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year 2 > Path Exam 2- GI disease & lymphomas > Flashcards

Path Exam 2- GI disease & lymphomas Flashcards

1
Q

Cholera

A
  • sites: SI
  • symptoms: severe watery diarrhea “rice water”
  • complications: dehydration, electrolyte imbalances

A subunits catalyzes ADP-ribosylation of GTP-binding proteins causing persistant activation of adeylate cyclase wwhich increases cAMP and Cl- secretion

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2
Q

Campylobacter spp.

A
  • sites: colon
  • symptoms: watery or bloody diarrhea
  • complications: Guillain-Barre, arthritis
  • -looks like UC
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3
Q

Shiegellosis

A
  • sites: left colon, Ileum
  • symptoms: bloody diarrhea
  • complications: Reiter syndrome, HUS
  • looks like UC
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4
Q

Salmonellosis

A
  • sites: colon and SI
  • symptoms: watery or bloody diarrhea
  • complications: sepsis, abscess
  • looks like UC
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5
Q

Enteric (typhoid) fever

A
  • sites: SI
  • symptoms: bloody diarrhea, fever
  • complications: chronic infection, carrier state, encephalopathy, myocarditis; *perforation

Fecal mononuclear leukocytes

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6
Q

Yersinia spp.

A
  • sites: ileum, appendix, right colon
  • symptoms: abdominal pain, fever, diarrhea
  • complications: Autoimmune e.g. reiter syndrome of reactive arthritis
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7
Q

E. coli (EnteroToxigenicEC)

A
  • sites: SI
  • symptoms: severe watery diarrhea
  • complications: dehydration, electrolyte imbalance
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8
Q

Enterohemorrhagic (EHEC)

A
  • sites: colon
  • symptoms: bloody diarrhea
  • complications: HUS
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9
Q

Pseudomembranous Colitis

A

C. difficile

  • sites: colon
  • symptoms: watery diarrhea, fever
  • complications: relapse, toxic megacolon, mushroom cloud/ volcanic erruption
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10
Q

Whipple disease

A
  • sites: SI
  • symptoms: diarrhea/malabsorption (lymphatic transport)
  • complications: arthritis*, CNS dis, PAS + macrophages in lamina propria

-male farmers 30-50yrs
-symptoms may last YEARS
Tropheryma whippelii

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11
Q

Follicular lymphoma

A
  • B cell
  • Indolent; median survival 7 yrs
  • CD 20, 19, 10
  • t(14:18) overexpress BCL2 (blocks apoptosis, chemo res.)
  • nodal
  • follicles that are BCL2 +
  • adults; middle yrs
  • poor response to chemo, can transform into large B cell
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12
Q

Large B cell lymphoma

A
  • B cell
  • Aggressive; months, good response to chemo
  • CD 19, 20
  • BCL6 (↑ growth ↓ differentiation ↓ apoptosis)
  • often extranodal: CNS in HIV, Waldeyer ring (destructive mass in liver or spleen)
  • diffuse large lymphs
  • any age, HIV and transplant, HIV assoc.
  • some arise form other B lymphoma
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13
Q

Burkitt

A
  • B cell
  • Aggressive; most rapid growing human malignancy
  • CD 19, 20, 10 (surface IgM and BCL6)
  • t(8;14) MYC aerobic glycolysis
  • starry sky (intermediate lymphocytes, abundant mitosis, apoptic bodies)
  • usually extra: kidney, ovary, adrenals
  • responsive to chemo

African: facial, mandible 100% EBV
HIV: node/extra nodal 25% EBV
Sporadic: all ages, youth-ileocecal mass, kidney, ovaries (1/3) 15-20% EBV

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14
Q

MALT/marginal

A
  • B cell
  • Indolent
  • CD 19, 20; post germinal center, memory B cells
  • t(11;18) BCL10 or MALT1
  • H. pyloryi, Sjogren, Thyroiditis
  • diffuse lymphs, B cells at diff stages of differentiation including plasma cells
  • reactive polyclonal prolif initially → monoclonal transformation (B-cell clone that is still dependent on T cells)
  • remain localized for prolonged periods, late systemic spread
  • regression may still occur
  • may regress w/ antibiotics
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15
Q

Anaplastic Large cell

A

-T cell
-aggressive; curable w/ chemo
-CD8. CD30
-ALK (2p23) rearrangement- defining
-Soft tissues of children
-Horseshoe shape nucleus
-curable
(immunoperoxidase stain for ALK)

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16
Q

Adult T cell leuk/lymphoma

A
  • T cell
  • subacute 1 yr
  • CD 4
  • Skin, nodes, bone marrow
  • convoluted nuclei (clover shaped)
  • hypercalcemia (demylination may occur), *peripheral lymphocytosis

-HTLV-1 infection (Japan, w africa, carrib)

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17
Q

Mycosis fungoides

A
  • T cell
  • Indolent, years
  • CD4 Sezary cell (CLA, CCR4, CCR10)
  • starts in skin, RASH patch phase → plaque → tumor → LN → bone marrow
  • cerebriform nuclei, *pautrier absc
  • topical treatment of “fungus” for years, not curable
18
Q

Sezary cell leukemia

A
  • T cell
  • Aggressive
  • CD4 Sezary cell
  • Erythroderm (exfoliative) BM: leukemia
  • Cerebriform nuclei
19
Q

HL: Nodular sclerosis type

A
  • most common type
  • lacunar and some diagnostic RS cell CD15+, CD30+, *EBV-
  • young adults or adolescents
  • M=F
  • collagenous bands
  • mediastinum (cough & dyspnea), lower cervical and supraclavicular nodes
  • very good prognosis (stage I or II when diagnosed)
  • background infiltrate: T-lymph, eosinophils, macrophages, plasma cells
20
Q

HL: Mixed cellularity

A
  • mononuclear & diagnostic RS cells CD15+, CD30+, *EBV+
  • Biphasic: young adults and older adults 55+
  • M > Fe
  • poorer prognosis (stage III or IV)
  • likely to have B symptoms (*cyclic fever, night sweats, weight loss)
  • background infiltrate: T-lymph, eosinophils, macrophages, plasma cells
21
Q

HL: Lymphocyte predominance

A
  • more common in young males
  • frequent L&H (popcorn cell)
  • -CD 15-. CD30-. *CD20+. *BCL6+
  • cervical/axillary lymphadenopathy, mediastinum
  • background: variants of follicular dendritic cells and reactive B cells
  • tend to recur
  • excellent prognosis
22
Q

Familial adenomatous polyposis (70% of FAP)

  • molecular defect:
  • genes:
  • transmission:
  • sites:
  • histology:
A

-molecular defect: APC/WNT pathway
-genes: APC
-transmission: *autosomal dominant
-sites: none
-histology: NUMEROUS tubular, villous; typical
adeonocarcinoma (need 100 to make diagnosis)

  • colorectal carcinoma 100% of untreated pts
  • congenital hypertrophy of retinal pigment epi
  • numerous colorectal adenomas as teenagers (30yrs)
23
Q

Familial adenomatous polyposis (<10% of FAP)

  • molecular defect:
  • genes:
  • transmission:
  • sites:
  • histology:
A
  • molecular defect: DNA mismatch repair
  • genes: MUTYH
  • transmission: *none, recessive
  • sites: none
  • histology: sessile serrated adenoma; adenoma; mucinous adenocarcinoma
  • colorectal carcinoma 100% of untreated pts
  • congenital hypertrophy of retinal pigment epi
  • numerous colorectal adenomas as teenagers (<30yrs)
24
Q

Hereditary nonpolyposis colorectal cancer (Lynch syndrome)

  • molecular defect:
  • genes:
  • transmission:
  • sites:
  • histology:
A
  • molecular defect: DNA mismatch repair (results in microsatellite instability)
  • genes: MSH2, MLH1 (inherit one mutant DNA repair gene)
  • transmission: autosomal
  • sites: right side
  • histology: sessile serrated adenoma; mucinous adenocarcinoma

-increased risk of colorectal & extraintestinal cancer

25
Q

Sporadic colon cancer (80%)

  • molecular defect:
  • genes:
  • transmission:
  • sites:
  • histology:
A
  • molecular defect: APC/WNT pathway
  • genes: APC
  • transmission: none
  • sites: left side
  • histology: tubular, villous; typical adenocarcinoma
26
Q

Sporadic colon cancer (10-15%)

  • molecular defect:
  • genes:
  • transmission:
  • sites:
  • histology:
A
  • molecular defect: DNA mismatch repair
  • genes: MSH2, MLH1
  • transmission: none
  • sites: right side
  • histology: sessile serrated adenoma; mucinous adenocarcinoma
27
Q

Peutz-Jeghers syndrome

  • age:
  • gene:
  • GI lesions:
  • extra-GI symptoms:
A

Hamartomatous polyps syndrome

  • age: 10-15 yrs
  • gene: KHB1/STK11; autosomal dominant

-GI lesions: arborizing polyps (CT, **SM, lamina propria and glands) (intussusception) ;
SI > colon > stomach; *colonic adenocarcinoma

-extra-GI symptoms: skin macules (around eye, mouth, nostrils, buccal mucosa, genitalia, perianal)

–INCREASED RISK of thyroid, breast, lung, pancreas, gonadal, and bladder cancer

28
Q

Juvenile polyposis

  • age:
  • gene:
  • GI lesions:
  • extra-GI symptoms:
A

Hamartomatous polyps syndrome

  • age: <5 yrs
  • gene: autosomal dominant (SMAD4, BMPR1A)
  • GI lesions: juvenile polyps in RECTUM (3-100);

–INCREASED RISK of gastric, SI, colonic, and pancreatic adenocarcinoma (GI cancers)

-extra-GI symptoms: pulmonary arteriovenous malformations, digital clubbing

29
Q

Gardner syndrome

A
  • variation of FAP (10-15 yrs, APC, MUTYH, multi adeomas)

- plus osteomas, desmoids, skin cysts

30
Q

Turncot syndrome

A
  • variation of FAP (10-15 yrs, APC, MUTYH, multi adeomas)

- plus CNS tumors (gliomas)

31
Q

Oligohydramnios (Potter’s) sequence

A

decreased amniotic fluid leads to fetal compression

  • flattened facies
  • positional abnormalities of hands and feet
  • compromised growth of chest wall (pulmonary hypoplasia)
  • nodules in amnion (amnion nodosum)
32
Q

Pentad of premature infants

A
  • RDS (hyaline membrane disease): deficiency of pulmonary surfactant (most produced after 35 wks)
  • retinolental fibroplasia: O2 toxicity- changes in expression of VEGF
  • bronchopulmonary dysplasia: O2 toxicity- arrested development of alveolar septation at saccular stage, dependent on oxygen after 28 days
  • necrotizing enterocolitis: inversely related to gestational age, symptoms dont appear until 1st feeding, intestinal ischemia prereq.
  • bloody stools, abdominal distention, circulatory collapse (sepsis and shock)

-subependymal hemorrhage: 2ry bleeding into ventricles, always causes brain damage

33
Q

Menetrier Disease

A

Hypoproteinemic Hypertrophic gastropathy

-excessive secretion of TGF-a →
-causes massive foveolar (surface MUCINOUS epi) hyperplasia of the fundus and body
-lots of mucus produced and not much acid:
*protein-losing enteropathy → hypoproteinemia
increased risk for adenocarcinoma

34
Q

Zollinger-Ellison syndrome

A
  • Gastrin secreting tumor → parietal cell proliferation (hyperplasia) → enlarged rugal folds
  • Gastrin secreting tumor → ECL release H2 → increased acid → duodenal ulcers or chronic diarrhea (Rx PPI)
  • Gastrinomas (duodenum, pancreas, antrum) may be sporadic or occur in MEN I pateints
  • over half act malignant
  • may be multiple and exceedingly small
35
Q

Reed Sternberg Cell

A

Malignant cell of Hodgkin lympoma
-binucleated, bilobed giant cells

Classic RS:

  • CD 15+, CD 30+
  • IL5 → eosinophillia
  • IL10 → ↓ Th1 & CTL response
  • TNFa & bFGF → fibrosis
  • M-CSF → monocyte/macrophage infiltration/ activation
  • various chemokines → increase Th2 response

Non-Classic RS:
-CD15-, CD30-, CD20 +

36
Q

Multiple Myeloma pathogenesis & morphology

A
  • tumor cells & BM stromal cells → IL-6 GF for MM
  • Plasma cells → MIP1a activates osteoclasts and causes bone lesions & hypercalcemia
  • increased risk of infection

excess Ig predisposes for:

  • serum hyperviscosity
  • proteinuria w/ renal damage
  • risk of infection

MORPHOLOGY:

  • Increased/abnormal plasma cells in BM
  • normal plasma cells
  • plasmablasts; mulitnucleated cells
  • Ig inclusions- Russell bodies
  • Mott cell- lots of russell bodies
  • Dutcher body: Ig in nucleus
37
Q

Multiple Myeloma clinical features & labs

A
  • Bone pain: lower back
  • Hypercalcemia (MIP1a): confusion, weakness
  • recurrent bacterial infections
  • renal insufficieny
  • symptoms of amyloidosis: racoon eyes, kindey problems (light chains?)
  • hyperviscosity syndrome

LABS

  • M protein
  • IgG, A, D, light chain (immunofixation)** or
  • Bence-Jones protein in urine (kappa or lambda light chain)**
  • peripheral cytopenia (↓ WNC, RBC, platelets)
  • bone marrow plasmacytosis (>30%)**
  • increased BUN & creatinine
  • increased uric acid & LDH
  • low albumin w/ high total protein
  • ROULEAUX

** + end organ damage & 1+ CRAB for diagnosis

38
Q

Cryptosporidium

A
  • sodium malabsorption & chloride secrtetion
  • tight junction permeability
  • attach to enterocyte brush border and form vacuole
  • persists in AIDS

location: throughout GI most numerous terminal ileum
- nonbloody watery diarrhea

39
Q

Tuberous sclerosis

A
  • age:
  • gene: TSC1, TSC2
  • GI lesions: hammartomatous polyps (rectal)
  • extra-GI symptoms: facial angiofibroma, cortical tubers, renal angiomyolipoma
40
Q

Lymphoplasmacytic lymphoma

A
  • indolent B-cell neoplasm
  • older adults >50+
  • portion of neoplastic B cells -> plasma cells
  • only IgM
  • no free light chains (no renal failure) or amyloidosis
  • bone marrow w/ russell and dutcher bodies
  • involves lymph nodes* live* and spleen*
  • hepatospenomegaly, lymphadenopathy
  • anemia
  • -BM replaced
  • -AI hemolytic anemia due to cold agglutinins (IgM)
  • Hyperviscosity syndrome (headache, dizzy, stupor, deaf, visual impairment)
41
Q

Achalasia

A

LES fails to relax

  • symptom onset in YOUNG ADULTHOOD
  • risk of regurg and aspiration
  • risk for squamous cell carcinoma
42
Q

shared systemic features of Crohns and UC

A
  • erythema nodosum
  • pyoderma gangrenosum (UC)
  • migratory polyarthritis
  • ankylosing spondylitis
  • uveitis
  • primary sclerosing chonalgitis (UC 10x)
  • occur in developed countries
  • mainly females, white, ashkenazi jews
  • begins in teens/late 20s
  • precipitated by stress

year 2 (15 decks)

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