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
|
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
|
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
|
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
|
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
|
|
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
|
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
|
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
|
|
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
|
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
|
Definition
FAP, chronic gastritis small, solitary lesions |
|
|
Term
|
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
|
|
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
|
Definition
the removal of RBCs in the spleen |
|
|
Term
|
Definition
removal of portions of RBCs by PMNs resulting in spherocytes |
|
|
Term
|
Definition
pallor of skin
pallor of conjunctivae
koilonychia (changes in nails) w/ Fe def.
skeletal chagnes in hemoglobinopathies |
|
|
Term
|
Definition
|
|
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
|
Definition
myelophthisicanemias and other disorders |
|
|
Term
echinocytes AKA burr cells
|
|
Definition
associated with liver disease and uremia |
|
|
Term
|
Definition
|
|
Term
|
Definition
associated with HE and Fe def, others |
|
|
Term
|
Definition
microangiopathic hemolytic anemias |
|
|
Term
|
Definition
associated with microangioanemias and valcular lesions |
|
|
Term
stomatocytes (slit area of pallor) |
|
Definition
associated with hereditary disease and Rh null disease, others |
|
|
Term
|
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
|
|
Term
|
Definition
nucleated RBCs with stainable iron granules |
|
|
Term
What stimulates erythropoietin release? |
|
Definition
|
|
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
|
|
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
|
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
|
Definition
dysphagia, glossitis, iron def. anemia
endoscopy performed |
|
|
Term
Most common immunohemolytic anemia |
|
Definition
|
|
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
|
|
Term
Clinical Features of G6PD |
|
Definition
splenomegaly, episodic hemolysis when exposed to something that generates oxidant stress
asymptomatic unless triggered
treatment: transfusions, avoidance |
|
|
Term
|
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
|
|
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
|
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
|
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
|
Definition
RBCs in sera containing antibodies or complement
if antigen present agglutination occurs |
|
|
Term
|
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
|
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
|
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
|
Definition
certain disorders that result in ineffective erythropoiesis. Supresses hepatic hepcidin despite elevated iron stores |
|
|
Term
|
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
|
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
|
|
Term
Treatment for Colonic Ulceration |
|
Definition
|
|
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
|
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
|
|
Term
|
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
|
|
Term
Dark-pigmented gallstones contain... |
|
Definition
|
|
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
|
|
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
|
|
Term
anti- Saccarychomes cerevisiae antibodies are typical a test for ___ |
|
Definition
|
|
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
|
|
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
|
|
Term
hepatocytes with fat droplets= |
|
Definition
|
|
Term
decreased ceruloplasmin: think? |
|
Definition
|
|
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
|
|
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
|
|
Term
gastric acholrhydria is typical for ___ |
|
Definition
chronic atrphic gastritis |
|
|
Term
Cirrhosis can lead to what cancer? |
|
Definition
|
|
Term
oral contraceptives are associated with which hepatic cancer |
|
Definition
|
|
Term
Which neoplasm is a common incidental finding in the liver? |
|
Definition
|
|
Term
Duodenal atresia is commonly associated with which underlying disease |
|
Definition
|
|
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
|
|
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
|
|
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
|
|
Term
Pathology of infectious mononucleosis |
|
Definition
enlarged paracortex, immunoblasts, reed-sternberg cells, dilated sinuses |
|
|
Term
|
Definition
proliferative lesions of macrophages and dendritic cells |
|
|
Term
|
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
|
|
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
|
|
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
|
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
|
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
|
Definition
AR mutation in genes that control chloride anion transport into mucosal epithelium, this results in impaired Na+ transport, thick mucus
salty sweat |
|
|
Term
|
Definition
impaired ATP7B gene which transports copper from hepatocytes to bile
penicillinamine binds copper leading to excretion |
|
|
Term
|
Definition
mutation in HFE which regulates iron absorption
increased AST and ALT, irondeposition on Prussian stain
frequent phlebotomy |
|
|
Term
|
Definition
damage to mitochondria
brain edema, fatty liver |
|
|
Term
Que es Focal nodular hyperplasia |
|
Definition
hyperplastic growth of hepatic tissue, all hepatic tissue |
|
|
Term
|
Definition
serum carcinoembryonic antigen CEA |
|
|