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Sys Path Old Exam 1
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123
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06/08/2011

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Term
Complications from Mitral Valve Prolapse
Definition
Stroke, Arrhythmias, Infective endocarditis
Term
Three thins a person may undergo one hour before ventricular fibrilllation?
Definition
tachycardia, hyperventilation, hypotension. (Also, substernal chest pain radiating to the neck. Occlusion and narrowing of a. of the heart)
Term
afebrile
Definition
having no fever
Term
Lab tests of SGOT (AST) show:
Definition
to detect liver damage
Term
Lab tests of CK-MB show:
Definition
Heart dying
Term
Lab tests of LDH show:
Definition
general ACUTE or CHRONIC tissue damage
Term
Lab tests of CRP show:
Definition
ACUTE Infection or acute inflammation
Term
Lab tests of SGPT (ALT) show:
Definition
to detect liver damage
Term
hemarthroses
Definition
bleeding into the joint (associated with elevated PTT) and lack of factor VIII
Term
A prolonged/ increased prothrombin (PT) time is associated with:
Definition
Malabsorption
Cirrhosis
Vitamin K deficiency
Bile duct obstruction
Term
Liver makes all clotting factors except:
Definition
von Willebrand factor
Term
Prolonged Bleeding Time is associated with:
Definition
Platelet dysfunction; vascular dysfuction (deficiency of von Willebrand factor)
Term
Late cyanosis Congenital heart disease (Left to Right shunts)
Definition
VSD
ASD
Complete endocardial cushion defect
Sinus venosus
Patent foramen ovale
Patent ductus arteriosus (PDA)
Term
Cyanotic Congenital Heart Disease (Right to Left shunts)
Definition
Tetralogy of Fallot
Transposition of the great vessels
Persistent truncus arteriosus
Term
Neoplastic disorder that INVOLVES LYMPH NODES:
Definition
Hodgkin disease
(has RS cells- "owl eyes")
Term
Non-Hodgkin Lymphomas presentation:
Definition
Usually discovered in only chain of nodes
Involves lymph nodes or lymphoid tissue in other parts of the body
No RS cells
Term
Burkitt Lymphoma presentation- histologically:
Definition
Large lymphocytes with lipid-containing vacuoles "STARRY SKY PATTERN"
Term
Multiple Myeloma keywords
Definition
excess abnormal immunoglobulins + infiltration of various plasma cells
Bone pain + hypercalcemia
urine Bence-Jones proteins
Cytoplasmic inclusions called Russell bodies
"Punched out" defects on x-ray
Amyloid deposition in various soft tissue
Term
Physical examination for Folic acid deficiency include:
Definition
Months to develop
Skin pallor
Angular cheilitis
Generalized depapillation of his tongue
Macrocytic anemia
Term
Physical examination for B12 deficiency include:
Definition
Years to develop
Burning sensation of tongue, lips, taste disturbances
Patchy or diffuse erythema, or pallor
Surface atrophy, lobulation
Skin may be yellow-gray
Term
Signs of acute leukemia
Definition
Anemia
Bleeding disorder
Gingival enlargement
Term
Chronic Leukemia Features what signs and symptoms:
Definition
Fatigure
Weakness
Weight loss
Anorexia Hepato and splenomegaly lymphadenopathy
Thrombocytopenia
Term
Red Blood Cells
Definition
(N = 3.5-6x106/ml)
Term
Reticulocyte count
Definition
0.5-1.5%
Term
Hemoglobin
Definition
12-18g/dl
Term
Hematocrit
Definition
packed RBCs/ volume (N = 37-54%)
Term
Mean Corpuscular (Cell) Volume
Definition
82-96 mm3
Term
Red Blood Cell Distribution Width
Definition
11.5-14.5
Term
children 6 mo-5 years: Hb
Definition
< 11g/dL
Term
children 5–11 years: Hb
Definition
< 11.5 g/dL
Term
adult males: Hb
Definition
< 13 g/dL
Term
non-pregnant females: Hb
Definition
< 12g/dL
Term
pregnant females: Hb
Definition
< 11g/dL
Term
severe anemia: Hb
Definition
< 7.0 g/dL
Term
Iron Deficiency Anemia
Definition
Inadequate intake of iron results in insufficient hemoglobin synthesis and hypochromic and microcytic red cells
Term
Anemia of Chronic Disease
Definition
Caused by production of inflammatory cytokines, which cause iron to be sequestered in macrophages, resulting in an anemia that is usually normochromic and normocytic
Term
Megaloblastic Anemia
Definition
Caused by deficiencies of folate or vitamin B12, results in enlarged abnormal hematopoietic precursors (megaloblasts) in the bone marrow, ineffective hematopoiesis, and (in most cases) pancytopenia
Term
Aplastic Anemia
Definition
Caused by bone marrow failure (hypocellularity) due to diverse causes, including exposures to toxins and radiation, idiosyncratic reactions to drugs and viruses, and inherited defects in DNA repair and the enzyme telomerase
Term
Myelophthisic Anemia- what is it? What does it lead to?
Definition
Caused by replacement of the bone marrow by infiltrative processes such as metastatic carcinoma and granulomatous disease. Leads to the release of early erythroid and granulocytic precursors (leukoerythroblastosis) and the appearance of tear-drop red cells in the peripheral blood
Term
Chronic blood loss results in
Definition
Iron Def Anemia
Term
Dec production of RBC due to
Definition
B12 or folic acid def
Thalassemia
Aplastic anemia
Term
Increased destruction of RBC in what diseases?
Definition
Thalassemia
Sickle cell anemia
Immunohemolytic
Term
Increased destruction of RBC in what diseases?
Definition
Thalassemia
Sickle cell anemia
Immunohemolytic
Term
Signs and symptoms of iron def:
Definition
“Burning” tongue
Patchy or diffuse erythema
Atrophy of filiform papillae
Taste alteration
Fissuring
All of the above may be due to candidosis (candiasis)!
Term
Signs and symptoms of Thalassemia
Definition
Frontal bossing
“hair-on-end” appearance of calvaria
Enlarged jaws
‘Honeycomb’ bone pattern
Term
Features of aplastic anemia (the numbers for granulocytes, platelets, reticulocytes / uL)
Definition

  • G <500
  • P <20, 000
  • R <10, 000

Term
granulocytes/mL
Definition
Normal = 3-8,000
Term
platelets/mL
Definition
Normal = 150-450,000
Term
reticulocytes/mL
Definition
Normal = 50,000
Term
Signs and Symptoms of Aplastic Anemia
Definition
pallor
gingival bleeding, mucosal petechiae, purpura, ecchymoses
gingival enlargement
mucosal ulcers
Term
Hemolytic anemia: Common features
Definition
Fatigue, weakness, headache, rapid pulse
Jaundice
Splenomegaly (early on)
Do not confuse hemolytic anemia with hypercarotinemia (too many carots, shrimp in diet) – sclerae not yellow!
Term
Signs and symptoms of sickle cell anemia
Definition

  • Reduced trabeculation 
  • “Hair-on-end” appearance of calvaria 
  • Delayed dental eruption
  •  Dental hypoplasia 
  • Ishemic necrosis 
  • Neuropathy

Term
Manifestation of Sickle Cell Crisis
Definition
Capillary blockage to ischemia, infarction to severe pain
Hemolysis: Anemia, jaundice
+/- fever
Term
What kind of anemia is Hereditary spherocytosis
Definition
Hemolytic; sphere shaped instead of looking donut shaped
Term
"Hair on end" that is seen in thalassemia major is due to:
Definition
Bone marrow expansion
Term
RBC appearance in thalassemia minor:
Definition
Target cells
microcytic
hypochromic
Term
Most frequent site for atherosclerosis?
Definition
Ab aorta
Term
calcific aortic valve stenosis most commonly develops due to:
Definition
AGING!

Other predisposing conditions include calcification of a congenital bicuspid aortic valve and acute rheumatic fever
Term
Most common cause of mitral valve stenosis?
Definition
Chronic rheumatic heart disease
Term
stenosis leads to a
Definition
heart murmur
Term
insufficiency leads to
Definition
regurgitation
Term
Three key facts with mitral valve stenosis?
Definition
No pathologic change in the LV
You can have stenosis with or without insufficiency
Fish mouth appearance of the defected valve
Term
Most common cause of aortic valve stenosis if pt is older than 50?
Definition
Degeneration w/ old age
Term
Most common cause of aortic valve stenosis if pt is younger than 50?
Definition
Congenital bicuspid aortic valve
Term
Two very important facts about AORTIC VALVE STENOSIS
Definition
Rheumatic heart diseae is the second most common cause
There IS A PATHOLOGIC CHANGE in the LEFT VENTRICLE
Term
von Willebrand disease characteristics:
Definition

  • The platelet count is normal 
  • Men and women are affected equally 
  • There is impaired platelet ADHESION 
  • Factor VIII levels are decreased 
  • The clinical presentation is variable 
  • Prolonged bleeding time

Term
Deficiency of vWF leads to:
Definition
failure of platelet adhesion
Term
The hallmark of left side failure is:
Definition
pulmonary edema
Term
Girl has track marks, fever, leukocytosis. What is true about staph infections in this setting?
Definition
Staph infection can infect normal valves
Tricuspid valve is usually damaged
Right heart failure can occur
Term
Chronic lymphocytic leukemia is often the consequence of:
Definition
small B cell lymphoma
Term
What is more common: B or T cell non-hodgkin lymphomas?
Definition
B
Term
In the absence of systemic signs and symptoms, bilateral non-painful symmetric enlargement of the palatine tonsils in a 24 yr old is MOST consistent with?
Definition
Reactive lymphoid hyperplasia
Term
Complications resulting from an acute MI include:
Definition
Arrhythmias
Mitral valve prolapse
Pericarditis
Mural thrombosis
Hemopericardium
Term
Severe with right leg. Angina, atrial fibrillation, and history of intermittent claudication in the right left:
Definition
Thromboembolism arising from the left atrium
Thrombotic occlusion of the femoral artery with underlying atheroma
Term
Three things associated with thrombocytopenia?
Definition
Leukemia
Atherosclerosis
B12/folate def.
Term
Intrinsic pathway affects?
Definition
PTT
Term
Intrinsic pathway includes:
Definition
factors 12, 11, 9, 8
Term
Extrinsic pathway affects?
Definition
PT
Term
Extrinsic pathway includes?
Definition
Factor 7
Term
Common Pathway affects?
Definition
both Intrinsic and Extrinsic
Factors 10, 5, 2 make fibrin
Term
Which factors are vitamin-K dependent?
Definition
Factors 2, 7, 9, 10 are Vitamin-K dependent and are the most sensitive to liver disease
Term
Platelet count and bleeding time are affected by?
Definition
Platelets and vasculature
Term
What three things get affected by coagulation factors?
Definition
PT, PTT, factor assays
Term
Quantitative platelet problems
Definition
(thrombocytopenia)  low CBC and prolonged bleeding time
Term
Qualitative platelet problems
Definition
(abnormal function)  normal CBC but prolonged bleeding time
Term
Vessel disorders
Definition
(affecting the endothelium)  normal CBC but prolonged bleeding time
Term
Intrinsic Pathway dysfunctions
Definition
(12, 11, 9, 8)  prolonged PTT
Term
Extrinsic Pathway dysfunctions
Definition
(Factor 7 only)  prolonged PT
Term
Common Pathway dysfunctions
Definition
(10, 5, 2)  prolonged PTT and PT
Term
Bleeding disorders- Hereditary etiologies (3) for vascular disorders:
Definition
Ehlers-Danlos Syndrome = problems w/ CT
• Henoch-Scholein Purpura = problems w/ vasculitis
• Hereditary Hemorrhagic Telangiectasia
Term
Thrombocytopenias: two clinical presentations:
Definition
superficial bleeding (petechiae, purpura)
• low platelet count and prolonged bleeding time
Term
thrombocytopenia- surgical bleeding if counts are
Definition
< 50,000
Term
thrombocytopenia- spontaneous bleeding if counts are
Definition
< 20,000
Term
thrombocytopenia- cerebral bleeding if counts are
Definition
< 10,000
Term
Bernard-Soulier Syndrome
Definition
GP1B deficiency  adhesion problems
Term
Glanzmann’s Thrombasthenia
Definition
GP2B deficiency  aggregation problems
Term
Clinical presentation (2) of thrombocytopathies
Definition
superficial bleeding (petechiae, purpura)
• normal platelet count and prolonged bleeding time
Term
4 Clinical presentations of hemophilias?
Definition
deep bleeding (ecchymosis, hemarthrosis, GI, GU)
• normal platelet count
• prolonged bleeding time
• prolonged PT and PTT
Term
Acquired etiologies for Hemophilias?
Definition
Vitamin-K deficiency = affects Factors 2, 7, 9, 10
• Liver disease = affects all coagulation factors – EXCEPT VWF – and 2, 7, 9, 10 are affected first,
• Kidney disease
• D.I.C.
Term
VWD Disease
Definition
(autosomal dominant)
- affects VWF and Factor-8
- prolonged PT and PTT
- treat w/ Cryoprecipitate
Term
Hemophilia-A
Definition
(autosomal recessive)
- affects Factor-8
- prolonged PTT
- treat w/ Factor-8 concentrate
Term
Hemophilia-B
Definition
(autosomal recessive)
- affects Factor-9
- prolonged PTT
- treat w/ Factor-9 concentrate
Term
anything that induces more RBC / WBC/ protein production ? (4)
Definition
- Thrombocytosis = platelets
- Polycythemia Vera = RBC’s
- Leukemias = WBC’s
- Myultiple myeloma = proteins
Term
Neutrophilia
Definition
(normal = 1800 – 8000; 50%) = an increase in circulating neutrophils in response to bacterial infections or tissue necrosis
Term
Eosinophilia
Definition
(normal = 0 – 500; 3%) = an increase in circulating eosinophils in response to parasitic infections, allergies, Hodgkin’s Disease, vasculitis
Term
Monocytosis
Definition
(normal = 0 – 800; 10%) = an increase in circulating monocytes in response to granulomatous diseases (like TB, SLE, SBE)
Term
Acute Lymphocytic Leukemia
Definition
- indicates that the mutation occurred early in the differentiation process  poor prognosis
- the abnormal cells in circulation are blastic (immature), poorly-differentiated
Term
Chronic Lymphocytic Leukemia
Definition
- indicates that the mutation occurred later in the differentiation process  better prognosis
- the abnormal cells in circulation may be well-differentiated but they are NOT normal
Term
3 types of lymphomas?
Definition
• Non-Hodgkin’s Lymphomas
• Hodgkin’s Disease
• Burkitt’s Lymphoma
Term
Myelodysplastic Syndrome
Definition
Myelodysplastic Syndrome can be mild and remain in the non-proliferative state
 immature cells are dysplastic = un-organized appearance but not yet malignant
 decreased cell count = cytopenia
• Myelodysplastic Syndrome can become aggressive and start rapidly proliferating into myeloproliferative syndromes
Term
Myeloprolferative Syndromes
Definition
Acute / Chronic Myelogenous Leukemia = if neutrophils predominate
• Acute / Chronic Monocytic Leukemia = if monocytes predominate
• Polycythemia Vera = if RBC’s predominate
Term
What would a bone marrow biopsy show in leukemias?
Definition
The bone marrow is so hypercellular that there just isn’t enough room for normal cells to proliferate
1) bone marrow expansion to bone pain
2) pancytopenia to makes you more susceptible to infections & bleeding
3) extramedullary hematopoiesis to hepatomegaly & splenomegaly
Term
Lymphocytic leukemia
Definition
lymphadenopathy occurs only in the early stages when the there is “homing” of cells back to the lymph nodes
Term
Myelogenous leukemia
Definition
lymphadenopathy occurs only in the late stages when there is diffuse organ infiltration
Term
hepatomegaly & splenomegaly due to several reasons:
Definition
1) extramedullary hematopoiesis
2) organ infiltration
3) horning back to the spleen for antigen presentation (lymphocytic type of leukemia only) -- recall that the spleen is also a lymphoid organ
4) liver infiltration  liver dysfunction  cant make coagulation proteins
Term
ACUTE LYMPHOCYTIC LEUKEMIA (ALL)
Definition
affects the B-cells = large & poorly-differentiated (blastic)
- involves deregulation of the C-myc oncogene
- pancytopenia  infections (↓ neutrophils); bleeding (↓platelets); anemia (↓RBC’s)
- early lymphadenopathy
- hepatosplenomegaly
Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
Definition
most likely to affect males 50+ yrs old
- affects the B-cells = small & well-differentiated
- CLL w/ aggressive mutations has a tendency to become aggressive ALL
- CLL patients who don’t have the aggressive mutation for that tendency will have a long survival
- there is some genetic predispotion
- pancytopenia  infections (↓ neutrophils); bleeding (↓platelets); anemia (↓RBC’s)
- early lymphadenopathy
- hepatosplenomegaly
Term
ACUTE MYELOGENOUS LEUKEMIA (AML)
Definition
- AML tends to occur within 2 yrs after exposure to radiation, chemotherapy, benzene
- AML can arise from Myelodysplastic Syndrome (non-proliferative)
- AML has the same clinical presentation as ALL
Same: Both ALL and AML have blastic cells w/ large cytoplasm b/c they are both acute leukemias involving immature cells
Different: Special tests and staining techniques must be done to be able to differentiate ALL blast cells -vs- AML blast cells
AML cells have AUER RODS and tests positive for Myeloperoxidase
Term
CHRONIC MYELOGENOUS LEUKEMIA (CML)
Definition
involves a translocation b/t chromosomes #9 & 22 = Philadelphia chromosome
- affects the granulocytes & thrombocytosis
- extreme splenomegaly
- elevated peripheral blood counts
- erythroid cell line can either be normal or low
- myelofibrosis of the bone marrow in severe end-stage cases  pancytopenia
- CML left un-treated has a tendency to become aggressive AML
Term
Leukemias can go from
Definition
chronic to acute (BUT NEVER THE OTHER WAY AROUND)
Term
Clinical presentation of lymphomas?
Definition
• painless lymphadenopathy
• fever, fatigue, weight loss, night sweats
• when the advanced disease becomes Leukemia, it has the same clinical presentation as Leukemia because IT IS Leukemia
Term
BURKITT’S LYMPHOMA
Definition
characterized by mandibular tumors (on top of the bone, not within the bone)
- STARRY SKY histology = characterized by tumor cells located against a background of many lymphocytes -- like Van Goh’s famous painting
- involves deregulation of the c-myc oncogene
- involves translocation between chromosomes #8 & #14
- this disease is considered a high-grade  aggressive & rapidly-proliferating
- therefore it is very curable in patients w/ good immune systems
Term
HODGKIN’s DISEASE
Definition
- bi-modal incidence = most common in 20’s (curable) and 50’s (poor prognosis)
- diagnosis is based on histology from lymph node & bone marrow biopsies LP
- REED-STERNBERG CELLS = owl’s eyes appearance due to the 2 big nuclei
- Pel-Epstein fever (fluctuating), prutitis (itchiness)
- otherwise, same exact clinical presentation as Lymphoma
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