MALT lymphoma |
Classification and external resources |
Endoscopic image of gastric MALT lymphoma taken in body of stomach in patient who presented with upper GI hemorrhage. Appearance is similar to gastric ulcer with adherent clot. |
ICD-9 |
200.3 |
ICD-O: |
M9699/3 |
OMIM |
604860 |
DiseasesDB |
31339 |
MeSH |
D018442 |
MALT lymphoma (MALToma) is a form of lymphoma involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be afflicted. It is a cancer originating from B cells in the marginal zone of the MALT, and is also called extranodal marginal zone B cell lymphoma.
Contents
- 1 Associations
- 2 Treatment
- 3 Epidemiology
- 4 See also
- 5 References
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Associations
Gastric MALT lymphoma is frequently associated (72–98%) with chronic inflammation as a result of the presence of Helicobacter pylori[1], potentially involving chronic inflammation, or the action of H. pylori virulence factors such as CagA.[2]
The initial diagnosis is made by biopsy of suspicious lesions on esophagogastroduodenoscopy (EGD, upper endoscopy). Simultaneous tests for H. pylori are also done to detect the presence of this microbe.
In other sites, chronic immune stimulation is also suspected in the pathogenesis (e.g. association between chronic autoimmune diseases such as Sjögren's syndrome and Hashimoto's thyroiditis, and MALT lymphoma of the salivary gland and the thyroid).
Treatment
If the disease is limited to the stomach (which is assessed with computed tomography), then 70–80% of patients will have a complete regression on treatment with antibiotic eradication of H. pylori.[3]
Others may be effectively controlled with the use of radiotherapy, or surgery. Both modalities may be curative in localized disease.
In contrast, if the disease has spread or has been refractory on antibiotics, chemotherapy may need to be considered.
A t(11;18)(q21;q21) chromosomal translocation, giving rise to a API2-MLT fusion gene,[4] is predictive of poor response to eradication therapy.[5]
Two other genetic alterations are known:
- t(1;14)(p22;q32) which deregulates BCL10, at the locus 1p22.
- t(14;18)(q32;q21), which deregulates MALT1, at the locus 18q21.
These seem to turn-on the same pathway as API2-MLT (i.e., that of NF-κB). They both act upon IGH,[6] which is at the locus 14q32.
Epidemiology
Of all cancers involving the same class of blood cell, 8% of cases are MALT lymphomas.[7]
See also
- Primary cutaneous marginal zone lymphoma
References
- ^ Parsonnet J, Hansen S, Rodriguez L, Gelb A, Warnke R, Jellum E, Orentreich N, Vogelman J, Friedman G (1994). "Helicobacter pylori infection and gastric lymphoma.". N Engl J Med 330 (18): 1267–71. DOI:10.1056/NEJM199405053301803. PMID 8145781.
- ^ Hatakeyama, M. & Higashi, H. (2005). "Helicobacter pylori CagA: a new paradigm for bacterial carcinogenesis". Cancer Science 96: 835–843. DOI:10.1111/j.1349-7006.2005.00130.x. PMID 16367902.
- ^ Bayerdörffer E, Neubauer A, Rudolph B, Thiede C, Lehn N, Eidt S, Stolte M (1995). "Regression of primary gastric lymphoma of mucosa-associated lymphoid tissue type after cure of Helicobacter pylori infection. MALT Lymphoma Study Group.". Lancet 345 (8965): 1591–4. DOI:10.1016/S0140-6736(95)90113-2. PMID 7783535.
- ^ Noels H, van Loo G, Hagens S, et al. (April 2007). "A Novel TRAF6 binding site in MALT1 defines distinct mechanisms of NF-kappaB activation by API2middle dotMALT1 fusions". J. Biol. Chem. 282 (14): 10180–9. DOI:10.1074/jbc.M611038200. PMID 17287209. http://www.jbc.org/cgi/pmidlookup?view=long&pmid=17287209.
- ^ Liu H, Ruskon-Fourmestraux A, Lavergne-Slove A, Ye H, Molina T, Bouhnik Y, Hamoudi R, Diss T, Dogan A, Megraud F, Rambaud J, Du M, Isaacson P (2001). "Resistance of t(11;18) positive gastric mucosa-associated lymphoid tissue lymphoma to Helicobacter pylori eradication therapy.". Lancet 357 (9249): 39–40. DOI:10.1016/S0140-6736(00)03571-6. PMID 11197361.
- ^ Ye H, Gong L, Liu H, et al. (February 2005). "MALT lymphoma with t(14;18)(q32;q21)/IGH-MALT1 is characterized by strong cytoplasmic MALT1 and BCL10 expression". J. Pathol. 205 (3): 293–301. DOI:10.1002/path.1715. PMID 15682443.
- ^ Turgeon, Mary Louise (2005). Clinical hematology: theory and procedures. Hagerstown, MD: Lippincott Williams & Wilkins. p. 283. ISBN 0-7817-5007-5. "Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001.)"
Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
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B cell
(lymphoma,
leukemia)
(most CD19, CD20)
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By development/
marker
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TdT+
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ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
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CD5+
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naive B cell (CLL/SLL)
mantle zone (Mantle cell)
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CD22+
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Prolymphocytic · CD11c+ (Hairy cell leukemia)
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CD79a+
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germinal center/follicular B cell (Follicular, Burkitt's, GCB DLBCL, Primary cutaneous follicular lymphoma)
marginal zone/marginal-zone B cell (Splenic marginal zone, MALT, Nodal marginal zone, Primary cutaneous marginal zone lymphoma)
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RS (CD15+, CD30+)
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Classic Hodgkin's lymphoma (Nodular sclerosis) · CD20+ (Nodular lymphocyte predominant Hodgkin's lymphoma)
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PCDs/PP
(CD38+/CD138+)
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see immunoproliferative immunoglobulin disorders
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By infection
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KSHV (Primary effusion) · EBV (Lymphomatoid granulomatosis, Post-transplant lymphoproliferative disorder) · HIV (AIDS-related lymphoma) · Helicobacter pylori (MALT lymphoma)
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Cutaneous
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Diffuse large B-cell lymphoma · Intravascular large B-cell lymphoma · Primary cutaneous marginal zone lymphoma · Primary cutaneous immunocytoma · Plasmacytoma · Plasmacytosis · Primary cutaneous follicular lymphoma
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T/NK
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T cell
(lymphoma,
leukemia)
(most CD3, CD4, CD8)
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By development/
marker
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TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
prolymphocyte (Prolymphocytic)
CD30+ (Anaplastic large-cell lymphoma, Lymphomatoid papulosis type A)
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Cutaneous
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MF+variants
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indolent: Mycosis fungoides · Pagetoid reticulosis · Granulomatous slack skin
aggressive: Sézary's disease · Adult T-cell leukemia/lymphoma
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Non-MF
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CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma · Pleomorphic T-cell lymphoma · Lymphomatoid papulosis type B
CD30+: CD30+ cutaneous T-cell lymphoma · Secondary cutaneous CD30+ large cell lymphoma · Lymphomatoid papulosis type A
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Other peripheral
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Hepatosplenic · Angioimmunoblastic · Enteropathy-associated T-cell lymphoma · Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma) · Subcutaneous T-cell lymphoma
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By infection
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HTLV-1 (Adult T-cell leukemia/lymphoma)
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NK cell/
(most CD56)
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Aggressive NK-cell leukemia · Blastic NK cell lymphoma
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T or NK
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EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma) · Large granular lymphocytic leukemia
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Lymphoid+myeloid
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Acute biphenotypic leukaemia
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Lymphocytosis
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Lymphoproliferative disorders (X-linked lymphoproliferative disease, Autoimmune lymphoproliferative syndrome) · Leukemoid reaction · Diffuse infiltrative lymphocytosis syndrome
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Cutaneous lymphoid hyperplasia |
Cutaneous lymphoid hyperplasia with bandlike and perivascular patterns · Cutaneous lymphoid hyperplasia with nodular pattern · Jessner lymphocytic infiltrate of the skin
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cell/phys/auag/auab/comp, igrc
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Tumors: digestive system neoplasia (C15–C26/D12–D13, 150–159/211)
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GI tract |
Upper GI tract
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Esophagus
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Squamous cell carcinoma · Adenocarcinoma
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Stomach
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Gastric carcinoma · Signet ring cell carcinoma · Gastric lymphoma (MALT lymphoma) · Linitis plastica
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Lower GI tract
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Small intestine
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Duodenal cancer (Adenocarcinoma)
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Appendix
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Carcinoid · Pseudomyxoma peritonei
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Colon/rectum
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colorectal polyp: Peutz–Jeghers syndrome · Juvenile polyposis syndrome · Familial adenomatous polyposis/Gardner's syndrome · Cronkhite–Canada syndrome
neoplasm: Adenocarcinoma · Familial adenomatous polyposis · Hereditary nonpolyposis colorectal cancer
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Anus
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Squamous cell carcinoma
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Upper and/or lower
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Gastrointestinal stromal tumor · Krukenberg tumor (metastatic)
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Accessory |
Liver
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malignant: Hepatocellular carcinoma (Fibrolamellar) · Hepatoblastoma
benign: Hepatocellular adenoma · Cavernous hemangioma
hyperplasia: Focal nodular hyperplasia · Nodular regenerative hyperplasia
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Biliary tract
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bile duct: Cholangiocarcinoma · Klatskin tumor
gallbladder: Gallbladder cancer
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Pancreas
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exocrine pancreas: Adenocarcinoma · Pancreatic ductal carcinoma
cystic neoplasms: Serous microcystic adenoma · Intraductal papillary mucinous neoplasm · Mucinous cystic neoplasm · Solid pseudopapillary neoplasm
Pancreatoblastoma
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Peritoneum |
Primary peritoneal carcinoma · Peritoneal mesothelioma · Desmoplastic small round cell tumor
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anat(t, g, p)/phys/devp/enzy
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noco/cong/tumr, sysi/epon
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proc, drug(A2A/2B/3/4/5/6/7/14/16), blte
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Infectious diseases · Bacterial diseases: Proteobacterial G− (primarily A00–A79, 001–041, 080–109)
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α |
Rickettsiales
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Rickettsiaceae/
(Rickettsioses)
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Typhus
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Rickettsia typhi (Murine typhus) · Rickettsia prowazekii (Epidemic typhus, Brill–Zinsser disease, Flying squirrel typhus)
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Spotted
fever
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Tick-borne
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Rickettsia rickettsii (Rocky Mountain spotted fever) · Rickettsia conorii (Boutonneuse fever) · Rickettsia japonica (Japanese spotted fever) · Rickettsia sibirica (North Asian tick typhus) · Rickettsia australis (Queensland tick typhus) · Rickettsia honei (Flinders Island spotted fever) · Rickettsia africae (African tick bite fever) · Rickettsia parkeri (American tick bite fever) · Rickettsia aeschlimannii (Rickettsia aeschlimannii infection)
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Mite-borne
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Rickettsia akari (Rickettsialpox) · Orientia tsutsugamushi (Scrub typhus)
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Flea-borne
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Rickettsia felis (Flea-borne spotted fever)
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Anaplasmataceae
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Ehrlichiosis: Anaplasma phagocytophilum (Human granulocytic anaplasmosis, Anaplasmosis) · Ehrlichia chaffeensis (Human monocytic ehrlichiosis) · Ehrlichia ewingii (Ehrlichiosis ewingii infection)
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Rhizobiales
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Brucellaceae
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Brucella abortus (Brucellosis)
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Bartonellaceae
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Bartonellosis: Bartonella henselae (Cat scratch disease) · Bartonella quintana (Trench fever) · either henselae or quintana (Bacillary angiomatosis) · Bartonella bacilliformis (Carrion's disease, Verruga peruana)
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β |
Neisseriales
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M+ Neisseria meningitidis/meningococcus (Meningococcal disease, Waterhouse-Friderichsen syndrome, Meningococcal septicaemia)
M- Neisseria gonorrhoeae/gonococcus (Gonorrhea)
ungrouped: Eikenella corrodens/Kingella kingae (HACEK) · Chromobacterium violaceum (Chromobacteriosis infection)
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Burkholderiales
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Burkholderia pseudomallei (Melioidosis) · Burkholderia mallei (Glanders) · Burkholderia cepacia complex · Bordetella pertussis/Bordetella parapertussis (Pertussis)
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γ |
Enterobacteriales
(OX-)
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Lac+
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Klebsiella pneumoniae (Rhinoscleroma, Klebsiella pneumonia) · Klebsiella granulomatis (Granuloma inguinale) · Klebsiella oxytoca
Escherichia coli: Enterotoxigenic · Enteroinvasive · Enterohemorrhagic · O157:H7 · O104:H4 (Hemolytic-uremic syndrome)
Enterobacter aerogenes/Enterobacter cloacae
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Slow/weak
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Serratia marcescens (Serratia infection) · Citrobacter koseri/Citrobacter freundii
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Lac-
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H2S+
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Salmonella enterica (Typhoid fever, Paratyphoid fever, Salmonellosis)
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H2S-
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Shigella dysenteriae/sonnei/flexneri/boydii (Shigellosis, Bacillary dysentery) · Proteus mirabilis/Proteus vulgaris · Yersinia pestis (Plague/Bubonic plague) · Yersinia enterocolitica · Yersinia pseudotuberculosis
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Pasteurellales
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Haemophilus: H. influenzae (Haemophilus meningitis, Brazilian purpuric fever) · H. ducreyi (Chancroid) H. parainfluenzae (HACEK)
Pasteurella multocida (Pasteurellosis) · Actinobacillus (Actinobacillosis)
Aggregatibacter actinomycetemcomitans (HACEK)
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Legionellales
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Legionella pneumophila/Legionella longbeachae (Legionellosis) · Coxiella burnetii (Q fever)
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Thiotrichales
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Francisella tularensis (Tularemia)
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Vibrionales
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Vibrio cholerae (Cholera) · Vibrio vulnificus · Vibrio parahaemolyticus · Vibrio alginolyticus · Plesiomonas shigelloides
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Pseudomonadales
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Pseudomonas aeruginosa (Pseudomonas infection) · Moraxella catarrhalis · Acinetobacter baumannii
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Xanthomonadales
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Stenotrophomonas maltophilia
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Cardiobacteriales
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Cardiobacterium hominis (HACEK)
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Aeromonadales
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Aeromonas hydrophila/Aeromonas veronii (Aeromonas infection)
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ε |
Campylobacterales
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Campylobacter jejuni (Campylobacteriosis, Guillain-Barré syndrome) · Helicobacter pylori (Peptic ulcer, MALT lymphoma) · Helicobacter cinaedi (Helicobacter cellulitis)
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gr+f/gr+a(t)/gr-p(c)/gr-o
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