Pathology exam 4
Terms
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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