Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
169 Cards in this Set
- Front
- Back
where is 1/3 of platelet pool stored
|
spleen
|
|
vWF receptor
fibrinogen receptor |
vWF - GpIb
fibrinogen - GpIIb/IIIa |
|
differentiate what's contained in dense granules and a-granules of platelets
|
dense granules (ADP and calcium)
a-granules (vWF and fibrinogen) |
|
differentiate where monocytes and macrophages are located
|
monocyte - blood
macrophage - tissue |
|
what activates macrophages
|
IFN-gamma
|
|
associated with large, kidney-shaped nucleus with "frosted-glass" cytoplasm
|
monocyte
|
|
associated with bilobate nucleus with large eosinphilic granules of uniform size
|
eosinphils
|
|
defends against helminthic infections via major basic protein
|
eosinophils
|
|
causes of eosinophilia
|
NAACP:
neoplastic, asthma, allergic process, collagen vascular disease, parasites (invasive helminths) |
|
mediates allergic reaction
|
basophils
mast cells |
|
can bind the Fc portion of IgE while mediating allergic reaction
|
mast cell
|
|
this drug prevents mast cell degranulation (used to treat asthma)
|
cromyolyn sodium
|
|
B cell markers
|
CD19 and CD20
|
|
associated with being a plasma cell neoplasm
|
multiple myeloma
|
|
where do B and T cells mature respectively - both are made in the bone marrow
|
B - bone marrow
T -thymus |
|
T cell markers
|
CD3, CD4, CD8
|
|
express MHC II and FcR on surface, main inducers of primary antibody response
|
dendritic cells
|
|
what type of Ig are anti-A/B vs. Anti-Rh
|
Anti-A/B - IgM
Anti-Rh - IgG (can cross the placenta) |
|
what causes hemolytic disease of the newborn
|
Rh- mother exposed to Rh+ fetus, future pregnancies the mother will have anti-Rh IgG that crosses placenta and causes hemolysis
|
|
thromboplastin = tissue factor = ?
|
Factor VII
|
|
deficiency of factor VIII
|
hemophilia A
|
|
deficiency of factor IX
|
hemophilia B
|
|
decreased synthesis of factors II, VII, IX, X, protein C and S
|
vitamin K deficiency
|
|
inhibits thrombin (II) and factors IX, X, XI, and XII
|
antithrombin
|
|
effects of bradykinin
|
vasodilation
increases permeability pain |
|
what activates vitamin K
|
epoxide reductase
|
|
what does warfarin inhibit
|
epoxide reductase (activates vitamin K)
|
|
what drug activates antithrombin
|
heparin
|
|
mutation that produces a factor V resistant to activated protein C's inhibition
|
factor V leidin
|
|
cleaves plasminogen into plasmin
*plasmin ultimately cleaves fibrin mesh |
tissue plasminogen activator
*thrombolytic |
|
two cofactors for protein C
|
protein S
thrombomodulin |
|
what does protein C do
|
cleaves and inactivates factors V and VIII
|
|
what binds to exposed collagen upon endothelial damage and starts the platelet plus formation
|
vWF
|
|
what do platelets release once they bind to vWF via GpIb receptor
|
release ADP and Ca - both are necessary for coagulation cascade)
|
|
two function of ADP once released from platelets
|
1. helps platelets adhere to endothelium
2. induces GpIIb/IIIa receptor expression at platelet surface |
|
function of fibrinogen
|
binds GpIIb/IIIa receptors and links platelets
|
|
thromboxane A2, PGI2 and NO in coagulation
|
TXA2 (pro-aggregation factor) - decreases blood flow and increases platelet aggregation
PGI2 and NO (anti-aggregation factor) - increases blood flow and decreases platelet aggregation |
|
inhibits cyclooxygenase and therefore thromboxane A2 synthesis
|
aspirin
|
|
inhibit ADP-induced expression of GPIIb/IIIa
|
ticlopidine
clopidogrel |
|
inhibits GpIIb/IIIa directly
|
abciximab
|
|
acanthocyte (spiny RBC)
|
liver disease
abetalipoproteinemia |
|
associated with basophilic stippling
|
TAIL:
thalassemias anemia of chronic disease iron deficiency lead poisoning |
|
associated with bite cells
|
G6PD deficiency
|
|
associated with schistocytes (helmet cells)
|
DIC
TTP/HUS traumatic hemolysis |
|
associated with teardrop cells
|
bone marrow infiltration
|
|
associated with target cells
|
HbC
asplenia liver disease thalassemia |
|
associated with Heinz bodies
|
a-thalassemia
G6PD deficiency |
|
mechanism of Heinz body
|
oxidation of iron from ferrous to ferric form leads to denatures hemoglobin precipitation and damage to RBC membrane - eventually forms bite cells in G6PD deficiency
|
|
associated with Howell-Jolly bodies
|
functional hyposplenia or asplenia
*basophilic nuclear remnants |
|
thalassmia associated with Asian and African populations
|
a-thalassemia
|
|
Hb Barts (y-4)
|
deletion of 4 a-globulin genes, incompatible with life
|
|
deletion of 3 a-globulin genes
|
HbH disease (B-4)
|
|
differentiate the defect found in a-thalassemia vs. B-thalassemia (biochemically)
|
a-thalassemia - a-globulin gene mutations (deletions)
B-thalassemia - point mutations in splicing sites and promoter sequences |
|
diagnosis confirmed by increased HbA2 (>3.5%)
|
B-thalassemia minor (heterozygote)
|
|
associated with severe anemia requiring blood transfusions and marrow expansion (crew cut on skull x-ray) leading to skeletal deformities
|
B-thalassemia major (homozygote)
|
|
associated with decreased ferrochelatase and ALA dehydratase along with inhibits rRNA degradation leading to aggregation of ribosomes
|
lead poisoning
|
|
associated with a X-linked defect in aminolevulinic acid synthase gene
|
siderblastic anemia
|
|
what causes the ringed siderblasts in siderblastic anemia
|
iron-laden mitochondria
|
|
associated with increased iron and ferritin, with normal TIBC
|
sideroblastic anemia
|
|
treatment of sideroblastic anemia
|
pyridoxine therapy (B6)
|
|
causes of siderblastic anemia (defect in heme synthesis)
|
ALA synthase deficiency
lead alcohol B6 deficiency |
|
causes of microcytic, hypochromic anemia (MCV < 80)
|
iron deficiency
a-thalassemia B-thalassemia lead poisoning siderblastic anemia |
|
characteristics of lead poisoning
|
LEAD:
1. lead lines on gingivae and long bones 2. encephalopathy and erythrocyte basophilic stippling 3. abdominal colic and anemia 4. drops - wrist and foot drop |
|
causes megaloblastic macrocytoic anemia (MCV > 100)
|
folate deficiency
B12 deficiency |
|
associated with increased homocysteine but normal methylmalonic acid
|
folate deficiency
|
|
associated with both increased homocysteine and methylmalonic acid
|
B12 deficiency
|
|
causes nonmegaloblastic macrocytic anemias
|
1. liver disease
2. alcoholism (can occur in absence of folate/B12 deficiency) 3. reticulocytosis (bigger than mature RBCs) 4. congenital deficiencies of purine or pyrimidine synthesis |
|
two general categories for nomocytic, normochromic anemia
|
nonhemolytic vs. hemolytic
|
|
associated with decreased heptoglobin, increased LDH, hemoglobin in urine
|
intravascular hemolysis
|
|
associated with increased LDH, increased unconjugated bilirubin, causes include: hereditary spherocytosis, G6PD deficiency, sickle cell anemia
|
extravascular hemolysis
|
|
nonhemolytic causes of normocytic, normochromic anemia
|
anemia of chronic disease
aplastic anemia kidney disease |
|
this disorder is associated with inflammation which increases hepcidin which decreases release of iron from macrophages
|
anemia of chronic disease
|
|
associated with decreased iron and TIBC, but increased ferritin
|
anemia of chronic disease
|
|
pancytopenia characterized by severe anemia, neutropenia, and thrombocytopenia
|
aplastic anemia
|
|
defect in proteins interacting with RBC membrane skeleton and plasme membrane, causes round RBC with no central pallor
|
hereditary spherocytosis
|
|
associated with increased MCHC and RDW
|
hereditary spherocytosis
|
|
lab test used for hereditary spherocytosis
|
positive osmotic fragility test
|
|
x-linked deficiency that causes decreased glutathione and increases RBC susceptiblity to oxidant stress
|
G6PD deficiency
|
|
associated with blood smear showing RBC with Heinz bodies and bite cells
|
G6PD deficiency
|
|
this autosomal recessive defect in RBCs is associated with hemolytic anemia in a newborn
|
pyruvate kinase deficiency
|
|
two diseases associated with "crew cut" on skull x-ray due to marrow expansion
|
sickle cell disease
B-thalassemia major |
|
what precipitates sickling in sickle cell anemia
|
low oxygen content of dehydration
|
|
what is associated with resistance to malaria
|
heterozygous sickle cell trait
|
|
complications associated with sickle cell disease
|
1. aplastic crisis (parvovirus infection)
2. autosplenectomy - increased risk of infection with encapsulated organisms 3. salmonella osteomyelitis 4. vaso-occlusive (painful crisis) 5. renal papillary necrosis 6. splenic sequuestration crisis |
|
treatment of sickle cell crisis
|
hydroxyurea (increases HbF)
bone marrow transplantation |
|
diseases associated with intrinsic hemolytic normocytic anemia
|
hereditary spherocytosis
G6PD deficiency pyruvate kinase deficiency sickle cell anemia HbC defect PNH |
|
diseases associated with extrinsic hemolytic normocytic anemia
|
autoimmune hemolytic anemia
microangiopathic anemia macroangiopathic anemia malaria and Babesia infections |
|
warm vs. cold agglutinin autoimmune hemolytic anemia
|
warm - IgG, chronic anemia seen in SLE, CLL, or drugs
cold - IgM, acute anemia triggered by cold, mycoplasma pneumoniae or infectious mononucleosis |
|
anti-Ig antibody added to patient's RBCs agglutinate if RBCs are coated with the Ig
|
direct coombs' test
|
|
normal RBCs added to patient's serum agglutinate if serum has anti-RBC surface Ig
|
indirect coombs' test
|
|
RBCs are damaged when passing through obstructed or narrowed vessel lumen; seen in DIC, TTP/HUS, SLE, and malignant hypertension
|
microangiopathic anemia
|
|
prosthetic heart valves and aortic stenosis may cause hemolytic anemia secondary to mechanical destruction
|
macroangiopathic anemia
|
|
iron storage protein of body
|
ferritin
|
|
transports iron in blood
|
transferrin
|
|
what is the serum iron, transferrin (TIBC), ferritin, and % saturation in iron deficiency
|
*decreased serum iron
increased TIBC decreased ferritin decreased % saturation |
|
what is the serum iron, transferrin (TIBC), ferritin, and % saturation in chronic disease
|
decreased serum iron
decreased TIBC *increased ferritin normal % saturation -TIBC is decreased because body stores iron in cells to prevent pathogens from acquiring circulating iron |
|
what is the serum iron, transferrin (TIBC), ferritin, and % saturation in hemochromatosis
|
*increased serum iron
decreased TIBC increased ferritin increased % saturation |
|
what is the serum iron, transferrin (TIBC), ferritin, and % saturation in pregnancy/oral contraceptives
|
normal serum iron
*increased TIBC normal ferritin decreased % saturation |
|
what is the serum iron, transferrin (TIBC), ferritin, and % saturation in lead poisoning
|
increased serum iron
decreased TIBC normal ferritin increased % saturation |
|
serum iron and TIBC are usually inverse of each other, which two disorders is this not the case
|
anemia of chronic dieases - decreased TIBC to keep iron away from pathogens
pregnancy/oral contraceptive use - directly increases TIBC regardless of what serum iron levels are |
|
associated with protoporphyrin accumulation in blood due to ferrochelatase and ALA dehydratase enzymes being affected
|
lead poisoning
|
|
associated with porphobilinogen deaminase defect leading to painful abdomen, red win-colored urine, polyneuropathy, and psychological disturbances
|
acute intermittent porphyria
|
|
treatment of acute intermittent porphyria
|
glucose and heme, these inhibit ALA synthase
|
|
associated with uroporphyrinogen decarboxylase defect leading to blistering cutaneous photosensitivity and tea-colored urine
|
porphyria cutanea tarda
|
|
most common porphyria
|
porphyria cutanea tarda (uroporphyrinogen decarboxylase defect)
|
|
what do the heme concentrations do to ALA synthase activity
|
inverse of each other:
decreased heme causes increased ALA synthase activity |
|
tests function of all factors except VII and XIII
|
PTT (intrinsic pathway)
|
|
associated with hemarthroses, easy bruising, and increased PTT
|
hemophilia A or B
|
|
PT and PTT of vitamin K deficiency
|
both are prolonged
|
|
symptoms of platelet abnormalities
|
mucous membrane bleeding
epistaxis petechiae/purpura increased bleeding time |
|
increased bleeding time due to defect in platelet-to-collagen adhesion
|
decreased GpIb - Bernard-Soulier disease
|
|
increased bleeding time due to defect in platelet-to-platelet aggregation
|
decreased GpIIb/IIIa - Glanzmann's thrombasthenia
|
|
decreased platelet survival due to anti-GpIIb/IIIa antibodies causing peripheral platelet destruction and megakaryocytosis
|
idiopathic thrombocytopenic purpura (ITP)
|
|
decreased platelet survival due to deficiency of ADAMTS 13 (vWF metalloprotease - decreased degredation of vWF multimers)
|
thrombotic thrombocytopenic purpura (TTP)
|
|
associated with increased large vWF multimers, increased platelet aggregations and thrombosis
|
TTP
|
|
symptoms associated with TTP
|
neurologic symptoms
renal symptoms fever thrombocytopenia microangiopathic hemolytic anemia |
|
most common inherited bleeding disorder, autosomal dominant
|
von Willibrand's disease
|
|
treatment of vWD
|
desmopressin which release vWF stores in endothelium
|
|
platelet count, bleeding time, PT and PTT seen in vWD
|
normal platelet count
increased bleeding time normal PT normal or slightly increased PTT (depends on severity due to carrying of factor VIII) |
|
platelet count, bleeding time, PT and PTT seen in DIC
|
decreased platelet count
increased bleeding time increased PT and PTT |
|
associated with schistocytes, increased fibrin split products (D-dimers), and decreased factors V and VIII
|
DIC
|
|
most common cause of inherited hypercoagulability
|
factor V Leiden
|
|
decreased ability to inactivate factors V and VIII
|
protein C or S deficiency
|
|
associated with increased risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
|
protein C or S deficiency
|
|
hereditary thrombosis syndromes leading to hypercoagulability
|
Factor V Leiden
prothrombin gene mutation ATIII deficiency Protein C or S deficiency |
|
prothrombin gene mutation
|
mutation in 3' untranslated region
associated with venous clots |
|
differentiate leukemia vs. lymphoma
|
leukemia - widespread involvement of bone marrow with tumor cells usually found in peripheral blood
lymphoma - discrete tumor masses arising from lymph nodes |
|
leukemoid reaction
|
increased WBC count with left shift (80% bands) and increased leukocyte alkaline phosphatase, due to infection
|
|
what determines prognosis of Hodgkin's lymphoma
|
increased lymphocytes with decreased Reed-Sternberg cells
|
|
cells markers from RS cells
|
CD30 and CD15 B-cell origin
|
|
which type of Hodgkin's lymphoma is associated with lacunar cells and primarily affects young adults
|
nodular sclerosing
|
|
what is the lymphocyte-to-RS cell ratio in nodular sclerosing Hodgkin's lymphoma
|
increased lymphocytes/RS cells
good prognosis |
|
4 B-cell non-Hodgkin's lymphomas
|
Burkitt's lymphoma
diffuse large B-cell lymphoma mantle cell lymphoma follicular lymphoma |
|
translocation associated with Burkitt's lymphoma
|
t(8:14) c-myc gene translocation
|
|
lymphoma with starry-sky appearance (sheets of lymphocytes with interspersed macrophages), associated with EBV
|
Burkitt's lymphoma
|
|
most common adult non-hodgkin lymphoma
|
diffuse large B-cell lymphoma
|
|
translocation in mantle cell lymphoma
|
t(11:14)
|
|
what corresponds with poor prognosis in mantle cell lymphoma
|
CD5+
|
|
translocation in follicular lymphoma
|
t(14:18) bcl-2 expression
|
|
what does bcl-2 expression in follicular lymphoma do
|
inhibits apoptosis
|
|
two types of T cell non-Hodgkin lymphomas
|
adult T-cell lymphoma
mycosis fungoides/sezary syndrome |
|
Lymphoma associated with HTLV-1 infection, adults present with cutaneous lesions
|
adult T- cell lymphoma
|
|
lymphoma associated with cutaneous patches/nodules that is CD4 positive
|
mycosis fungoides/sezary syndrome
|
|
most common primary tumor arising within the bone in the elderly
|
multiple myeloma
|
|
what is associated with: primary amyloidosis, punched-out lytic bone lesions, M spike on protein electrophoresis (gamma area), Bence Jones protein (Ig light chains in urine), and routleaux formation
|
Multiple Myeloma
|
|
other problems associated with MM
|
hypercalcemia
renal insufficiency anemia bone lytic lesions |
|
distinguish which types of Ig are overproduces in MM vs. Waldenstrom's macroglobulinemia
|
MM - IgG or IgA
Waldenstrom's - IgM |
|
disease with hyperviscosity symptoms and M spike due to increased IgM production
|
Waldenstrom's macroglobinulinemia
|
|
leukemia associated with being TdT+ and CALLA+
|
ALL
|
|
marker of pre-T and pre-B cells
|
TdT+
|
|
leukemia seen in older individual (>60 years) and is associated with warm antibody autoimmune hemolytic anemia
|
CLL (chronic lymphocytic leukemia)
|
|
leukemia associated with staining TRAP (tartrate-resistant acid phosphatase) positive
|
hairy cell leukemia
|
|
leukemia associated with Auer rods
|
acute myelogenous leukemia
|
|
which form of AML responds to all-trans retinoic acid (vitamin A)
|
M3 subtype
|
|
leukemia associated with t(9:22) bcr-abl Philadelphia chromosome
|
CML
|
|
what happens when CLL has a "blast crisis"
|
transforms into AML or ALL
|
|
leukemia associated with very low leukocyte alkaline phosphatase
|
CML
|
|
which leukemia responds to imatinib
|
CML
anti-bcr-abl antibody |
|
leukemia associated with non-antibody-producing B cells and therefore these patients are susceptible to infection
|
CLL
|
|
peroxidase-positive cytoplasmic inclusions in granulocytes and myeloblasts of AML
|
Auer Rods
|
|
translocation seen in M3 type of AML
|
t(15:17)
|
|
proliferative disorder of defective S-100 and CD1a expressing cells with Birbeck granules (tennis rackets)
|
langerhans cell histiocytosis
|
|
which chronic myeloproliferative disorders are associated with JAK2 mutatioins
|
polycythemia vera
essential thrombocytosis myelofibrosis |
|
what is JAK2 involved in
|
hematopoietic growth factor signaling, mutations are implicated in meyloproliferative disorders other than CML
|
|
associated with decreased RBCs with teardrop shaped myeloid cells (including RBCs)
|
myelofibrosis
|
|
plasma volume, RBC mass, O2 saturation, and erythropoietin seen in relative polycythemia
|
decreased plasma volume
normal RBC mass, O2 saturation, and EPO |
|
plasma volume, RBC mass, O2 saturation, and erythropoietin seen in appropriate absolute polycythemia
|
normal plasma volume
increased RBC mass decreased O2 saturation increased EPO |
|
plasma volume, RBC mass, O2 saturation, and erythropoietin seen in inappropriate absolute polycythemia
|
normal plasma volume
increased RBC mass normal O2 saturation increased EPO |
|
plasma volume, RBC mass, O2 saturation, and erythropoietin seen in polycythemia rubra vera
|
increased plasma volume
increased RBC mass normal O2 saturation decreased EPO |