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GI- Schemes/Pathology
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214
Bartending
Professional
01/18/2011

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Term

Macrocytic anemia

(common causes)

Definition

megaloblastic anemia (vitamin B12 or folate deficiencies)

liver disease

alchohol, hypothyroidism, reticulocytosis

Term

Microcytic anemia

(common causes)

Definition

iron deficiency (blood loss, malabsorption, dietary deficiency, increased requirement)

thalassemia

sideroblastic

anemia of chronic disease

Term

normocytic anemia

(common causes)

Definition

decreased production (aplastic anemia, renal disease)

increased destruction

hemorrhage

Term
Common causes of intrinsic RBC destruction
Definition

hemoglobinopathy (sickle cell anemia)

enzymopathy (G6PD deficiency, pyruvatic kinase deficiency)

membranopathy (hereditary spherocytosis)

 

Term
common causes of extrinsic RBC destruction
Definition

immune- autoimmune hemolytic anemia

non-immune- DIC, hypersplenism

Term
define koilonychia
Definition
Term

6 laboratory tests for microcytic anemia

 

Definition

Fe levels

total binding capacity

ferritin

review peripheral blood smear

hemoglobin electroporesis

bone marrow biopsy

Term

laboratoy findings for iron deficiency anemia

(Hgb, sFe, TIBC, sFerritin, MCV, RDW, reticulocyte, bone marrow)

Definition

low blood hemoglobin concentration

low serum iron

high TIBC

low serum ferritin

low MCV

high RDW

low reticulocyte count

in bone marrow:  Prussian blue stain for iron is absent

Term
Three mechanisms of decreased erythropoietin disorders
Definition

1. impaired source: renal failure

2. reduced stimulation: endocrine disorders, hypothyroidism and testosterone deficiency

3. inflammatory cytokines, anemia of chronic disease

Term
three intrinsic bone marrow diseases
Definition

hypoplasia:  aplastic anemia, pure red cell aplasia

infiltration:  multiple myeloma, leukemia, fibrosis, metastatic malignancies

myelodysplastic disorders

Term
five important laboratory findings of hemolytic anemia
Definition

elevated LDH

elevated indirect bilirubin

elevated reticulocyte count

decreased haptoglobin

MCV increased or normal

Term
hemolytic infections
Definition
malaria, clostridium perfringens
Term
four common hemolytic membrane abnormalities
Definition

hereditary spherocytosis

elliptocytosis

acanthocytosis

paroxysmal nocturnal hemoglobinuria

Term

polycythemia rubra vera

(definition, lab result, CP, population, cause)

Definition

clonal disorder of the pluripotent hematopoietic stem cell

increased red cell mass

leukocytosis and thrombocytosis

splenomegaly

males>females

avg. age is >60

JAK2 gene mutation

Term
symptoms of polycythemia rubra vera
Definition
headaches, paresthesis, dizziness, tinnitus, visual disturbances, erythromelalgia
Term
complications of polycythemia rubra vera
Definition

vascular thromboses (venous and arterial)

bleeding

leg ulcres

splenic infarcts

myeloid metaplasia

acute leukemia

Term
Diffuse Esophageal Spasm
(D, G, CF, D)
Definition
20% or more simultaneous contractions
Manometry reveals simultaneous contractions. endoscopy may be normal. barium may reveal normal, rosary bead, corkscrew esophagus
heartburn, dysphagia, or chest pain
manometry is diagnostic
Term
manometry of nutcracker esophagus
Definition
avg. peristaltic pressures exceeding 220 mmHg during 10 or more 5 mL liquid swallows
Term
manometry of hypertensive LES
Definition
resting LES pressure above 45
Term
Zencker's diverticulum
(D, E, P, G, M)
Definition
posterior diverticulum, proximal to the circopharyngeal muscle
considered a developmental defect
- due to high pressure, intrabolus, or UES abnormalities
- located immediately above the UES
- outpouching of mucosa through Killian's triangle
-
Term
Esophageal Rings- Schatzski's rings
(D, E, P, CF)
Definition
- at GE junction, squamous mucosa above epithelium
- GERD, congenital, or developmental
- chronic damage from GERD
- solid food dysphagia
Term
achalsia
(D, E, P, M, CF)
Definition
- incomplete LES relaxation, increased LES tone, and aperistalsis of the esophagus
- idiopathic, or secondary to Chagas disease
- idiopathic- failure of distal esophageal inhibitory neurons, secondary- neuron destruction
- myenteric plexus is absent
- dysphagia to both solids and liquids
Term
Reflux esophagitis
(E, P, CF)
Definition
obesity, hiatus hermia, pregnancy, exgenous estrogen
- tLERs, hypotensive LES, anatomic disruption of GE junction
Term
CF of Candidial esophagitis
Definition
odynophagia localized to a discrete retrosternal area
Term
E of esophageal varices
Definition
alcoholic liver disease and chronic active hepatitis, fatty liver
Term
Barrett's esophagus (D, E, P, AI)
Definition
- intestinal metaplasia in esophagus - GERD - noxious factors cause the aberrant differentiation of immature, proliferating cells - can lead to adenocarcinoma
Term
What disorders cause microcytic hypochromic anemias?
Definition
disorders of hemoglobin synthesis
Term
What disorders cause macrocytic anemias?
Definition
abnormalities that impair the maturation of ethyroid precursors in bone marrow
Term
Two common features of hemolytic anemia
Definition
elecated erythropoietin levels, accumulation of hemoglobin degradation products released by red cell breakdown dervied from hemoglobin
Term

 

36y/o obese female presents with RUQ pain x 4hr, after a fatty meal, radiating to the R scapula, associated with nausea. Pt is pain-free now. No prior episodes. On physical exam, minimal RUQ tenderness was observed, no Murphy’s sign elicited. RUQ U/S reveals (marked in arrow) without GB wall thickening (<3mm) or pericholecystic fluid. Diagnosis: ?

Definition
Acute Cholesterol Cholesithiasis
Term

 

36y/o Asian female presents with RUQ pain x 4hr, after a fatty meal, radiating to the R scapula, associated with nausea. Pt is pain-free now. No prior episodes. On physical exam, minimal RUQ tenderness was observed, no Murphy’s sign elicited. Patient is a known case of sickle cell anemia. RUQ U/S reveals (marked in arrow) without GB wall thickening or pericholecystic fluid. Indirect bilirubin is elevated. Ultrasound shows beaded appearance. Diagnosis: ?

Definition
Acute cholesithiasis with pigmented gall stones
Term

 

46yo F w RUQ pain > 24hrs of RUQ pain radiating to the R scapula, started after fatty meal, a/w nausea, vomiting, fever. Exam: Palpable, tender gallbladder, guarding, +Murphy’s = inspiratory arrest. No similar episodes in past.U/S: solitary gallstone, wall thickening (>4mm), GB distension, pericholecystic fluid, sonographic Murphy’s sign present. Lab results point to infection.

Definition
acute cholesterol cholesithiasis
Term

 

87yo M critically ill, on long-term TPN w RUQ pain. Abdomen is tender. Murphy’s sign elicited. No similar episodes in past. Ultrasound: No GB wall thickening, pericholecystic fluid present, no gallstones. Diagnosis: ?

Definition
aute acalulous cholecystitis
Term

 

76y/o female presents with RUQ pain x 24hr, after a fatty meal, radiating to the R scapula.. Several previous episodes are noted over last several years. Management was always conservative. Minimal RUQ tenderness noted, no Murphy’s. Ultrasound: GB wall 6mm, no pericholecystic fluid, no gallstones. Diagnosis: ?

Definition
Chronic calculous cholecystitis
Term

 

46yo F p/w RUQ pain, jaundice, acholic stools, dark tea-colored urine, no fever. Past history of cholelithiasis. No treatment ever undertaken. Exam: unremarkable. HepB/C neg. Ultrasound: solitary CBD stone, dilated CBD > 1cm. Diagnosis: ?

Definition
Choledocholithiasis (in duct leading to the duodenum)
Term

 

46yo F p/w acute RUQ pain, jaundice, acholic stools, dark tea-colored urine. Her blood cultures came back positive for a facultative anaerobic bug. Gram staining is shown. Two weeks ago, she had a biliary colic like pain. U/S at that time revealed multiple small stone in the common bile duct. She decided against any management at that time.

High temperature, increased direct bilirubin, decreased urobilinogen, high leukocytes

Definition
descending cholangitis, choledocholelithiasis
Term

 

72 yo F p/w mild RUQ pain since last several months. She also complains of intermittent episodes of acute colicky pain. She gives past history of gall stones. Her last U/S taken 2 years ago reveal thickend gall bladder, with multiple stones. No pericystic fluid was observed. CBD was not affected.  Her present U/S reveals an infiltrative mass occupying most of the gall bladder. Also noticed are gall stones. Cholecystectomy was performed. Gross and histo-pathology are shown. Diagnosis?

Definition
Carcinoma of the Gall Bladder
Term
What is the parasite associated with CCA?
Definition
Opisthorchis sinensis
Term
Explain the HP and CF of gastric adenocarcinoma
Definition

signet ring cells are present, increased extracellular mucin release

increased desmoplasia causes gastric wall to become rigid, giving patient the 'leather bottle' feeling

Term
radiology of gall stones
Definition

cholesterol, brown stones- radioluscent

calcium carbonate, black stones- radiopaque

 

Term
Define acute calculous cholecystitis
Definition
acute inflammation of the gall bladder resulting from chemical irritation and inflammation of the obstructed gall bladder. Theses events occur in the absence of bacterial infection
Term
Define haptoglobin
Definition
an alpha-2 globulin that binds free hemoglobin, preventing its excretion in the urine
Term

Menckel's diverticulum

(location and complications)

Definition

antimesenteric side of the ileum

ulceration, obstrutction, perforation, intussusception, and neoplasms

Term
What antigen is commonly associated with Celiac sprue?
Definition
HLA-B8
Term
Describe the histopathology of Celiac disease
Definition
villus atrophy with hyperplasia of the underlying crypts and increase mitotic activity
Term
Define Hirshsprung's disease adn risk factors of this disease
Definition
congenital megacolon characterized by lack of ganglion cells/enterix nervous plexuses in segment of biopsy. Involves rectum.  Increased risk with Down Syndrome.
Term
CF of acute gastric ulceration
Definition

hematemesis/ melena, obstruction, pain, nausea, vomiting

chronic gastritis is from H.pylori infection or autoimmunity

Term

Peptic Ulcer Disease

(E, G, CF)

Definition

predisposing factors (H. pylori, NSAIDs, psychologic stress, caffeine, alcohol, and tobacco, radiation injury, chronic bile reflux, mechanical injury, systemic diseases such as Crohn's disease, amyloidosis, graft-versus-host, Zollinger Ellison syndrome)

smooth, oval, punched out single ulcer

pain occurs 1-3 hours after meals, worse at night

Term
Define gastritis cystica
Definition
an exuberant reactive epithelial proliferation associated with entrapment of epithelial-lined cysts found in submucosa
Term

Zollinger Ellison syndrome

(D, G, M)

Definition

gastinomas found in small intestine and pancreas

multiple ulcers in stomach

doubling of oxyntic cells, mucosal thickness

Term
Gastric adenoma
(E, G)
Definition
FAP, chronic gastritis
small, solitary lesions
Term
Carcinoids
(D, E, CF)
Definition
well-differentiated neuroendocrine carcinoma
60-70 yos
chronic atrophic gastritis, increase hormone levels
Term
Gastro-intestinal stromal tumors
(G, M, AI)
Definition
large, solitary, well-circumscribed covered by ulcerated or intact mucosa
spindle cells, different from carcinomas
responds to imantinib, part of Carney triad
Term
Name the five levels of erythropoiesis
Definition
proerythroblast, basophillic erythroblast, polychromatophilic erythroblast, orthochromatophilic erythroblast, reticulocyte
Term
Descrine the degradation of heme
Definition
- heme oxygenase of RE cells--> biliverdin --> bilirubin, bilirubin is bound to albumin and transported to the liver, in hepatocytes 2 glucoronic acids are attached to bilirubin donated from UDP, compound is transported into bile, this is reduced by gut bacteria to form urobilinogen which is then oxidized to stercohilin, urobilinogen is also reabsorbed and converted to uroblin in kidney
Term
In the RBC membrane, what does spectrin bind to?
Definition
actin at multiple sites
connected to Band 3 by ankyrin and band 4.2
bound to glycoprotein A by protein 4.1
Term
What are the products of the Rapoport-Leubering pathway?
Definition
2,3 BPG
Term
What is the composition of the white pulp of the spleen?
Definition
nodules of B lymphocytes, periarteriolar lymphoid sheaths of T cells
Term
Describe the splenic cords of the spleen
Definition
a reticular fiber network
Term
What are the immune functions of the spleen?
Definition
antigen presentation
activation/ proliferation of B an T lymphocytes
production of antibodies
removal of macromolecular antigens
Term
Describe the hematopoietic functions of the spleen
Definition
remove senescent, abnormal, and damaged RBCs and platelets
reclaim iron from the RBCs that it removes
formation of RBCs in early fetal life
Term
Define Culling
Definition
the removal of RBCs in the spleen
Term
Define pitting
Definition
removal of portions of RBCs by PMNs resulting in spherocytes
Term
General CF of anemia
Definition

pallor of skin

pallor of conjunctivae

koilonychia (changes in nails) w/ Fe def.

skeletal chagnes in hemoglobinopathies

Term
define anisocytosis
Definition
variation in RBC size
Term

acanthocytes

(description, mechanism, disorders)

Definition

small spherical cells with irregular thornlike projections

membranes with altered lipid content

associated with abetalipoproteinuria, liver disease, etx.

Term

target cells

(description, disorders)

Definition

increased surface to volume ratio

associated with liver disease, thalassemia, iron def., Hgb S, Hgb C

Term

tear drop

(disorders)

Definition
myelophthisicanemias and other disorders
Term

echinocytes AKA burr cells

 

Definition
associated with liver disease and uremia
Term
ellip
Definition
Term
elliptocytes
Definition
associated with HE and Fe def, others
Term
helmet cells
Definition
microangiopathic hemolytic anemias
Term
schistocytes
Definition
associated with microangioanemias and valcular lesions
Term
stomatocytes (slit area of pallor)
Definition
associated with hereditary disease and Rh null disease, others
Term
spherocytes
Definition
HS, immune hemolytic anemias
Term

basophilic stippling

(composition, disorders)

Definition

aggregated ribosomes

associated with thalassemia, Pb poisoning

Term

Howell-Jolly bodies

(appearance, composition, disorder)

Definition

dark blue spherical granules in RBCs

nuclear DNA fragments

splenectomy

Term
What are Heinz bodies composed of?
Definition
denatured hemoglobin
Term
What are sideroblasts?
Definition
nucleated RBCs with stainable iron granules
Term
What stimulates erythropoietin release?
Definition
decreased oxygen content
Term
Compare the fate of iron in internal and external bleeding
Definition

internal- recycled

external- lost

Term
How does anemia turn your urine red brown?
Definition

when RBCs lyse and haptoglobin is depeleted free Hgb is oxidized to methemoglobin

if reabsorbed it is catabolized by renal tubular cells

Term
What is the cause of renal hemosiderosis?
Definition
iron accumulation within the tubular cells
Term
What type of bilirubin is found in excess during hemolysis?
Definition
unconjugated
Term
Important Lab Tests in Hemolytic anemia
Definition
increased MCV= reticulocytes
Term
What lab tests is useful to classify polycythemia?
Definition

EPO, secondary erythrocytosis = increased EPO

polycythemia vera does not

Term
Define G6PD deficiency
Definition

X-linked recessive disorders involving the hexose monophosphate shunt or glutathione metabolism resulting from deficient or impaired enzyme function impair the ability of the red blood cell to protect themselves from oxidative injury. This results in hemolysis.
Term
Plummer-Vinson Syndrome
Definition

dysphagia, glossitis, iron def. anemia

endoscopy performed

Term
Most common immunohemolytic anemia
Definition
warm antibody type
Term
does bleeding and thrombosis occur in PCV?
Definition
yes, Budd Chiari syndrome
Term
what is the spent phase of PCV?
Definition
appearance of myelofibrosis
Term
What is the function of reduced glutathione?
Definition
protects against ROS
Term
Clinical Features of G6PD
Definition

splenomegaly, episodic hemolysis when exposed to something that generates oxidant stress

asymptomatic unless triggered

treatment:  transfusions, avoidance

Term
How do bit cells occur?
Definition
biting occurs in splenic cords, PMNs bite to remove inclusion
Term
What is the point mutation in sickle cell disease?
Definition
point mutation in the 6th codon of beta hemoglobin causing a replacemnt of glutamate residue with a valine residue. Affords protection from faliparum malaria
Term
How does HbS gain its sickle shape?
Definition
when hemoglobin S is deoxygenated the HbS molecules polymerize
Term
What is the mutation in HbC?
Definition

a point mutation on beta globin

lysine substitutes glutamate

Term
what are some variables that affect the rate and degree of sickling?
Definition

a reduction of intracellular pH results in a decreased oxygen affinity for hemoglobin

prolonged transit time in the microvasculature, can result in irrreversible sickling

Term
What does it mean that sickle cells are sticky?
Definition
increased adhesion molecules
Term
What are some sequelae of sickle cell disease?
Definition

gall stones

vascular occlusion

aplastic crisis (B19)

ischemic necrosis due to salmonella or staph infection

ulceration on distal leg

Term
What are some laboratory test for sickle cell disease?
Definition
polymers of deoxygenated HbS obstruct light resulting in opacity
Term
What are treatment options for sickle cell anemia?
Definition

painful crises are treated with fluid, oxygen supplementation, and analgesics

blood transfusions used for manifestations of chest syndrome, stroke, and priapism

hydroxyurea increased HbF and decreases chance of vas-occlusion

Term

Hemoglobin C disease

(CF, LF)

Definition

homozygous- same as HbS

target cells and rectangular RBCs

Term
what are the most commone mutattions in beta thalassemia?
Definition

- splicing mutations (beta +)

- promoter region mutations (beta +)

- chain terminator mutations (beta 0)

Term
What is the pathogenesis of beta thalassemia?
Definition

unpaired alpha chains form insoluble inclusions. red cell precursors suffer from membrane damage--> apoptosis

ineffecitve erythropoeisis results in cachexia and excessive iron absorption

bone marrow hyperplasia leading to skeletal deformity

Term
What are the clinical features of beta thalassemia major?
Definition

if untreated--> growth retardation

skeletal abnormalities

Term
what is the major hemoglobin in beta thalassamia major?
Definition
F
Term
which hemoglobin is increased in beta thalassemia minor?
Definition
Term
What are the clinical and laboratory features of Hemoglobin H disease?
Definition

deletion of 3 a-globin chains

asian

 

Term
What are the clinical features of hydrops fetalis?
Definition

deletion of 4 alpha-globin chains

hemoglobin Barts

pallor, edema, massive hepatosplenomegaly

fetuses die soon

Term

PNH

(D, P)

Definition

acquired clonal stem cell disorder

mutations in PIGA(x-linked) or lyonization (inactivation)--> lack of synthesis of GPI-anchor proteins, all blood cells can be affected

GPI-anchored, complement-regulating proteins CD55 (inhibites C3 convertase), CD59 binds C8-->cell lysis vulnerability--> acidosis during sleep

Term

PNH

(CF, LF, T)

Definition

hemosiderinuria--> iron def.

thrombosis (platelet inhibitor attached to GPI)

can lead to AML

flow cytometry of GPI-linked proteins

only cure is bone marrow transplantation

Term
DAT
Definition

RBCs in sera containing antibodies or complement

if antigen present agglutination occurs

Term
IAT
Definition

patient serum mixed with red cells that have known antigens

if antibodies present agglutination will occur

Term
Describe warm antibody type and its treatment
Definition

extravascular hemolysis

IgG coated red blood cells bind Fc receptors on phagocytes, biting, Rh is often targeted

DAT is positive

unconjugated bilirubin

treatment:  steroids, immunosuppressive therapy

 

Term
Describe Cold Agglutinin Disease
Definition

IgM antiobodies bind at "cold" temperature

from infections or B cell neoplasms

exposed body parts may experience pallor, cyanosis, and Raynaud phenomenom due to occlusion of vasculature by agglutinated red blood cells

exacerbated in winter

DAT positive for complement

Term
Define Paroxysmal Cold Hemoglobinuria
Definition

IgG autoantibodies bind the P blood group antigen in cooler regions of the body

severe intravascular hemolysis

Term
What is the drug adsoprtion mechanism?
Definition
the drug binds to the RBC membrane and the antibody attaches to the drug without interacting with the erythrocyte
Term
Describe microangiopathic hemolytic anemia
Definition

 microvascualr lesions narrow the vessel lumen usually due to platelet or fibrin deposition

causes:  DIC, TTP, HUS, malignant hypertension

Term
CF, LF of vit B12 def.
Definition

neuro, glossitis

erythroid hyperplasia, ineffective erythropoiesis

elevated methylmalonic acid

Schilling test- radioactive B12 if less than 7.5% is excreted than PA is suspected

Term
where is ferritin mainly found?
Definition
in cytosol and lysosome of liver, spleen, bone marrow, and skeletal muscle cells
Term
explain iron metabolism
Definition

iron balance is regulated by absorption in the duodenum

no excretory regulation, removed by cell sloughing

non-hemem iron need DMT1 to cross apical membrane

Term
Define hemochromatosis
Definition
certain disorders that result in ineffective erythropoiesis. Supresses hepatic hepcidin despite elevated iron stores
Term
Explain the iron studies
Definition

serum iorn- transferrin-bound iron

TIBC- iorn that can be bound to transferrin in serum

transferrin saturation

ferritin- amount of iron stored

Term
Iron deficiency precautions
Definition
if iron deficiency is seen in adult men and postmenopausal women it is assumed to be GI blood loss until proven otherwise.
Term
LF for depletion of iron reserves
Definition

lower serum iron and transferrin saturation levels w/o causing anemia

storage iron, hemoglobin, transport iron, enzyme iron

Term

CF of iron deficiency anemia

 

Definition

fatigability, tachycardia, palpitations and tachypnea upon min. exertion

pallor

pica

plummer-vinson syndrome (eesophageal webs, microcytic hypochromic anemia, glossitis

chronic (koilonychia, alopecia, malabsorption)

Term
CF and LF of anemia of chronic disease
Definition

symptoms are that of underlying disorder

increased iron in marrow macrophages

elevated serum ferritin level (decreased iron incorporation and it is an acute phase reactant)

decreased TIBC

Term
Describe aplastic fanconi anemia
Definition

AR, defect DNA repair complex, associated with hypolplasia of kidney and spleen, bone anomalies

aplastic anemia can also be a result of telomerase defects

Term
Causes of pure red cell aplasia
Definition
neoplasia, drugs, autoimmune disorders, parvovirus infection
Term
Describe myelophthisic anemia
Definition
marrow failure secondary to space-occupying lesions
Term

Viral Enterocolitis

(Morphology)

Definition

shorter intestinal villi

lymphoid lamina propia inflammation

Term

Crohn's disease

(CF, RF)

Definition

RLQ, diarrhea

aphtous ulcers, pyoderma gangrenosum, uveitis, erythema nodosum, sclerosing cholangitis, arthritis, clubbing

Ashkenazi Jews, family history

assymetric distribution, cobblestone mucosa, fistulation

Term
What are the autoantibodies in IBD?
Definition
pANCA-UC, ASCA-CD
Term
Treatment for Colonic Ulceration
Definition
infliximab
Term
Etiology of malabsorption diarrhea
Definition

gluten sensitive eneteropathy

whipple's disease

eosinophilic gastroenteritis

pancreatic insufficiency

intraluminal bile salt deficiencies

parasitic infestation

drug induced

Term

Celiac Sprue

(D, RF,

Definition
Term
What are some clues for recognizing a reactive lymphocyte?
Definition

increased cytoplasmic volume

scalloping RBC

basophilia on edge touching RBC

Term
Which Brady sister is the leukemoid reaction?
Definition
Jan
Term
Histology and LF of ALL
Definition

darker nuclei

CD10 and CD19 positive

Tdt and CD22

Term
In type of gastric carcinoma would you find signet ring cells?
Definition
diffuse, poorly-differentiated
Term
true of false: gastric carcinoma usually metastasizes to lymph nodes
Definition
false
Term
Dark-pigmented gallstones contain...
Definition
bilirubin
Term
yellow or green gallstones contain ____ found in such disorders as _____
Definition

cholesterol

Crohn's disease

Term
amylase and lipase are serum markers for ____
Definition
acute pancreatitis
Term
____is a common test for cystic fibrosis
Definition
elevated sweat chloride test
Term
What organism is commonly seen in chronic gastritis, showing no ulcer or mass and no reflux?
Definition
H. pylori
Term
anti- Saccarychomes cerevisiae antibodies are typical a test for ___
Definition
Crohn's disease
Term
AML and ALL: which is more predominant in children? in adults?
Definition

AML-adults

ALL- children

80-90%

Term
hypercalcemia is a common cause for ____ in patients with hyperparathyroidism
Definition
acute pancreatitis
Term
In what type of pattern does Crohn's disease affect the bowel?
Definition

segmental, whereas UC is diffuse

 

Term
Which antibody is associated with Crohn's Disease?
Definition

ASCA

saccharomyces cerevisiae antibody

Term
A patient with diffuse ulcerations along intestine is most likely at risk for developing what condition?
Definition

sclerosing cholangitis

and adenocarcinoma

Term
Define sclerosing cholangitis
Definition
destruction of cells inside and outside bile ducts
Term
What are the symptoms of Gardner syndrome?
Definition
hundreds of colonic polyps and extraintestinal lesions such as osteomas
Term
Symptoms of familial polyposis coli
Definition

mutated APC--> defective mismatch repair-->genomic instability

hundreds of colonic polyps by 20s

Term
hereditary non-polyposis colon carcinoma
Definition
FEW POLYPS in right colon
Term
increased direct bilirubin: think?
Definition
biliary obstruction
Term
hepatocytes with fat droplets=
Definition
chronic alcoholism
Term
decreased ceruloplasmin: think?
Definition
Wilson's disease
Term
define a1-antitrypsin deficiency and give lab tests
Definition
misfolded gene product protein accumulates in hepatocellular ER. decreased elastic tissue in lungs.  PAS-positive globules in liver, codominant trait
Term
Pernicious anemia can lead to what symptom in the stomach and what cancer?
Definition

linitis plastica or shrunken stomach

from autoimmunity against parietal cells

can lead to adenocarcinoma

Term
Gross pathology of hepatitis B
Definition

inflammation into lobules form portal triads

ballooning degeneration of hepatocytes

Term
elevated serum ferritin suggests?
Definition
hemochromatosis
Term

primary biliary cirrhosis

population and pathology

Definition
middle aged woman, autoimmune destruction of bile ducts
Term
gross pathology of sclerosing cholangitis
Definition
periductular onion skin fibrosis
Term
urea breath test is for___
Definition
H. pylori
Term
gastric acholrhydria is typical for ___
Definition
chronic atrphic gastritis
Term
Cirrhosis can lead to what cancer?
Definition
hepatocellular carcinoma
Term
oral contraceptives are associated with which hepatic cancer
Definition
hepatic adenoma
Term
Which neoplasm is a common incidental finding in the liver?
Definition
hemangiomas
Term
Duodenal atresia is commonly associated with which underlying disease
Definition
Down Syndrome
Term
gross pathology of CREST syndrome affecting the esophagus
Definition
submucosal atrophy along with fibrosis of smooth muscle
Term
Gross pathology of Celiac sprue
Definition
absence of villi, increased surface intraepithelial lymphocytes, and hyperplastic appearing crypts
Term
CF of pancreatic pseudocyst
Definition

upper abdominal pain

extensive peritoneal fluid collection in dilated loops of small bowel

Term
MP and CF of Whipple's disease
Definition

PAS-positive macrophages

extraintestinal defects- polyarthritis, hallucinations

Term
AFP is a marker for what type of cancer
Definition
hepatocellular carcinoma
Term
How does chronic alcoholism make acetaminophen toxicity more likely?
Definition
by depleting the glutathion that helps detoxify the metabolites
Term
stony hard nodes are suggestive of ___
Definition
malignancy
Term

Chronic Nonspecific Lymphadenitis

cause

Definition

results from chronic immunological stimuli

(viral, protozoal, antibodies, etc.)

Term

Follicular Hyperplasia

M, Causes

Definition

large germinal centers surrounded by a collar of small resting naive B cells

rheumatoid arthritis

Term
Trigger for Paracortical Hyperplasia (T cell immune response)
Definition
infectious mononucleosis
Term
Pathology of infectious mononucleosis
Definition
enlarged paracortex, immunoblasts, reed-sternberg cells, dilated sinuses
Term
Define histiocytoses
Definition
proliferative lesions of macrophages and dendritic cells
Term
Hodgkin lymphoma is ____
Definition
a neoplasm of Reed-Sternberg cells
Term

Chronic Lymphocytic Leukemia

(markers, population)

Definition

CD5, 19, 23, smudge cells

Old males

 

Term
Define follicular lymphoma and give cancer biology of disease
Definition

middle age lymphoma composed of cleaved and noncleaved b cells in a nodular pattern, seen in BM

14:18 translocation, antibody to BCL2 locus

overexpression of BCL2

Term
Markers for follicular lymphoma
Definition
CD19, CD20, CD10, BCL6
Term

Burkitt Lymphoma

M, cancer bio, associated virus

Definition

B cells with basophilic cytoplasm and numerous mitotic figures, Starry Night (Van Gogh) pattern

(8:14) c-MYC translocations are always present- does not express BLC2

EBV virus

Term

Mantle Cell Lymphoma

cancer bio, population, involvement, prognosis

Definition

(11:14)(13;32) and cyclin D1 overexpression

50-60 male

blood, BM, spleen

poor

 

Term

Define Plasma Cell Neoplasms:

 

multiple myeloma

plasmacytoma

smoldering myeloma

Waldenstreom macroglobulinemia

monoclonal gammopathy of undetermined significance

Definition

MM: neoplastic masses scattered throughout the P: skeletal system

SM: single mass in bone or soft tissue

WM: high plasma M component without symptoms

MGUS:  M compmonents in blood

Term

Multiple Myeloma

(populaiton, CF, LF, prognosis)

Definition

male, Africans, late 60s

bone pain, constipation, recurrent infections, Bence Jones proteinuria (light chains), punched out lytic bone lesions

binucleated, plasmablases, mott cells, CD138 marker

median survival is 4 to 6 years

Term

Hodgkin's lymphoma

D, E, LF, spread

Definition

heterogenous cellularity

arises from single node or chain of nodes

Reed-Sternberg cells CD15, 30

nodal, splenic, hepatic, marrow and others

Term

Adult T cell Luekemia

associated virus, CF, LF, prognonsis

Definition

endemic HTLV-1

skin lesions, lymphoid tissue enlargement, hypercalcemia

clover leaf cells

less than 1 year

Term

Mycosis Fungoides

Defintion, prognosis, CF

Definition

tumor of skin CD4 helper cells

median survival 8-9

cutaneous lesions inflammation--> plaque --> tumor

Term

Anaplastic Large-Cell Lymphoma

definition, cancer bio, population, prognosis

Definition

cells with abundant cytoplasm and horshoe nuclei

rearrangement in the ALK gene on chromosome 2p23

children

very good prognosis

Term
most common primary origin for left supraclavicular metastasis
Definition
abdominal cancer
Term

Crigler-Najjar syndrome

D, CF, LF, T

Definition

rare, AR, results in absent or decreased levels of UDPGT

Type I- jaundice appears early, Type II- jaundice appears later

plasma exchange transfusion, phototherapy

Term

Gilbert's Syndrome

D, E

Definition

common, AD, unconjugated bilirubin buildup

gene coding region is normal, but transcriptor is mutated

Term

Dubin-Johnson syndrome

D, E, M, D

Definition

increases in conjugated bilirubin without elevations of liver enzymes due to defective calanicular transport of conjugated bilirubin from hepatocytes

common in Iranian Jews, AR

pigment deposition in lysosomes causes the liver to turn black

elevated GGT. increased urinary coprpporphyrin

Term

Rotor Syndrome

D

Definition
similar to Dubin Johnson, but it occurs in young children and liver is not pigmented
Term

Primary Biliary Cirrhosis

D, P, C, T

Definition

chronic, autoimmune cholestatic liver disease, destruction of intrahepatic bile ducts

elevated IgM, Anti-mitochondrial antibodies

eyelid xanthelasmas, skin hyperpigmentationm, arthropathy, cirrhosis

MTX,

Term

Primary Sclerosing Cholangitis

D, M, LF

Definition

chronic cholestatic disorder characterized by inflammation, fibrosis, and strictures of intra and extrahepatic bile ducts with dilated of preserved segments, associated with UC

onion skin fibrosis, beading of biliary tree on radiographs

pANCA, SMA

Term

Cystic Fibrosis

D, P,

Definition

AR mutation in genes that control chloride anion transport into mucosal epithelium, this results in impaired Na+ transport, thick mucus

salty sweat

Term

Wilson's Disease

P, T

Definition

impaired ATP7B gene which transports copper from hepatocytes to bile

penicillinamine binds copper leading to excretion

Term

hemochromatosis

D, LF, T

Definition

mutation in HFE which regulates iron absorption

increased AST and ALT, irondeposition on Prussian stain

frequent phlebotomy

Term

Reye's syndrome

P, M

Definition

damage to mitochondria

brain edema, fatty liver

Term
Que es Focal nodular hyperplasia
Definition
hyperplastic growth of hepatic tissue, all hepatic tissue
Term

Cholangiocarcinoma

P

Definition
serum carcinoembryonic antigen CEA
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