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Pathology exam 4

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_______ kidney diseases include ectopic, horseshoe, agenisis, polycystic and cystic.
developmental
Nephrotic syndrome is characterized by ______ proteinuria, lipiduria and hypolbuminemia due to increased glomerular permeability resulting in severe edema.
massive >3.5 g/day
Nephritic syndrome is characterized by oliguria, ______ hematuria, uremia and ______
gross, Hypertension
Ag Ab complexes form due to a strep infection causing inflamation and increased numbers of mesangial cells and PMN's. This reaction compresses the GBM making it more permiable (nephritic syndrome) in this type of glomerulonephritis?
Acute
__________ glomerulonephritis is caused by injury resulting in increased glomerular pressure due to macrophage exudate and fibrin causing GBM rupture --> nephritic syndrome
Crescentic
Goodpasture's syndrome, SLE, IgA nephropathy and Wegener's granulomatosis are causes of _____ _______
Crescentic GN
Which type of GN is noninflammatory, caused by a thickening of the GBM "lumpy-bumpy" due to granular immune complex deposition --> nephrotic syndrome?
Membranous
________ is the most common cause of nephrotic syndrome in adults and is non responsive to therapy.
Membranous GN
Lipoid nephrosis is the most common cause of nephrotic syndrome in children and is caused by the fusion of podocyte ______.
processes
Type of syndrome? Acute and Crescentic GN and Goodpasture's
nephritic (oligo/hematuria, hypertension)
________ and ______ are nephrotic (massive proteinuria).
Membranous GN and Lipid nephrosis
IgA nephropathy, Lupus nephritis and Membranoproliferative GN are _______ syndromes.
mixed nephritic/nephrotic
This is the term used for multiple disease processes which: chronic --> end stage kidney disease, nephrotic/nephritic, do not respond to Tx and cause kidney shrinkage.
Chronic proliferative GN
Berger's nephropathy (IgA deposits) and Membranoproliferative GN (duplicate GBM = "tram track") are both examples of ____ _______ _______.
Chronic proliferative GN
Berger's nephropathy is the most common cause of GN in s T/F?
TRUE
Signs of end stage Gn include: uremia, renal/tubular atrophy, hyalinized _______.
glomeruli
Acute, cerescentic, chronic proliferative and membranous GN, lipoid nephrosis and end stage glomerulopathy are all _________ kidney diseases.
Immunological
_________ and _____ _____ are example of metabolic kidney diseases.
diabetes, urinary stones
Diabetes causes a thickening of the GBM which increases permiability (proteinuria) resulting in _______ syndrome.
nephrotic
Increase mesangial matrix --> nodular glomerulosclerosis (Kimmelsteil-Wilson disease) is due to this disease?
diabetes
Pathological complications due to diabetes include ischemia (atrophy, arteriosclerosis, papillary necrosis) and a predispostion to _____.
pyelonephritis (infection)
T/F kidney stones are usually found in the renal pelvis or bladder.
TRUE
Kidney stones hematuria and renal colic and are more commonly found in men/women?
MEN
Acute tubular necrosis, Nephroangiosclerosis and Hypertension are all forms of _______ kidney infections.
Circulatory
The most common cause of sudden onset renal failure is ATN due to MI's and _____.
shock (hypovolemic, toxic)
Which circulatory kidney disease is caused by atherosclerotic plaques which cause infarcts, scaring and fibrosis?
Nephroangiosclerosis
Hypertension stimulates arteriole contraction leading to thickening and hyalinization of the vessel walls causing reduced perfusion. _____ is released due to perfusion contributing to further hypertension.
Renin
Polynephritis and Cystitis are kidney diseases caused by _______.
Infection (UTI's)
Bacteria can reach the UT through ________ or through the urethra (ascending inf.)
blood (hematogenous)
Acute pyelonephritis affects the entire kidney while chronic forms abcesses usually durring childhood, pregnancy or in the elderly. T/F
False - chronic = entire kidney, acute = abscess
Women are affected with UTI's more than men. T/F
TRUE
Predisposing facors to UTI's include: stones, surgery, pregnancy, prostate hyperplasia and ______.
tumors
Cystitis, more common in women young and old, is an infection of the _____.
bladder
Cranberry juice is a must for newlyweds (Chase) to avoid ______ ______ caused by being "naughty". Infections are also related to pregnancy, urinary stones and cystoscopy.
honeymoon cystitis
Acute csytitis shows visible conjestion and mucosal hemorrhaging. T/F
TRUE
Chronic cystitis causes ulceration and thickening of the bladder. _____ _______ causes soft yellowish plaques.
renal malakoplakia
Renal cell carcinoma, Wilms tumor and Transitional cell carcinoma are kidney diseases due to _______.
neoplasms/cancer
Kidney neoplasms are usually benign. T/F
FALSE malignant (common in older men)
_______ tumors are the most common in the urinay tract.
Bladder
55 year old male presents with hematuria, flank pain and a palpable mass (yellow invading renal vein) with increased EPO levels. He was later diagnosed with ______.
renal cell carcinoma - characterized by clear polygonal cells
3 year old presents with a multinodular renal mass replacing his kidney. Surgery and chemo are the treatment for this tumor.
Wilms
This hematuria causing tumor of the urinary collecting duct is twice as common as renal cell carcinoma.
Transitional cell carcinoma
What two things make up the composition of ?
Plasma (55%) and Cells (45%)
What cells are contained in ?
Erythrocytes, leukocytes, platelets
What percent of plasma is water?
92%
What makes up plasma?
Water and proteins
Hematopoesis occurs in __________ in the liver, bone marrow, spleen and lymph nodes?
Fetus
Hematopoesis occurs in ____________ in the bone marrow (flat and long bones), the thymus and lymph nodes?
Children
Hematopoeisis occurs in ____________ in the bone marrow of flat bones only.
Adults
____________ hematopoesis subsides after birth and the __________ _______ remains the primary -forming organ.
Extramedullary; Bone Marrow
If hematopoetic bone marrow is destroyed, ___________ _________ may resume in the _______, _______, and _________ _______.
extramedullary hematopoiesis; spleen, liver, lymph nodes
Mature cells are descendants of pluripotent hematopoietic stem cells. Two major cell lineages are formed. What two are they and what do they give rise to?
Lymphoid and Myeloid lineages; Lymphoid -->B and T lymphocytes; Myeloid --> erythrocytes, megakaryocytes, neutrophils, monocytes, eosinophils, basophils.
Most growth factors (ie interluekins) are produced in the bone marrow. Which one is the exception and where is it formed?
Erythropoeitin, Kidney
This cell has an average life span of 120 days, biconcave disk shape, thin center.
Red Cell
RBC formation is stimulated by ________ which is produced in the kidney.
Erythropoeitin
RBC formation requires these three specific nutrients.
Iron, Folic Acid, Vitamin B12
This accounts for 90% of the dry weight of the RBC.
Hemoglobin
Adult hemoglobin has __ globin peptides, __ alpha and __ beta.
4; 2; 2
Which portion of hemoglobin is the binding portion?
Heme portion
Iron deficiency anemia is characterized by _______________.
Low hemoglobin values
What part of filtered hemoglobin is converted into blirubin?
Pyrrole Rings
When is excreted in the it is called _________ when it is exctreted in the feces it is called ___________.
urobilinogen; stercobilinogen
Iron is a component of the _______ moiety of hemoglobin.
Heme
Iron is absorbed in the intestine in the ______ form.
heme
Iron is absorbed via a receptor mechanism in its _______ form.
free
What enzyme transports iron?
transferrin
What molecule stores iron?
ferritin
What form is iron stored as in the spleen, liver, and bone marrow?
hemosiderin
60-80% of total body iron is stored in circulating red cells, the rest is bound to __________ and is stored as __________.
ferritin; hemosiderin
T or F: most of the iron released from destroyed red cells (in the spleen) is lost.
False; most is reutilized
decrease # of platelets
thrombocytopenia
decrease # of neutrophils
Neutropenia
Decrease # of leukocytes
leukopenia
decrease in all elements
pancytopenia
A reduction in Hb content in
anemia
This RBC disease is characterized by somnolence (inadequate brain O2), fatigue (inadequate O2), and paleness of skin
anemia
This type of anemia characterized by RBC's with normal color and size; could occur due to massive loss.
normocytic, normochromic anemia
This type of anemia characterized by small pale RBC's; could occur due to iron deficiency or thalassemaias.
Microcytic, hypochromic anemia
This type of anemia characterized by large, normal color RBC's; could occur due to Vitamin B12, folic acid deficiency
macrocytic, normochromic anemia
This type of anemia characterized by it's shape (poikilocytosis).
sickle cell anemia
variation in SIZE of RBC's
anisocytosis
Two types of aplastic anemia.
Idiopathic, Secondary
The prognosis for idiopathic aplastic anemia compared to secondary aplastic anemia is __________.
poorer (a word?)
This type of anemia is a generalized bone marrow failure (pancytopenia) and is usually accompanied by leukopenia and thrombocytopenia.
Aplastic anemia
The symptoms of this anemia are uncontrollable infections (leukopenia) and a bleeding tendency (thrombocytopenia).
Aplastic anemia
What is the only treatment for idiopathic aplastic anemia?
Bone marrow translplant
T or F: it is necessary to use Ab prophy for those with aplastic anemia.
TRUE
The causes of this disease are: metastatic carcinomas, diseminated granulomatous diseases, lymphomas, and other rare diseases (like gaucher's)
Myelophthisic anemia (aka replacement of marrow by neoplastic cells)
Inc. bleeding, Inc. infections and presence of a neoplastic population in bone marrow are characteristic of what disease.
Myelophthisic anemia (aka replacement of marrow by neoplastic cells)
What is the most common cause of anemia?
Iron deficiency anemia
The oral findings of this disease are pallor of soft palate, tongue, sublingual; atrophic tongue, glossodynia (buring sensation)
Iron deficiency anemia
This type of anemia: depletion of body iron stores, impaired hemoglobin synthesis
Iron deficiency anemia
In iron deficiency anemia the lab shows RBC's to be _________ and contain less ___________
small; hemoglobin (microcytic, hypochromic)
T or F: dental pts w/ Iron deficiency anemia usually require treatment modifications.
false
This syndrome is associated with dysphagia, anemia (ie iron deficiency), and increased risk of oral SCCA
Plummer-Vinson syndrome
T or F: there is no cure for iron deficiency anemia
false; just replace iron
Symptoms of this include: immature megaloblasts released into circulation; decreased RBC formation, Macrocytic anemia, hypersegmentation of neutrophils.
Vit. B12 deficiency (Cobalamin)
What does Vit. B12 bind to?
intrinsic factor
Lack of intrinsic factor causes what?
pernicious anemia
The most common cause of B12 deficiency anemia
pernicious anemia
T or f: vit b12 deficiency in pernicious anemia can be corrected with oral supplementation of vit b12
false; must be injected
What disease: spinal cord neuropathy associated, also could be associated with autoimmunity to parietal cells
pernicious anemia
What is the second cause of Vit B12 deficiency?
Malabsorbtion (stomach resection, celiac disease, crohn's disease)
What is the a common finding in both folic acid def. And vit b12 def.?
megaloblastic anemia
What tx modifications should you take when treating a pt. W/ vit b12 deficiency?
avoid nitrous (depletes b12 further)
the medications dilantin, methotrexate, and 5-FU (yes that's F. U.) are causes of what?
Folic Acid Deficiency
What symptom does vit b12 def have that folic acid def. Doesn't?
nuerologic abnormalities (the rest are essentially the same)
spina bifida, anencephaly are associated w/ what?
Folic Acid Deficiency
T or F: Folic acid sups can be taken orally.
true (vit b12 CAN'T)
Sickle cell anemia and thallassemia are what class of abnormal hematopoiesis?
hemoglobinopathies
Spherocytosis is what class of abnormal hematopoiesis?
Structural protein defects
What type of anemia: inherited, point mutation, Hb beta chain, N6 point mutation, result=abnormal hemoglobin
sickle cell anemia
T or F: those with the sickle cell train and those with the disease exhibit the same symptoms.
FALSE
Sickle cell anemia is most common among what population?
blacks
this disease characterized by abnormal Hemoglobin (HbS) that polymerizes at LOW O2 tension; induced by: fever, acidosis, dehydration, hypoxia.
sickle cell anemia
In Sickle cell anemia, RBC's are _________ sickled if they are deoxygenated, and they are _________ sickled if HbS polymerizes.
reversibly, irreversibly
The onset of symptoms for SC anemia first appear at what age?
one-two years (when HbF is usually replaced by HbA)
What disease? Marked underdevelopment, chronic anemia, jaundice, multiple organ infarcts, severe bone, spleen pain, intellectual impariment, recurrent infection, heart failure
sickle cell anemia
Dental findings of this disease include: spontaneous necrosis of multiple teeth, enlarged marrow spaces, "hair on end" in x-ray, delayed tooth eruption, osteomyelitis
sickle cell anemia
What is the prognosis for sickle cell anemia?
50% survival past age 50
How do you treat sickle cell
mainly avoid triggering factors, Ab prophy, hydroxy urea
what disease? Genetically inherited, 200 + mutations, quantitative defect of hemoglobin, decreased production of alpha chain, deficient or absent beta chain, end result=defect in AMOUNT of globin chain synthesized
Thalassemias (in general)
for the defective thalassemia gene, heterozygotes are thalassemai _________ and homozygotes are thal. ___________.
minor, major
Which thalassemia is more common, alpha or beta?
beta
What happens when deletion of all alpha globin transcripts occurs?
fetal demise
What disease: defect in synthesis of HbA that reduces the rate of globin chain synthesis. NO abnormal hemoglobin is produced (quantitative, not qualitative). Hypochromic, microcytic anemia results, crew-cut skull.
Thalassemias (in general)
In thalassemia minor, __ of four chains is missing, in thallassemia major ___ of four genes are missing.
one; two
Which thalassemia (alpha or beta) causes more severe anemia?
beta
What is the regular name of 'mediterranean anemia"
thalassemia
What disease? Mild symptoms, normal life expectancy, microcytic, hypocromic, (defective HbA synthesis, lower conc. Of HbA per RBC), no tx necessary
Thalassemia minor
which disease? Severe, high mortality, splenomegaly hemosiderosis, hepatomegaly, jaundice, Inc. hematopoeisis in flat bones, retarted growth, intellectual impairment, SOB (not "son of a bitch", "shortness of breath" chase), due to hea
thallassemia major
These dental findings are for what disease? Hair on end, chipmunk face, bimaxillary protrusion, teeth spacing, rarefaction of alveolar bone, widened marrow spaces, tooth discoloration (due to excess iron)
thallassemia major
T or F: there is an iron deficiency in thalassemia major.
false; an iron overload, side effect of hemolysis
What is the most common inherited disease of RBC's in caucasians?
hereditary spherocytosis
what disease: splenomegaly, jaundice, anemia, increased fragiligy of RBCs in hypotonic solution, gene mutation in structural membrane proteins (ankrin or spectrin).
hereditary spherocytosis
What are the diseases associated with Abnormal hematopoiesis?
Sickle cell anemia, thalassemia, hereditary spherocytosis
What are the diseases associated with decreased hematopoeisis?
aplastic anemia, replacement of marrow by neoplastic cells, iron deficiency anemia, vitamin B12 (perniciuos anemia) & folic acid deficiency, protein deficiency
Anemia due to loss will be ______chromic and _______cytic.
normo; normo
Immune hemolytic anemias can be caused by what 2 types of antigens?
autoantigen (anti-self) or alloantigen (anti-foreign)
what disease? Maternal abs cross placenta into fetal circulation, rh incompatibilities
erythroblastosis fetalis
what disease? attach to rbc surface, act as hapten, produciton of antibodies, rbc hemolysis
drug-related hemolytic anemia
what disease? Abs recognize self-antigens on rbc's, unknown cause
autoimmune hemolytic anemia
what disease is worldwide, the most common cause of hemolytic anemia?
anemia secondary to malaria
What is another name for polycythemia?
erythrocytosis
what disease? Hypertension, increased hemoglobin, elevated hematocrit, red complexion, headaches, splenomegaly, increase incidence towards clotting
Polycythemia Vera (aka primary)
How do you treat polycythemia?
phlebotomy, anti-leukemia
what disease? Non-neoplastic, high altitude causes it, anoxia, inc. erythropoeitin, compensatory increase in rbc
secondary polycythemia
What types of luekopenia are there?
Neutropenia, lyphopenia, selective lymphopenia
causes of ________ include: (cancer tx), radiation, aplastic anemia cyclic neutropenia, metastatic dx to bone marrow
leukopenia
A patient with neutropenia has a high risk of __________ infection, while a patient with lymphopenia has a high risk of _________________ infections
bacterial; bacterial, viral, fungal, parasitic
What types of WBC disorders are there? (4)
leukopenia, leukocytosis, leukemias and lymphomas, multiple myeloma
An increase in WBC's above 10,000 per mL is what?
leukocytosis
what type of leukocytosis occurs in reaction to an acute bacterial infection?
Granulocytosis (neutrophilia)
what type of leukocytosis occurs in reaction to allergies (hay fever, asthma) and skin disieases or parasitic infections
eosinophilic leukocytosis
What type of leukocytosis occurs in rxn to vial infections & chronic infections (tb), and some autoimmune disorders
lymphocytosis
Leukocytosis is often accompanied by ___________, a lymph node enlargement.
lymphadeopathy
What is a common symptom in all of the following: URTI, infectious MONO (hopefully paul doesn't have this, cuz I heard you get it from kissing lots of hot s), AIDS, cat-scratch disease, TB, metastatic cancers
lymphadeopathy
Malignant disease that involves wbc precursorsin bone marrow and associated w/ malignant wbc's in peripheral .
leukemia
malignant wbc's in lymph nodes or other solid organs or tissues
lymphoma
T or F: bone marrow biopsy must be performed to confirm diagnosis of leukemia
true
T or F: most cases of luekemias are caused by HTLV-1.
False; most cases are unknown
Why does anemia, recurrent infection and bleeding occur in leukemia, a WBC disorder?
because the malignant cells overtake the RBC, WBC and platelet precursors in the marrow
What is the most common eventual cause of in patients w/ leukemias?
uncontrollable infection
As a result of leukemia, what lab findings do you find in the PERIPHERAL ?
immature cells (due to pancytopenia in the marrow)
Flow cytometry and cluster of differentiation are lab procdures to detect what?
leukemias
What are the two major types of leukemias?
myeloid (granulocytic-monocytic) and lymphoid (lymphocytic)
What are the most common leukemias?
Acute LymphoBLASTIC leukemia (ALL); Acute Myelogenous leukemia (AML); Chronic myelogenous leukemia (CML); Chronic LymphoCYTIC leukemia (CLL)
What disease? Bimodal peaks--first in children under age 5, then in elderly; Most common malignant tumor in children under age 5; overall 20% of leukemias, most responsive to therapy
ALL
Symptoms of this disease: sudden onset, fatigue, fever, bleeding, flu-like symptoms, bone pain, lymphadenopathy (generalized), splenomegaly, hepatomegaly, child age less than 5
ALL
What lab findings in ALL?
elev. WBC, immature WBC's (blasts) in and marrow
T or F: with Tx, ALL never comes back
false; 50-75% remission (w/out chemo, 3-6 mos)
What are the 3 steps of chemotherapy?
Induction, Cosolidation, and Maintenance
Which step of chemo is to achieve a complete remission?
consolidation
What type of chemo is used to prepare a patient for bone marrow transplant?
conditioning chemo
What disease? Massive infiltration of the bone marrow with immature myeolblasts (immune cells) with immature blasts that spill over into peripheral , fatigue, pallor, diffuse BOGGY gingival enlargement, peak age 60
AML
What is the most common leukemia overall?
AML
_________ leukemia occurs in middle aged men showing signs of pancytopenia, spleno-hepato-megaly and lymphadenopathy. TRAP- tartrate resistant acid phosphatase- stains for the diseased cells.
Hairy cell
What disease? adults, fatigue, infections, splenomegaly, thrombosis, anorexia
CML
What do 90% of patients with CML have in common?
the Philadelphia Chromosome
50% of pts w/ CML progress to an _________ phase while the other 50% progress straight to a ________ ________.
accelerated(before blast crisis); blast crisis
Tx for CML:
Bone marrow translplant; Gleevec (tyrosine kinase inhibitor)
What disease? B-cell proliferation --> slow progressive autoimmune hemolytic anemia.
CLL
What disease? Old ppl (>50 yrs), cells indistinguishable from normal mature lymphocytes, slowly progressive, progonsis 7-10 yrs, chemo not indicated (NO Tx)
CLL
T or F: All lymphomas are malignant
TRUE
When a lymphoma is spread to other organs in the body (not lymphoid tissue) what is this spread called?
extranodal spread of lymphoma
What are the 2 main categories of lymphomas?
Hodgkins Disease & Non-Hodgkin's Lymphoma (NHL--not to be confused with the National Hockey League--who cares about hockey anyway?)
T or F: lymph nodes are always involved in lymphomas
FALSE
Immunohistochemistry and Flow cytometry are methods to detect what disease?
lymphoma
HIV & EBV, environmental causes, gene translocations, antigenic stimulation, are supposed etiology factors for what disease?
Lymphomas
Which NHL is the most common and is slow-growing (indolent)?
Follicular lymphoma
Follicular, Mantle Cell, Diffuse large B-cell, Burkitt's, and Extranodal NK/T cell lymphomas are what class of lymphoma?
Non-Hodgkin lymphomas (NHL's)
Which NHL appears to be related to EBV, associated w/ children, max. & man. Involvement, and an endemic african variant (also curable)?
Burkitt lymphoma
Which NHL can appear as an ulcer on the hard palate?
extranodal NK/T cell lymphoma
What disease? Bimodal distribution (25 & 55), Reed-Sternberg Cells (bilobed/multilobed nucleus w/ clear zones), involves a single node or chain of nodes, possible EBV related.
Hodgkins disease
What is the most important in determining prognosis for hodgkins disease?
staging (lower stages have best prognosis)
Matching Types of Hodgkins: 1. lymphocyte predominant 2. Lymphocyte depleted 3. Mixxed cellularity 4. nodular sclerosing ---- a. most common b. worst prognosis c. best prognosis d. common in elderly
1.c 2.b 3.d 4.a
Dental diagnosis of a cervical lymphadenopathy could mean presence of __________.
a lymphoma (or a hicky on chase's neck)
Dental diagnosis of intraoral bleeding, fever, malaise, tiredness, boggy gumms (generalized!!!!) could mean presence of __________.
myeloid leukemias
Dental diagnosis of intraoral boggy red soft tissue mass (Localized!!!!) could mean presence of ____________
a lymphoma
Dental diagnosis of a destructive bone lesion could mean presence of ________
a lymphoma
What complications of leukemia/lymphoma tx are there in dentistry?
oral mucositis, viral, bacterial, fungal infextions,
As a long term side effect of tx for lymphomas/leukemias, lichenoid lesions (in the mouth) suggest onset of ______________.
Graft v. Host disease
T or F: some multiple myelomas are benign.
FALSE
What WBC disorder involves malignant plasma cells?
Multiple myeloma
What disease? s (45-50 yrs), NOT in children, bone destruction, punched out radiolucencies
Multiple myeloma
What disease? Bone pain & destruction, hypercalcemia, metastatic calcification, renal damage, bone fractures
Multiple myeloma
What disease? Lab findings: monoclonal Ig spike, Bence-Jones Protein, Abnormal plasma cells, amyloid deposition, light chain proteinuria
multiple myeloma
What other variants of Multiple myeloma are there?
solitary myeloma, extramedullary plasmacytoma (soft tissue), monoclonal gammopathy of undetermined significance (MGUS)
What disease? Bone destruction, punched out radiolucencies, soft tissue lesions, children.
Langerhans cell histiocytosis
Which laboratory evaluation? In vivo assessment of platelet response to vascular injury, normal=several minutes, elevated=defect in platelet # or function
Bleeding Time
What is the normal platelet count?
150,000-450,000 platelets/mm3
What lab evaluation? Time needed for plasma to clot in presence of exogenous source of thromboplastin and calcium
PT (prothrombin time)
What lab evaluation? Tests adequacy of Extrinsic and common coagulation pathways
PT (prothrombin time)
Deficiency of factors V, VII, or X, prothrombin, or fibrinogen is a diagnosis of ____________ Prothrombin time (PT)
increased
What lab evaluation? Time required for plasma to clot
Partial thromboplastin time (PTT)
Assesses the integrity of the INTRINSIC and common clotting pathways
PTT
Increased PTT is shown with a defect of factors __,___,___,___,___,___,prothrombin, fibrinogen or acquired antibody that interferes with intrinsic pathway.
V,VIII (especially, emphasized in class!), IX, X, XI, XII
What is the international normalized ratio (INR)?
PT/reference lab control plasma (normal = 1.0, elevated # equals clotting problem)
What are 3 mechanisms of vascular bleeding disorders?
Trauma, Vessel wall weakness, immune mechanisms
Scurvy, Amyloid, chronic systemic steroid use, inherited conditions infectious causes, and aging can all cause what type of bleeding disorder?
Vascular disorder
What bleeding disorder? Autoimmune vasculitidies, allergic rxns.
Immune mechanisms
What bleeding disorder? Petechiae & eccymoses, skin, mucous membranes, after minor trauma/spontaneously; normal platelet count, PT & PTT bleeding time
Vessel wall weakness & immune-related bleeding disorder
This type of bleeding disorder can be qualitative or quantitative
Platelet-related bleeding disorders
What platelet level characterizes thrombocytopenia?
<100,000/mm3
What occurs when platelet levels reach below 20,000/mm3?
Sponaneous bleeding, elevated bleeding time
Petechiae, and ecchymoses (skin+mucous membranes) are signs of what bleeding disorder?
thrombocytopenia
The etiology of this disease is: Decreased production of platelets, Increased loss of platelets
thrombocytopenia
Aplastic anemia, leukemia, infecious agents (ie rubella), and (interfere w/ megakaryocytes) are contributory to ______________.
decreased platelet production
Autoimmune destruction, SLE, HIV,Heparin-induced thrombocytopenia, all contribute to _________.
increased platelet loss (via DESTRUCTION)
Hypersplenism leads to _____________.
increased platelet loss (via REMOVAL)
DIC (yes, that's right), (Shock, infection tumors, injured endothelial cells, activation of coagulation system, widespread thrombi, fibrinolysis, bleeding) all contribute to __________.
Increased platelet loss (via CONSUMPTION)
Two forms of Qualitative platelet bleeding disorders:
Acquired; congenital
Aspirin, NSAIDS, ESRD (accumulation of metabolites) and Plavix all lead to ___________
qualitative (acquired) platelet related bleeding disorder
________ clotting factor deficiencies are caused by inadequte production, excessive consumption, inhibition by anticoagulants.
Acquired
Why does chronic liver disease cause inadequate production of clotting factors?
All clotting factors (except von willenbrand's factor) are produced in the liver, thus, liver disease will cause a decrease in these
Why is chronic liver disease also known as hypofibrinogenemia
Because fibrinogen is the most abundant clotting factor produced in the liver
What does the anticoagulant coumadin do?
prevents vitamin K utilization in the liver (decreases clotting factors)
DIC is responsible for what clotting factor disease?
Acquired, excessive consumption of platelets
What are 2 anticoagulants that inhibit coagulation factor production?
Heparin and Warfarin
Hemophilia A & B are what type of bleeding disorders?
congenital clotting factor defeiciency
What clotting factor is congenitally deficient in Hemophilia A?
Factor VIII
What disease? Defeiciency of Factor VIII, -linked recessive, males, females carriers, 1:5000 males, 30% are new mutations
Hemophilia A
T or F: Hemophilia is an "all or nothing" disease that has no variance in severity.
FALSE
What percent of Factor VIII must be present (at least) to not have severe bleeding?
1%
T or F: petechiae are present in Hemophilia A
FALSE
If a patient presents with normal bleeding time and normal prothrombin time(PT), normal platelet count, and prolonged partial thromboplastin time (PTT), what disease does he have?
Hemophilia A
What pathway does the PTT test for?
Intrinsic pathway
What is the leading cause of for hemophiliacs?
AIDS
What is the treatment for hemophilia A?
recombinant factor VIII
What disease? AKA Christmas Disease, Deficiency of Factor IX, X-linked recessive
Hemophilia B

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