
usually on extensor surfaces (psoriasis/ eczema)
psoriasis
A type I hypersensitivity rxn associated with asthma and rhinitis (psoriasis/ eczema)
eczema = atopic dermatitis
Associated with HLA-C, lesions arise more frequently with trauma (psoriasis/ eczema)
psoriasis
diffuse epidermal hyperplasia is called ________
Acanthosis
perakeratosis, with excess keratin and retention of nucleii is (psoriasis/ eczema)
psoriasis
pinpoint bleeds with elongated dermal papilla (psoriasis/ eczema)
psoriasis
What are the p’s of lichen Planus?
pruritic planar polygonal purple papules
Autoimmune destruction of desmosomes leading to blisters (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Pemphigus Vulgaris
Autoimmune destruction of hemi-desmosomes at basement membrane (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Bullous Pemphigoid
IgG mediated (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Bullous Pemphigoid and Pemphigus Vulgaris
Bullae or blisters rupture more easily (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Pemphigus Vulgaris, blisters in stratum spinosum, not at basal layer
Mediated by IgA at tips of dermal papillae (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Dermatitis Herpetiformis
Strong association with celiac disease (Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Dermatitis Herpetiformis
Resolves with a gluten free diet(Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Dermatitis Herpetiformis
Most commonly seen with HSV infection (Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Erythema Multiforme
Multiple erythmentaous rashes with white middle targetoid appearance (Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Erythema Multiforme
Rank from least severe to most severe (Steven Johnson syndrome/ Erythema Multiforme/ Toxic epidermal necrolysis )
Erythema Multiforme, Steven Johnson syndrome, TEN. the are all the same, just different levels of involvement
A benign proliferation of squamous cells with a “ stuck on appearance” (Acanthosis Nigricans/ Seborrheic keratosis)
seborrheic keratosis
The Lesser-Trelat sign is when multiple of these arise sugesting an underlying GI carcinoma (Acanthosis Nigricans/ Seborrheic Keratosis/ Basal cell carcinoma)
seborrheic keratosis
Epidermal hyperplasia with darkening of the skin and a “velvet-like” appearance (Acanthosis Nigricans/ Seborrheic Dermatosis/ Basal cell carcinoma)
Acanthosis Nigricans
Associated with insulin resistance, diabetes, or underlying malignancy
(Acanthosis Nigricans/ Seborrheic Dermatosis/ Basal cell carcinoma)
Acanthosis Nigricans
Risk factors include prolonged exposure to sunlight, albinism and xeroderma pigmentosum (Acanthosis Nigricans/ Squamous cell carcinoma/ Basal cell carcinoma)
Basal cell carcinoma
appears as a nodule with a central area of ulceration and surrounded by telangectasia, especially on the upper lip. (Acanthosis Nigricans/ Squamous cell carcinoma/ Basal cell carcinoma)
Basal cell carcinoma
Risk factors include sunlight exposure, immunosupressive thereapy, arsnic poisoning and chronic inflammation. (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
Squamous cell carcinoma
Usually presents on the lower lip (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
Squamous cell carcinoma
Actinic keratosis is a precursor to (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
squamous cell carcinoma
Melanocytes are derived from which neural layer (ecterderm/ mesoder/ endoderm/ neural crest)
neural creast
vitiligo is (autoimmune/congenital)
autoimmune
Albinism is (autoimmune/congenital)
congenital
Due to defect in the enzyme tyrosinase (vitiligo/ albinism)
albinism
A freckle is darker than the surrounding sking due to a localized increase in (melanocytes/ melanosomes)
melanosomes, the number of melanocytes remains the same
A mask like hyperpigmentation of the cheeks is called _____
melasma
Which will have hair growing from it? (melanoma/ nevus)
Nevus, benign
Which has the worst prognosis? (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
melanoma
Squamous cell and basal are very fixable
Melanomas: Which two have a good prognosis (Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Lentigo Maligna Melanoma and
Superficial Spreading Melaona
Melanomas: which has an early vertical phase, therefore a poor prognosis?
(Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Nodular Melanoma
Melanomas: Which arises on palms and soles?
Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma
Acral Lentiginous Melanoma
Which is only along the dermal + epidermal junction with no invasion? (Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Lentigo Maligna Melanoma
Which only invades the dermis superficially and has a good prognosis? (Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Superficial spreading melanoma
Painless separation of the nail from the nailbed is called _______
onycholysis
Do petechia blanch with pressure? (yes/no)
no. They are bleeding into the skin
When RBC’s are of unequal size it is called _______
anisocytosis
the luebering rapoport pathway is used to make ______
2,3, BPG
which has HLA on their membrane (RBCs/ platelets)
Platelets have HLA
Decreased serum ferritin is diagnostic of ______ deficiency
iron deficiency
Increase in ferritin during inflammation is due to increased IL-__
IL-6
Ferritin is primarily in (serum/macrophages) while transferrin is in (serum/macrophages)
ferritin is in macrophages
transferrin is in serum
TIBC correlates with (ferritin/ serum transferrin)
serum transferin
decreased ferritin stores leads to (increased/decreased) transferrin synthesis
increased, therefore TIBC increases
The master iron regulator protein is called (hepcidin/ haptoglobin)
hepcidin
Which is directly absorbed in the duodenum? (Fe3+/Fe2+)
Which is transported by transferrin in the blood? (Fe3+/Fe2+)
Absorbed: Fe2+
Transported: Fe3+
In iron deficiency anemia, serum ferritin is (down/up)
In ACD serum ferritin is (down/up)
Iron deficiency, ferritin down
ACD, ferritin is up
An acute phase reactant that complexes with Hb to be degraded by macrophages is (hepcidin/ haptoglobin)
haptoglobin
Which indicates loss of function of splenic macrophages? (Heinz bodies/ Howell-Jolly bodies)
Howell-Jolly bodies
Which is caused by oxidative stress that damages hemoglobin? (Heinz bodies/ Howell-Jolly bodies)
Heinz bodies
Which is associated with SS disease? (Heinz bodies/ Howell-Jolly bodies)
Howell-Jolly bodies
Which is associated with G6PD deficiency? (Heinz bodies/ Howell-Jolly bodies)
Heinz bodies
Hereditary spherocytosis is inherited by (AD/AR/XR) pattern
AD
Sickle cell anemia is inherited by (AD/AR/XR) pattern
AR
G6PD deficiency is inherited by (AD/AR/XR) pattern
XR
“smudge cells” appear in (ALL/CLL/AML/CML/APL/HCL)
CLL: chronic lymphocytic leukemia
TRAP stain is positive in (ALL/CLL/AML/CML/APL/HCL)
HCL: Hairy cell leukemia
Auer rods are present in (ALL/CLL/AML/CML/APL/HCL)
APL: Acute promyelocytic anemia
The most common type of Hodgkin’s lymphoma is (Nodular sclerosis/ Mixed cellularity/ Lymphocyte depleted/ Lymphocyte rich)
Nodular sclerosis
The hodgkin’s lymphone most associated with EBV is (Nodular sclerosis/ Mixed cellularity/ Lymphocyte depleted/ Lymphocyte rich)
- mixed cellularity: 60-70%
- Lymphocyte rich: 40%
- Lymphocyte depleted: many
