Small Bowel Flashcards

1
Q

What are some important small bowel parasites?

A

Protozoa:

  • Giardia
  • Cryptosporidia

Fungi

  • Microsporidia

Bacteria:

  • Tropherma whipplei (Whipples disease)
  • E. Coli

Flatworm:

  • Schistosoma
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2
Q

Describe Giardia

A
  • Trophozoites (protozoan)
  • Pear-shaped
  • Binucleate (“Monkey face”)
  • Faintly basophilic
  • Seen between villi, classically in duodenum
  • Does not invade
  • Sometimes mild villous blunting and IELs
  • Looks like falling leaves in the bowel lumen
  • Aquired from contaminated water, faeco-oral route, sexually transmitted
  • More commen in children, travel and immunocompromised
  • Diarrhoea/Malabsorption - watery and foul-smelling

INVESTIGATIONS:
* Stool - cysts
* PCR

STAINS:
* Masson’s Trichrome
* Giemsa
* CD117+

TREATMENT:
* Metronidozole

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3
Q

Des

Describe Cryptosporidia

A
  • Protozoan
  • Most common - C. Hominis, C. Parvum
  • Acute, watery diarrhoea
  • Can last up to four weeks, relapsing and remitting
  • Located at apex of epithelial cells along luminal border - dot-like organisms
  • Small bowel most common site
  • Also colon, stomach, biliary tree, respiratory tract
  • Seen in children and immunocompromised patients
  • Usually correlates with CD4 counts < 100
  • Faeco-oral route
  • Basophilic
  • Villous blunting
  • Mixed inflammatory infiltrate

STAINS:
* Modified Acid-Fast (AFB)
* Giemsa

TREATMENT:
* Nitazoxanide

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4
Q

Describe Microsporidia

A
  • Obligate intracellular organism
  • Enterocytozoon bieneusi - best seen in electron microscopy
  • Usually seen in the setting of AIDS
  • Jejunum shows highest parasitic burden
  • Bifringent spores seen as intra epithelial inclusions
  • Lamina propria macrophages

INVESTIGATIONS:
* Stool examination
* PCR
* Electromicroscope

STAINS:
* Giemsa
* ZN can highlight spores

Treatment:
* Albendazole

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5
Q

Describe Whipple Disease

A
  • Bacterial infection
  • Tropherma whipplei
  • Small bowel –> Oesophagus –> Stomach –> Appendix –> Colon
  • Malabsorption, diarrhoes, arthralgias, pain, neurological symptoms, lymphadenopathy
  • Lamina propria expanded by foamy macrophages

STAINS:
* PAS +ve
* AFB -ve

Associated with HLA-B27

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6
Q

Describe Schistosomiasis

A
  • Mansoni, japonicum, mekongi, hematobium. intercalatum
  • Contaminated water in endemic areas
  • Focal ulcers
  • Eggs may be calcified, surrounded by fibrosis/granuloma

INVESTIGATIONS:
* Eggs in faeces or urine
* Wet mount/Formalin ethyl acetate

TREATMENT
* Praziquantel

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7
Q

Describe E. Coli

A
  • Enterohaemorrhagic E. coli - 0157:H7
  • Shiga toxin interferes with protein synthesis –> causes cell damage
  • Contaminated food
  • Most self-limiting
  • Can cause** haemolytic-uremic syndrome **and thrombocytopenic purpura

Micro:
* Mucosal haemorrhage, infarct, pseudomembranes
* Neutropholic infiltrates, cryptitis, crypt abscesses

Diagnosis:
* Serotyping
* PCR
* DNA hybridisation
* E coli O157:H7 –> detected from stool culture with selective growth media

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8
Q

Describe Coeliac Sprue

A
  • Immune mediated
  • Caused by sensitivity to prolamins - Wheat (gliadin), Barley (hordein), Rye (secalin), Oats (avenin)
  • Duodenum > Ileum

Associated with:

  • **Autoimmune diseases **- Dermatitis herpetiformis, T1SM, Hashimotos, Graves)
  • Idiopathic diseases - Dilated cardiomyopathy, epilepsy, MS
  • Chromosomal diseases - Downs, Turner, William

Can lead to:

  • Iron deficiency anaemia (microcytic)
  • B12 deficiency (macrocytic anaemia)
  • Osteopenia/Osteoporosisa - altered absorption of calcium and vitamin D3
  • Growth retardation, tooth enamel defects, stomatitis, hypertransaminasemia

Aetiology:

  • HLA DQ2 (98%)
  • HLA DQ8
  • Ingestion of gluten

Microscopy:

  • Shortening of villi (villous atrophy)
  • Intraepithelial lymphocytes (>30 per 100 enterocytes or >12 in villous tips)
  • Loss of brush border at surface of epithelium
  • Increased mitotic activity in crypts
  • Crypt elongation and hyperplasia
  • May see thickened subepithelial collagen band (collagenous sprue is refractive to treatment with no antibodies)

Classification:

  • Marsh Classification
  • Based on: IEL, Crypts (normal or increased), Villi (atrophy)
  • 0 (normal) to 3c (Destructive)

IHC:
* 70% intraepithelial lymphocytes express CD8

DIAGNOSIS:

Histology of duodenal biopsy and serology

  • tTGA (tissue transglutaminase) - highest sensitivity
  • EMA (endomysial antibody) - almost absolute specificity
  • AGA (IgA class antigliadin) 0 now obsolete

PROGNOSIS:

  • Excellent prognosis with gluent free diet
  • Enteropathy associated T cell lymphoma in small bowel - seen in pateints with long lasting, well controlled coeliac
  • Increased risk of small bowel adenocarcinoma
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9
Q

Describe Tropical Sprue

A
  • AKA Post infective sprue
  • May be caused by E. coli or haemophilus
  • Tropics (not jamaica), africa, india
  • Causes malabsorption within weeks of acute diarrheal enteric infection
  • **Ileum **> Duodenum
  • No increased risk to intestinal lymphoma

Treatment:

  • Broad spectrum antibiotics (tetracycline)
  • Folic acid
  • Vitamin B12
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10
Q

Describe CVID

A
  • Common Variable Immunodeficiency syndrome
  • Primary immune defect
  • Reduction in B cells, causing hypogammaglobulinemia (IgG)
  • IgA or IgM deficiency
  • Frequent infections, recurrent giardiasis

Manifestations:

  • Recurrent respiratory infections
  • Chronic lung disease (bronchiectasis)
  • Autoimmune disease’
  • GI disorders

Micro:

  • Absence of plasma cells in the lamiba propria (but can be present in some cases)
  • Lymphoid aggregates
  • Mild villous blunting
  • Increased IELs
  • May see Giardia

Treatment:

  • Immunoglobulin replacement therapy
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11
Q

Describe Abetalipoproteinaemia

A
  • Rare autosomal recessive disorder
  • Interferes with normal absorption of fat and fat-soluble vitamins

Symptoms:

  • Diarrhea
  • Steatorrhea
  • Ataxia (Posterior column of spinal cord involved)
  • Retinitis pigmentosa

Aetiology:

  • MTP deficiency - mutation in microsomal triglyceride transfer protein

Micro:

  • Marked fat vacuoles in apical villous cytoplasm
  • Normal villi

Lab Features:

  • Acanthocytosis on periperal blood smears (burr cells)
  • Decreased serum cholesterol
  • Low levels of LDL and VLDL
  • Low Triglycerides
  • Increased high-density lipoprotein cholesterol levels

Treatment:

  • High doses Vitamin E
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12
Q

Describe Microvillous Inclusion Disease

A
  • AKA Davidson’s disease or Congenital microvillus atrophy
  • Autosomal recessive
  • Disorder of intestinal brush border
  • Intractable watery diarrhoea with steatorrhoea in infants
  • Require TPN
  • Rarely live beyond 2 years of age

AETIOLOGY:
* Mutations in the MYO5B gene
* Small amount have STX3 gene mutations

MICRO:
* severe villous abnormality and crypt hypoplasia
* Resembling coeliac sprue without lymphocytosis
* Increased enterocyte apoptosis and proliferation
* Bubbly vaculated apical cytoplasm
* Extensive or patchy absence of brush border
* Definitive diagnosis on electron microscopy - shows microvillus inclusions

IHC:
* PAS
* CD10

CD10 and PAS stain positive within the enterocytes (cytoplasmic staining).
Normal should just be linear brush border staining,

  • CEA
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13
Q

Describe Diaphragm disease

A
  • Small bowel pathology related to NSAID use

Macro:

  • Small bowel with multiple tight short diaphragm like strictures caused by prominent raised fold of bowel.
  • Ulceration at crests of the folds.

Micro:

  • Thin circumferential membranes
  • Resemble plica circularis
  • Composed of mucosa and submucosa with accompanying fibrosis

Treatment:

  • Cessation of NSAIDs
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14
Q

Describe Small bowel Adenomas

A

INTESTINAL TYPE
* Similar to tubular adenoma of colon

FOVEOLAR
* Tubulovillous architecture
* Characteristic mucinous cap
* Prone to high grade dysplasia

PYLORIC GLAND
* Tightly packed tubules
* Monolayer of cuboidal cells with granular eosinophilia cytoplasm
* NO spivsl mucinous cap

Duodenum is most prone to developing adenomas

Molecular:

  • Non-ampullary duodenal adenomas (60%) —> associated with FAP or MUTYH-associated polyposis
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15
Q

Describe Gangliocytic Paraganglioma

A
  • Rare tumour of periampullary region
  • Second part of the duodenum
  • Unencapsulated
  • Submucosal

Three characteristic cell types:
* Epithelioid
* Spindle-shaped
* Ganglion-like

  • Ususally benign
  • 7% can metastasise

IHC:

Epithelial cells
* CD56
* Synaptophysin
* Chromogranin-A
* Somatostatin
* Progesterone receptor
* Pancreatic polpeptide

Ganglion cells
* CD56
* Synaptophysin
* Chromogranin-A
* Somatostatin
* Pancreatic polpeptide
* S100

Spindle cells
* S100
* BCL2

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16
Q

Describe Enteropathy-Associated T-Cell Lymphoma

A

Features of coeliac disease
* Increased IELs
* Crypt Hyperplasia
* Villous atrophy

  • T-Cell lymphoma that occurs in coeliac disease
  • Jejunum > Ileum/Duodenum
  • Stronger association with** refractory disease **
  • Aggressive clinical course

Micro:

  • Pleomorphic infiltrate
  • Medium to large sized neoplastic cells
  • Angular nuclei, anaplasia, mitotic figures, necrosis

Molecular:

  • Clonal TCR gene rearrangement

IHC:

Positive
* CD3
* CD7
* CD30
* CD103
* Perforin
* Granzyme B

Negative
* CD20
* EBER

Loss of some T cell markers:
* CD2
* CD4
* CD5
* CD8

17
Q

Describe Follicular Lymphoma, Duodenal-Type

A
  • Lymphoma derived from follicular B lymphocytes
  • Ususally affects second part of duodenum
  • May involve jejunum and ileum
  • Confined to mucosa/submucosa
  • No systemic involvement
  • Excellent prognosis
  • Local treatment
  • Indolent course
  • <10% progress to nodal disease

Micro:

  • Almost always low grade (grade 1 - 2)
  • Lamina propria infiltrated
  • Neoplastic follicles lack tingible body macrophages and mantle zones

Molecular:
* t(14;18)

IHC:
* CD10+
* CD20+
* BCL2+
* Low Ki-67

18
Q

Describe Well-Differentiated Neuroendocrine Tumours

A
  • Majority of tumours are non-functioning

Functional:

Gastrinoma
* Gastrin expressing NET in duodenum
* Causes Zollinger-Ellison syndrome - hypergastrinaemia, peptic ulcer, diarhoea
* Associated with MEN type 1

Somatostatinomas
* Somatostatin expressing NET in the ampulla
* Psammoma bodies
* Causes Diabetes, stones, diarrhoea
* Associated with Neurofibromatosis 1

Carcinoid syndrome
* Occurs when liver mets present
* Diarrhoea, bronchospasm, flushing, tricuspid valve fibrosis

Micro:
* Monotonous tumour cells
* Round/oval nuclei
* Salt and pepper chromatin
* Trabecular, nested, corded, ribbons, acinar and/or pseudoglandular architecture

Grading:
1: <2 mitoses/2mm, Ki-67 <3%
2: 2-20 mitoses/2mm, Ki-67 3-20%
3: >20 mitoses/2mm, Ki-67 >20%

IHC:

  • AE1/3+
  • Cam5.2+
  • CK7-
  • CK20-
  • Chromogranin A+
  • Synaptophysin+