Final Exam Flashcards
What is myeloid tissue?
Hematopoietic tissue including
- Blood cell precursors located in the bone marrow
- Blood cells (erythrocytes, platelets, granulocytes, monocytes) located in the blood
What is lymphoid tissue?
Tissue located in the lymphoid organs and can also be located in the blood (lymphocytes)
e.g. thymus, lymph nodes, spleen, MALT
What is hematopoiesis
the process by which all of your blood cells are formed, develop and mature into their final adult types.
– myeloid cells produced from multipotential cells in bone marrow
– 4 cell lines, each line undergoes differentiation into committed cells
•erythroid/megakaryocytic
•macrophage/granulocytic
• eosinophilic
• mast cell/basophilic
What is lymphopoiesis?
generation of lymphocytes - happens in lymphoid tissue
Plasma vs. Serum
- plasma is the fluid component of blood (need to anticoagulate)
- serum is the fluid remaining if blood is allowed to clot
What is CBC?
Complete blood test - used to evaluate features of blood
number of each blood cell type
What is a differential test?
- Differential refers to the different types of wbc present
What is a blood smear?
- done to assess the morphology (shape) of blood cells
- can often be used as a diagnosis
What is anemia?
Anemia is a decrease in the red blood cell mass (and
therefore Hb) present in blood;
signs and symptoms are due to decreased ability of blood to carry oxygen
How is anemia diagnosed?
How is the diagnosis different in men and women?
Usually assessed by looking at the Hb level
– anemia in male is defined as Hb <130
– anemia in female is defined as Hb < 115
How are anemias classified?
– morphology
- etiology
What are the 3 major groups of anemias based on morphology ?
- hypochromic microcytic (the presence of small, often less coloured, red blood cells in a peripheral blood smear)
- normochromic normocytic
Red blood cells appear to be normal, just fewer - macrocytic (A macrocytic class of anemia is an anemia (defined as blood with an insufficient concentration of hemoglobin) in which the red blood cells (erythrocytes) are larger than their normal volume.)
What are the mechanisms of anemia?
- Loss of blood (chronic and acute)
- Destruction of red cells (hemolytic anemias)
- Decreased red blood cell production
What are the inherited destructive abnormal anemias?
- abnormal membrane [Hereditary spherocytosis]
- abnormal enzymes [G6PD deficiency]
- abnormal Hb [Hemoglobinopathies]
What is the acquired destructive abnormal anemias?
abnormal membrane [Paroxysmal nocturnal Hburia]
premature death and impaired production of blood cells
What are the usual causes of destruction of normal RBC?
• immune, mechanical, infections (malaria), chemical
What are the pathogeneses and names of decreased red blood cell production anemias?
– bone marrow failure [aplastic anemia, infiltration of bone marrow]
– defective DNA synthesis [megaloblastic anemias]
– defective HB synthesis [iron deficiency anemia]
What are the signs and symptoms of anemia?
– pale skin and mucosa (conjunctiva) – fatigue, dyspnea on exertion – brittle nails – koilonychia (spoon shaped nails) – headache, decreased vision, drowsiness
What are the causes of iron deficiency anemia?
- decreased intake
- decreased absorption
- increased loss
- increased requirements
What is aplastic anemia?
Anemia characterized by loss of multipotent myeloid stem cells in the bone marrow with resulting pancytopenia
– decreased production of all three cellular components of the blood (red cells, platelets, and granulocytes)
What are the causes of aplastic anemia?
– primary (Idiopathic)
– secondary
• cytotoxic drugs, radiation, viral infection
How do you diagnose aplastic anemia?
– pancytopenia (CBC)
– bone marrow showing few blood-forming cells
What are the clinical signs of aplastic anemia?
- ↓ wbc results in infections
- ↓ platelets results in bleeding
- ↓ Hb results in anemia
What is megaloblastic anemia?
Anemias characterized by abnormal blood cell precursors
(megaloblasts) in the bone marrow and their descendants
in blood
What are the two major causes of megaloblastic anemias?
B12 deficiency and folate deficiency
– defective nuclear maturation due to impaired DNA synthesis
– causes production of abnormal cells
– abnormal cells are destroyed resulting in anemia
How do you diagnose megaloblastic anemias?
- peripheral blood (hypersegmented PMN, macrocytosis)
- bone marrow biopsy and aspiration (hypercellular and
megaloblasts)
What is Vitamins B12 and how does a deficiency occur?
– B12 is an essential nutrient because not able to make in body
– B12 is important because required for DNA synthesis
– Absorption of B12 requires intrinsic factor (made in stomach)
– impaired absorption
– decreased intake
– increased requirement
What is pernicious anemia?
- megaloblastic anemia due to lack of intrinsic factor with resulting
vitamin B12 deficiency - lack of intrisic factor is due to autoimmune destruction of parietal
cells in stomach
What is the role of folate and what are the causes of deficiency?
- important because cofactor in DNA synthesis
– causes of folate deficiency • decreased intake • impaired absorption • increased loss • increased requirements
What are hemolytic anemias?
Hemolysis is the destruction of red cells
What are the causes of hemolysis?
– intracorpuscular defect (problem with rbc itself)
– extracorpuscular defect (problem with body)
What are the extracorpuscular defects involved in hemolytic anemias?
- autoimmune destruction
- mechanical destruction
- infection
What are the intracorpuscular defects involved in hemolytic anemias?
- membrane defect [hereditary spherocytosis]
- Hb defect [sickle cell anemia]
- enzyme defect [G6PD defect]
What is sickle cell anemia?
• Inherited disorder of Hb due to a defect in β globin gene
– defective gene substitutes neutral aa for an acidic aa [HbS (α2S2)]
– in low oxygen, abnormal Hb polymerizes making red cells sickle
– results in chronic hemolytic anemia and small vessel occlusion
What is sickle disease vs. sickle trait?
Sickle disease
– homozygote for defective gene (>80% HbS)
– sickle cells in blood and sickling crises
Sickle Trait
– heterozygote for defective gene (<40% HbS)
– asymptomatic
What are Thalassemias?
Group of disorders caused by defect in genes coding for
Hb chains resulting in decreased synthesis of normal Hb
How are Thalassemias classified?
on basis of type of chain affected
– β Thalassemia [defective synthesis of β chain]
– α Thalassemia [defective synthesis of α chain]
How do Thalassemias present clinically?
- depends on number of genes affected and nature of defective gene
– anemia is a hypochromic microcytic anemia
– in severe disease get iron overload
What are the different types of α thalassemias?
– silent carrier state (single gene deletion, asymptomatic)
– α thalassemia trait (2 gene deletion, mild anemia)
– hemoglobin H (HbH) moderate severe anemia)
• HbH is tetramer of β globins, doesn’t carry oxygen
– hydrops fetalis (4 gene deletion, intra-uterine death)
How do β thalassemias present?
– thalassemia minor (mild disease, asymptomatic)
– thalassemia intermedia (moderate severe anemia, no transfusion required)
– thalassemia major (severe disease, requires transfusions)
What is hereditary spherocytosis?
Group of disorders characterized by inherited defect of structural proteins in red cell
– defective red cells unable to maintain shape and form spherocytes
– the defective red cells are removed prematurely by the spleen
How are most hereditary spherocytosis inherited?
autosomal dominant inheritance
How does the severity of spherocytosis vary and present?
– chronic anemia of mild to moderate severity
– spherocytes present in blood
– hemolytic crises
– mild jaundice, splenomegaly
What are immune hemolytic anemias?
Group of anemias resulting from destruction of red cells by
antibodies
Incompatible blood transfusion (previously discussed)
• Hemolytic Disease of the Newborn (previously discussed)
What is autoimmune hemolytic anemia?
– body identifies antigen on own rbc as foreign, makes antibody
– Ag-AB complexes form, complement activation, rbc destruction
What are the causes of autoimmune hemolytic anemia?
• idiopathic
• secondary
– lymphoproliferative disease
– drugs
What is polycythemia?
An increase in number of red cells (and Hb)
What are the causes of polycythemia?
– primary
– secondary
• living at high altitude
• chronic lung disease
What is polycythemia rubra vera?
– neoplastic disease of red cell precursors in the bone marrow
– increased viscosity of blood
– insensitive to erythropoeitin
– require blood to be removed
What are leukopenia?
Abnormal decrease in number of white blood cells
What are the 2 types of leukopenia?
Neutropenia and lymphopenia
What is • neutropenia?
– decreased number of neutrophils
– susceptible to bacterial infections
What are the causes of neutropenia?
- drugs
- chemicals
- radiation
- aplastic anemia
What is lymphopenia?
– decreased number of lymphocytes
What is leukocytosis?
Abnormal increase in number of white blood cells
What is neutrophilic leukocytosis?
(increased numbers of neutrophils)
– bacterial infections
– toxic changes, shift to the left
What is eosinophilia?
(increased number of eosinophils)
– parasites, allergies, drug reactions
What is lymphocytosis?
(increased number of lymphocytes)
– viral infections
– chronic infections
What are leukemias?
– malignant diseases of leukocytes
– malignant proliferation of blood cell precursors in the bone marrow
malignant cells also enter peripheral blood
What are lymphomas?
– malignant diseases of lymphocytes
– malignant proliferation of cells in lymphoid tissue (usually lymph nodes)
– Hodgkin’s vs. Non Hodgkin’s Lymphoma
What is Hodgkin’s lymphoma vs. non-Hodgkin’s lymphoma?
Hodgkin’s disease (lymphoma) = a form of malignant disease that is pathologically distinct from other lymphomas; Diagnosis = binucleated and multinucleated cells;
– group of lymphomas characterized by presence of Reed Sternberg cell (binucleated and multinucleated cells)
– bimodal age distribution (25 yo, 55 yo)
– staging important re prognosis
– chemotherapy very effective
Non-hodgkin’s lymphoma = OTHER LYMPHOMAS
– diverse group of diseases
– classification (based on histological features) of low, intermediate and
high grade
What are leukemias?
is a group of cancers that usually begin in the bone marrow and result in high numbers of abnormal white blood cells
What are the different types of leukemias?
– acute vs. chronic – myeloid vs. lymphoid (depends on what type of cell is being affected) – ALL – AML – CLL – CML
What is ALL?
(leukemia)
– most common type in children (most common malignancy in child < 5 yo)
– treatment with chemotherapy (remission in most patients, cure in 50%)
– very treatable
What is AML?
(leukemia) – most common acute leukemia in adults – several categories (FAB Classification) – treatment with bone marrow transplant – classified by genetics
What is CML?
(leukemia)
– chronic phase, accelerated phase, blast crisis
– chronic phase, blast crisis
– characterized by Philadelphia chromosome (2 genes fused together)
– can progress to AML
What is CLL?
(leukemia)
– elderly, low grade with slow progression
– malignant lymphocytes in blood may be very high
– B cells are more likely to become malignant
What is the most common type of leukemia in children?
ALL
What is the most common accute type of leukemia in adults?
AML
What are patechei?
- small microbleeds in skin
What is lymphadenopathy?
Symptom of lymphoma; disease of the lymph nodes, in which they are abnormal in size, number, or consistency
What are the symptoms of lymphomas?
- lymphadenopathy
- constitutional symptoms
What are constitutional symptoms?
- symptoms of lymphomas
- weight loss, fever, fatigue, etc.
What is follicular lymphoma?
(Non-hodgkin's) – most common – low grade – elderly – the follicular structure of the lymph nodes is partially preserved and the tumor cells resemble mature lymphocytes or follicular center-activated lymphocytes
What are diffuse large b-cell lymphomas?
– complete effacement (elimination) of the normal lymph node architecture
– Instead of normal lymphocyte, tissue is infiltrated with large lymphoid cells that have irregular nuclear outlines and prominent nucleoli.
– several types (50% of lymphomas)
– intermediate or high grade
– poor prognosis (spread into the parenchyma of major organs is common)
What is Burkitt’s lymphoma?
– highly malignant tumor composed of small B cells that divide rapidly
– children from Northern Africa
– Epstein–Barr virus (EBV) infection
– chemotherapy
What is multiple myeloma?
Malignant disease of plasma cells
- monoclonal population results in monoclonal spike on
serum protein electrophoresis
What are clinical feature of multiple myeloma and when does it usually appear?
– Bone lesions
– Kidney damage
– Middle age usually
How do you diagnose multiple myeloma?
– bone marrow biopsy
– x-ray
– Serum Protein Electrophoresis (SPE)
What is hemostasis?
- a process which causes bleeding to stop
- Interaction of vessels, platelets, coagulation factors
What are the 3 types of bleeding disorders?
- Vascular
- Platelet
- Clotting Factor
What are vascular bleeding disorders?
– mechanical trauma (most common)
– vessel wall fragility (can cause spontaneous bleeding)
– immune damage
What are platelet disorders?
– qualitative (number of platelets)
• Congenital
• Acquired [Chronic renal failure, Aspirin]
– quantitative (abnormality in structure)
What are clotting factor deficiencies and how are they classified?
- deficiency in clotting factor that leads to bleeding disorder
– congenital
• Hemophilia A (Factor VIII)
• Hemophilia B (Factor IX)
– acquired
• decreased/ defective production [Liver disease, Vitamin K
deficiency]
– increased consumption [Disseminated Intravascular Coagulation - DIC]
(Consumption of coagulation factors occurs during the formation of thrombi of any type and is most prominent in DIC)
– anticoagulants
What clotting factor is associated with Hemophilia A?
Factor VIII
What clotting factor is associated with Hemophilia B?
Factor IX
What are streptococcus mutans and what do they promote?
Bacteria that cause caries
What are some complications associated with dental caries?
- pulpitis (inflammation of dental pulp tissue)
- apical abscess (most common form of dental abscess and is caused by infection of the root canal of the tooth)
- periapical granuloma (chronic inflammation located at the apex of a non vital tooth)
- radicular cyst (most frequent cystic lesion related to teeth and result from infection of the tooth)
What is periodontitis
– inflammation of periodontal recesses (gingiva, periodontal membrane, alveolar bone)
What is the most common cause of tooth loss
periodontitis
What is stomatitis?
– inflammation of the mouth (oral mucosa)
What causes stomatitis?
– infectious causes
• viruses [herpes], bacteria, fungi [candida]
– non infectious causes
• aphthous ulcers (a recurrent round or oval sore or ulcer inside the mouth)
• immunologic
What is leukoplakia?
clinical term for a persistent white lesion
What is erythroplakia?
clinical term for a persistent red lesion
What is the most common type of malignant oral neoplasm?
squamous cell carcinomas (>95%)
What are some risk factors for malignant oral neoplasm?
tobacco, alcohol
How do malignant oral neoplasms present?
– may present as leukoplakia
– most common locations are anterior 2/3 of tongue, lower lip
– metastasize to regional lymph nodes
What is the treatment and survival rate for malignant oral neoplasms?
treatment with surgery and radiation (5 yr survival = 45%)
What is sialadenitis?
– inflammation of a salivary gland, usually the parotid gland
What causes sialadenitis?
– infectious causes
• viral [mumps]; bacterial [Staphylococcus aureus]
– autoimmune causes
• Sjogren’s syndrome is immune mediated inflammation of salivary + lacrimal glands
What is pleomorphic adenoma?
- benign salivary neoplasm of both epithelial and stromal elements
- most common salivary gland tumor
- needs proper excision, may recur locally
What is esophagitis?
inflammation of the epithelial lining of the esophagus
What are the causes of esophagitis?
– infectious causes [viral (Herpes), fungal (Candida)]
– chemical causes [Gastroesophageal disease (GERD)]
What is Gastroesophageal disease (GERD)?
– reflux of gastric contents into esophagus resulting in inflammation
– relaxed tone of lower esophageal sphincter allows reflux of acid
What is Barrett’s esophagus?
- symptom of GERD
• presence of metaplastic intestinal type epithelium in lower esophagus
• increased risk of developing adenocarcinoma of the esophagus
What is hiatus hernia?
– displacement of portion of the stomach above the diaphragm
What’s the difference between a sliding hiatus hernia and a paraesophageal hiatus hernia?
- sliding hernia (90%) refers to “sliding” of stomach upwards
- paraesophageal hernia (10%) refers to portion of stomach protruding upward beside esophagus
What is achalasia?
– disorder of esophagus resulting in increased resting tone of LES (lower esophageal sphincter)
– food is unable to enter stomach due to increased tone of LES
What is esophageal varices?
– dilation of submucosal veins of the distal esophagus
What causes esophageal varices?
– often due to portal hypertension secondary to hepatic cirrhosis
– significant morbidity and mortality associated with rupture
What are the most common malignant esophageal neoplasms; where do they typically occur?
– usually carcinomas
• either squamous cell carcinoma or adenocarcinoma
– typically occur in lower portion of esophagus
What are some risk factors for malignant esophageal neoplasms?
– risk factors for squamous carcinoma include tobacco, alcohol
– risk factors for adenocarcinoma include Barrett’s esophagus
How do malignant esophageal neoplasms usually present?
– presents as ulceration or a mass
– lymphatic invasion
What is gastritis?
– inflammation of the mucosal lining of the stomach
What causes acute gastritis compared to chronic gastritis?
– Acute gastritis
• acute erosive inflammation of the mucosal lining of the stomach
– stress, drugs (aspirin), alcohol
– Chronic gastritis
• chronic inflammation of the mucosa with acute exacerbations
• Helicobacter pylori infection
– H pylori is a bacteria that survives in the acidic gastric environment
– associated with one type of chronic gastritis
– chronic H. Pylori infection associated with increased incidence of gastric adenocarcinomas and lymphomas
What is autoimmune gastritis?
Chronic gastritis
• autoimmune destruction of parietal cells in the stomach (that secrete HCl)
• associated with increased risk of gastric adenocarcinoma
What is peptic ulcer disease?
– chronic multifactorial disease characterized by mucosal ulceration that extends through the entire gastric epithelial layer and into the muscularis
– localized chronic ulceration of gastric or duodenal mucosa
What causes peptic ulcer disease and what factors are associated?
– due to action of acid on weakened gastric or duodenal mucosa
– factors include H. pylori, stress, hormones
What are some complications associated with peptic ulcer disease?
– complications
• hemorrhage [melena, iron deficiency anemia, hematemesis]
• perforation [extend through the intestinal wall and form a hole; peritonitis]
• scarring [stenosis (narrowing), obstruction]
What is the most important neoplasm of the stomach?
carcinoma of the stomach (gastric carcinoma)
What are some risk factors for gastric carcinoma?
• risk factors include nitrosamines, Japanese, H. pylori
How do you classify gastric carcinomas?
All are adenocarcinomas
– on basis of gross appearance (polypoid, fungating, ulcerating, diffuse)
– on basis of histological appearance (intestinal type, signet cell)
What is the prognosis for gastric carcinoma?
[5 year survival 20%]
What is a Virchow node?
lymphatic spread of a gastric carcinoma
What is MALToma?
a low grade lymphoma arising in chronic H. pylori
located in the MALT of the gastric lining
What is the most common site of gastrointestinal lymphomas?
the stomach
What is Meckel’s diverticulum?
– developmental disorder of small bowel due to persistence of the omphalomesenteric (vitelline) duct (Connection of the yolk sac to the embryonic digestive system)
How common is Meckel’s diverticulum?
– 2 % of population, 2 ft. from ileocecal valve, 2% ectopic gastric mucosa, 2 % develop symptoms
What are some causes of malabsorption in the small bowel?
• maldigestion, decreased absorption, impaired transport
What is an example of a malabsorption disease?
Celiac disease
What is celiac disease?
- damage to small bowel mucosa due to hypersensitivity reaction to gluten, a protein present in wheat
- malabsorption results from the damage to the small bowel mucosa
What is Girardia and what does it cause?
• Parasite
• Infections of the small bowel (beaver fever)
- It presents as diarrhea or malabsorption secondary to heavy colonization of the duodenum and the proximal small bowel.
How common are neoplasms of the small bowel?
Rare
What are 2 examples of malignant neoplasms in the small bowel?
• carcinoids
– term for low grade malignant neoplasm of neuroendocrine cells
– typically located in the submucosa, where they form small nodules elevating the overlying mucosa
– may produce carcinoid syndrome (diarrhea, flushing, bronchospasm)
– locally invasive
• lymphomas
What are 2 major inflammatory bowel diseases?
- Crohn’s disease
- ulcerative colitis
What is Crohn’s disease?
- a chronic inflammation of the gastrointestinal tract that most often involves the terminal ileum and the colon
How does Crohn’s disease present?
- skip lesions (a wound or inflammation that is clearly patchy, “skipping” areas that thereby are unharmed)
- transmural inflammation (inflammation does not remain limited to the mucosa but extends through the entire wall of the intestine
- granulomas (typically produced in response to infection, inflammation, or the presence of a foreign substance)
Where does Crohn’s disease affect?
– may affect any part of the gastrointestinal tract (mouth to anus)
What are some complications associated with Crohn’s disease?
• fissures (a tear in the lining)
• strictures (narrowing of a section)
• fistulas (abnormal connection or passageway that connects two organs or vessels that do not usually connect),
• adhesions (bands of fibrous tissue that can form between abdominal tissues and organs)
• dysplasia less common than ulcerative carcinoma
• extra-colonic manifestations
– arthritis, eye involvement, primary sclerosing cholangitis (inflammation and scarring of the bile ducts), skin lesions
What is ulcerative colitis?
an intestinal inflammation of unknown etiology that most often involves the large intestine (always includes rectum)
How are ulcerative colitis and Crohn’s disease different?
- Crohn’s disease of the colon, which is typically segmental, ulcerative colitis is a diffuse disease
- UC doesn’t extend to ileum
How does ulcerative colitis present?
- confluent (diffuse) involvement from rectum proximal to cecum
- small bowel is not involved
- inflammation is confined to mucosa
What are some complications of ulcerative colitis?
• toxic megacolon (acute toxic colitis with dilatation of the colon)
• dysplasia (abnormal growth)
• extra-colonic manifestations
– arthritis, eye involvement, primary sclerosing cholangitis, skin lesions (similar to Crohn’s)
What is Hirschprung’s disease?
large bowel pathology
– congenital absence of colonic nerve ganglia resulting in portion of colon with no peristalsis
– dilation of colon proximal to aganglionic (no ganglia) segment
What is diverticular disease?
– disease, generally of the elderly, characterized by outpouchings (bulging) of colonic mucosa (pseudodiverticulum - False diverticula that do not involve muscular layers or adventitia)
- also includes diverticulosis and diverticulitis
What is diverticulosis vs diverticulitis?
Diverticulosis - disease characterized by the formation of diverticula (i.e., outpouchings of the intestinal wall). These diverticula may be solitary or multiple, congenital or acquired
Diverticulitis - term for inflammation of a diverticulum
What are some complications associated with diverticular disease?
- pericolonic (around or encircling the colon) abscess
- peritonitis (inflammation of the peritoneum)
- colonic stenosis (is a condition in which a part of the colon is very narrow, resulting in a partial blockage)
What is an example of infectious inflammation of the large bowel?
pseudomembranous colitis
What is pseudomembranous colitis?
– acute colitis (swelling of the colon) characterized by formation of a pseudomembrane
– due to toxin produced by bacterium Clostridium difficile
– due to broad spectrum antibiotic use
What is ischemic bowel disease?
– includes several disorders that compromise blood flow through segments of the intestine
• certain parts of bowel susceptible to ischemia (watershed areas - regions of the body that receive dual blood supply from the most distal branches of two large arteries)
• ischemia (an inadequate blood supply) may result from atherosclerosis (disease in which plaque builds up inside your arteries)
- affects large bowel
What are hemorrhoids?
– variceal (varicose vein) dilation of veins in the submucosa of the anorectal area
What are hyperplastic polyps?
• most common colonic polyp (protruberant mass), no malignant potential
What are hamartomatous polyps?
- they are developmental abnormalities in which the normal components of the tissue aggregate in an abnormal manner
- occur in children
- commonly from Peutz-Jeghers syndrome
What is Peutz-Jeghers syndrome?
– autosomal dominant, characterized by multiple hamartomatous polyps and pigmented lesions on lips, peri-oral skin
– increased risk of malignancies
How is Peutz-Jeghers inherited?
autosomal dominant
What are adenomatous polyps?
- They are gland-like growths that develop on the mucous membrane that lines the large intestine
- adenomas
- benign neoplasms (tubular, villous, tubulovillous)
- increased risk of carcinoma if villous or high grade dysplasia
- familial adenomatous polyposis is autosomal dominant hereditary tumor syndrome
What is familial adenomatous polyposis?
autosomal dominant hereditary tumor syndrome in which adenomatous polyps are common
What is the prevalence of colonic carcinoma?
– 3rd most common malignant tumor in North America
– 3rd most common cause of cancer-related death in North America
What ages are associated with colonic carcinoma??
– peak incidence in 60 - 80 years age group
– rare before age 40 unless predisposing condition
What are some risk factor for colonic carcinoma?
- Inflammatory bowel disease (IBD)
- Familial adenomatous polyposis (FAP)
How do the present and how can they be diagnosed?
– histologically adenocarcinomas
– metastasize via lymphatics or blood stream
– staging takes into account depth of penetration, nodes, mets
– majority occur in distal colon
– digital rectal exam as part of routine physical exam
What is appendicitis?
- something gets stuck in appendix = causes ->
– acute bacterial infection of appendix secondary to luminal obstruction (fecolith (extreme form of fecal impaction), lymphoid hyperplasia (increase in the number of lymphocytes),
pinworms)
How does appendicitis present?
– abdominal pain (McBurney’s point (over the right side of the abdomen), rebound tenderness), systemic features, leukocytosis (an increase in the number of white cells in the blood)
– rupture leads to peritonitis (inflammation of the peritoneum)
What is most common neoplasm of appendix?
Carcinoid
adenocarcinomas also occur
What is the function of the hepatobiliary system?
– detoxify metabolic waste products – remove old red blood cells (with spleen) – produce bile – synthesize plasma proteins – synthesize plasma lipoproteins – detoxify drugs
Blood flow of hepatobiliary system
– portal vein, hepatic artery supply the sinusoids
– blood drains through sinusoids in lobule into central vein
– blood exits via hepatic vein into IVC
What is bilirubin?
– breakdown product of hemoglobin – conjugated (solubilized) in liver – excreted into bile – bile excreted into bowel – altered by bacteria present in bowel
What is urobilinogen?
– reabsorbed (yellow urine)
altered by bacteria present in bowel
What is stercobilinogen?
– not reabsorbed (stool brown)
altered by bacteria present in bowel
What is cirrhosis?
End stage liver disease characterized by fibrosis and regenerative nodules
What are the causes of cirrhosis?
– alcohol
– viral hepatitis (HBV&HCV)
– metabolic and hereditary (hemachromatosis - causes your body to absorb too much iron from the food you eat, Wilson’s disease - genetic disorder in which copper builds up in the body)
– drugs
– biliary cirrhosis (immune system attacks the liver causing slow, progressive damage to the bile ducts)
What are some complications that arise from cirrhosis?
Portal hypertension
• varices (extremely dilated veins),
• ascites (abnormal accumulation fluid in the abdominal (peritoneal) cavity),
• splenomegaly (abnormal enlargement of the spleen)
What does hepatitis mean?
Inflammation of the liver parenchyma
What are some non-infectious causes of hepatitis?
Metabolic disorders
– hemochromatosis
– Wilson’s disease
– Alpha 1 antitrypsin deficiency
Drug/toxin induced
– acetaminophen
– alcohol
What are one main infectious cause of hepatitis?
Viral hepatitis
How many types of hepatitis are there?
6 (A,B, C, D, E, G)
What is hemochromatosis?
• autosomal recessive disorder of iron metabolism resulting in increased deposition of iron in various organs including
liver, heart, pancreas
What is Wilson’s disease?
• autosomal recessive disorder of copper metabolism resulting in increased deposition of copper in various organs
including liver, brain, and eye
What is alpha 1 antitrypsin deficiency?
• autosomal recessive disorder resulting in decreased alpha 1 antitrypsin, may cause emphysema, cirrhosis
How does acetaminophen cause hepatitis?
dose related necrosis of liver cells
How does alcohol cause hepatitis?
• three pathologic changes linked to alcohol use
1)fatty liver (steatosis)
– all alcoholics show steatosis [fatty yellow liver]
2) alcoholic hepatitis
– acute inflammation with fibrosis
3) cirrhosis
What is hepatitis A, how is it transmitted?
- fecal oral transmission
- no chronic state
- rarely lethal
- vaccine available
What is hepatitis B, how is it transmitted?
- parenteral, perinatal, sexual transmission
- 5-10% progress to chronic hepatitis
- massive hepatic necrosis and death are uncommon
- increased incidence of hepatocellular carcinoma
- vaccine available
What is hepatitis C, how is it transmitted?
- parenteral, sexual transmission
- 50-70 % progress to chronic hepatitis
- increased incidence of hepatocellular carcinoma
- no vaccine
What is hepatitis D, how is it transmitted?
- parenteral, possibly sexual transmission
* requires coinfection with hepatitis B
What is hepatitis E, how is it transmitted?
• fecal oral transmission
What is a hepatic abscess and how are they caused?
– abscesses may form in liver parenchyma
– maybe caused by bacteria or by ameba (a parasite)
What is hydatid disease and how are they caused?
– a disease of various organs cause by a parasite [echinococcus (cestode (tapeworm))] characterized by
formation of cysts on liver
What is schistosomiasis?
– liver disease results from schistosome (a parasite) depositing eggs in branches of portal vein
What is ascariasis?
– liver disease resulting from obstruction of bile ducts by the parasite
What is primary sclerosing cholangitis?
Immune disorder
– disease of unknown etiology characterized by destruction of intra-hepatic and extra- hepatic bile ducts by
lymphocytes and macrophages
– blocked bile ducts
When does primary sclerosing cholangitis usually occur?
Younger males
most also have inflammatory bowel disease (Ulcerative Colitis > Crohn’s Disease)
– increased incidence of cholangiocarcinoma (cancer that arises from the cells within the bile ducts)
What is auto-immune hepatitis?
– chronic hepatitis in young females characterized by presence of autoantibodies to specific antigens
– favorable response to steroids
– associated with other vautoimmune diseases
What is primary biliary cirrhosis?
– disease of unknown etiology characterized by destruction of small intra-hepatic bile ducts and eventual
cirrhosis
– possibly T-cell mediated
– autoimmune disease affecting middle age females
– antimitochondrial antibodies in 95%
– cirrhosis develops over 10-15 yrs.
– no cure
What are the common benign neoplasms of the liver and which is most common?
– cavernous hemangioma (most common)
– hepatocellular adenoma
What is cavernous hemangioma?
- benign neoplasm of endothelial (blood vessel ) origin
* most common benign neoplasm in
What is hepatocellular adenoma?
- benign neoplasm of hepatocyte origin
* young females on oral contraceptive pill
What are the most common malignant neoplasms of the liver?
– hepatocellular carcinoma [HCC]
– metastatic carcinoma
What is hepatocellular carcinoma (HCC)?
• malignant neoplasm of hepatocytes
• tumors may be diffuse, solitary, or multiple nodules
• AFP is a protein usually secreted by fetal hepatocytes
– AFP levels are elevated in HCC and useful as tumor marker
What are the risk factors for HCC?
• risk factors include cirrhosis, HepB Virus, HepC Virus, hemochromatosis (too much iron absorption), alpha 1 antitrypsin deficiency
What is the most common malignancy of the liver?
metastatic carcinoma
What is cystic duct?
from gallbladder empties into common hepatic duct forming common bile duct
What is cholelithiasis?
Presence of gallstones in the gall bladder
What are the different types of gall stones?
- cholesterol stones (10 %)
- pigment stones (15%)
- mixed stones (75%)
How do you diagnose gall stones?
diagnose by ultra-sound
What are complications of cholelithiasis?
- cholecystitis (inflammation of the gallbladder)
- obstructive jaundice (symptoms develop due to a narrowed or blocked bile duct or pancreatic duct, preventing the normal drainage of bile from the bloodstream into the intestines)
- ascending cholangitis (inflammation of the bile duct)
- gallstone ileus (small bowel obstruction caused by an impaction of a gallstone within the lumen of the small intestine)
What is cholecystitis?
inflammation of the gallbladder
– usually due to gallstones
– usually due to gallstones
– acalculous cholecystitis = no stone
What is choledocholithiasis?
stone present in the common bile duct
What are the neoplasms associated with the biliary tract?
– adenocarcinomas
– cholangiocarcinoma
• associated with Primary sclerosing cholangitis, clonorchiasis (infectious disease caused by the Chinese liver fluke)
What is pancreatitis?
– inflammation of the cells of the pancreas
What is acute pancreatitis?
– acute inflammation with tissue necrosis due to release of pancreatic enzymes
What are the main causes of acute pancreatitis?
alcohol and gallstones responsible for 80 % of cases
What are some complications that arise from acute pancreatitis?
- abscess
- pseudocyst (a fluid-filled cavity resembling a cyst but lacking a wall or lining)
- peritonitis (inflammation of the peritoneum)
- chronic pancreatitis
- diabetes
What is chronic pancreatitis and what is an important factor?
– persistence of inflammation after original inciting agent removed
mation after original inciting agent removed
– progressive fibrosis
– alcohol important factor
What is an example of an exocrine pancreas neoplasm?
– Malignant
• pancreatic carcinoma
– adenocarcinoma arising from duct epithelial cells
– poor prognosis
What type of epithelium lines the urinary tract?
Transitional type epithelium
What are the major renal syndromes?
- nephritic syndrome
- nephrotic syndrome
- asymptomatic hematuria, asymptomatic proteinuria
- acute renal failure
- chronic renal failure
- renal tubular defects
Oliguria
– decreased urine production
Anuria
– no urine production
Polyuria
What is an example of when this happens?
– increased urine production
Diabetic - because increased osmotic pressure due to high blood glucose levels
- In kidney, gets filtered into urine - more pee
Hematuria
– passage of blood in urine
Proteinuria
– protein in urine
What is acute nephritic syndrome
- diagnosed on the basis of typical clinical and laboratory findings – syndrome includes • oliguria • hematuria • proteinuria • edema • hypertension
When does nephritic syndrome usually happen?
– typically occurs 2-3 wks after throat infection with Gp A strep
Nephritic vs. nephrotic
At the most basic level, remember that nephrotic syndrome involves the loss of a lot of protein, whereas nephritic syndrome involves the loss of a lot of blood.
- also, difference in cause:
Nephritic - strep
Nephrotic - glomerulonephritis and systemic diseases (diabetes mellitus, lupus)
What is nephrotic syndrome?
– syndrome characterized by loss of large amounts of protein in urine with resulting hypoalbuminemia and edema
What causes nephrotic syndrome?
– variety of causes including
• minimal change glomerulonephritis (inflammation of the glomeruli)
• membranous glomerulonephritis
• membranoproliferative glomerulonephritis
• focal segmental glomerulosclerosis
• systemic diseases (diabetes mellitus, lupus)
What are some complications associated with nephrotic syndrome?
– prone to infections and thrombi due to loss of proteins involved in immunity and coagulation
What is acute renal failure?
– your kidneys suddenly become unable to filter waste products from your blood
– acute onset of decreased urine production
– develops over days to weeks
– decreased renal function, oliguria, electroloyte disturbances
What is the prognosis for acute renal failure?
– usually reversible
• if not reversible, require dialysis or transplant
What are the causes of acute renal failure and how are they divided?
– causes divided on basis of location of disorder
• prerenal
– eg. decreased renal perfusion due to congestive heart failure
• renal
– eg. glomerular disease such as acute glomerulonephritis
• postrenal
– eg. Ureteric obstruction due to stones
What is chronic liver failure?
– condition characterized by a gradual loss of kidney function over time
– insidious decrease in renal function due to damaged kidneys
What are the stages of chronic renal failure?
usually several stages as renal function decreases
• diminished renal reserve (kidney’s ability to increase its basal glomerular filtration rate)
• renal insufficiency (poor function)
• renal failure
• end stage renal failure
– end stage kidney refers to common appearance of a terminally damaged kidney that may be due to a number of
causes
– require dialysis or transplant
What are the two classifications that you can divide glomerular diseases into?
Primary (of unknown cause) and secondary (from a different known cause or medical condition)
