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Primary GI lymphomas
Most common extranodal lymphomas
Heterogeneous
Extranodal lymphomas: 1/3 of all lymphomas
GIT, skin; CNS, testis, bone, soft tissue salivary glands, thyroid, Waldeyer ring, lung kidney, liver, spleen, female genital tract
MALT lymphoma
stomach, intestine (IPSID)
chronic antigenic stimulation
- Helicobacter pylori
Regulation: specific activated T-cells
Slow progression- 90%: stage IE, IIE
(bone marrow involvement: rare, 10%)
MALT lymphoma
Immunohistochemistry
No specific MALT lymphoma markerPositivity: CD20, CD79a; Ig light chains; Ig
heavy chains: IgM; CD43Negativity: CD5, CD10, bcl6, IgD, cyclin D1
CD21, CD10, Ki-67: residual lymphoid follicles
MALT lymphoma
Differential diagnosis
HP gastritis
other lymphomas: DLBCL, MCL, FL…
Integrated approachfavoring MALT lymphoma:dense lymphoid infiltrate prominent LELDutcher bodiesinfiltration of muscularis mucosaeatypia of lymphoid cellsB - cell monoclonality
Macroscopy: often noncharacteristic
Microscopy: Wotherspoon criteria - spectrum
0 normal mucosa
1 chronic active gastritis
2 chronic active gastritis with lymphoid follicles
3 suspicious lymphoid infiltrate,
probably reactive
4 suspicious lymphoid infiltrate,
probably lymphoma
5 MALT lymphoma
B-cell monoclonality detection
Imunohistochemistry
Ig light chainsMolecular biology
PCR
IgH rearrangement
CDR III
It is often not possible to establish a clear diagnosis in a single biopsy.
repeat the biopsy; sampling
MALT lymphoma/gastritis?
Large cell component?
Correct diagnosis and treatment
• Interdisciplinary communication
• Repeated biopsies
• Specialized methods
MALT lymphoma after therapy
• Response: regression of lymphoid infiltrate and LEL
• Gastric mucosa: atrophy, intestinal metaplasia, empty, fibrotic, basal lymphoid aggregates
• Always assess Helicobacter pylori
• B-cell clonality assessment by PCR: not clear
Gastric MALT lymphomaRecurrent genetic abnormalities
• t(11;18)(q21;q21)/ API2-MALT1
usually the sole genetic abnormality, 25% of g. MALT l., H. p. neg., no response to ATB
• t(14;18)(q32;q21)/ IgH-MALT1
non-gastric
• t(1;14)(p22;q32)/ IgH-BCL10; t(1;2)(p22;p12)
MALT lymphoma versus DLBCL
Gastric DLBCL de novotransformation of a low-grade lymphoma
clonal progression in timeIndependent coexistence of 2 clones:
low /high grade component
DO NOT USE “HIGH-GRADE MALT LYMPHOMA“
Mantle cell lymphoma
Multiple lymphomatous polyposis
M60 bad prognosis imunohistochemistry genetics WHO 2001
Lymphomatous polyposis:follicular lymphoma g. 1 of the colon
M, 55, 2 polyps; stage IE, no therap, no disease 3 ys after the diagnosis
F, 53-ys,“ileocaecal carcinoma“
follicular lymphoma stage IV, 7x CHOP; no disease detected 4 ys after the diagnosis
Bcl-2ileum
appendix
Enteropathy-associated T-cell lymphoma
Proximal jejunum Very rare x most common GI T-cell lymphoma
Acute abdomen (40%) – emergency surgeryObstruction/perforation, peritonitis, sepsis, death
Non-acute: pain, weight loss, malabsorption
Age 60, M=F
Enteropathy-associated T-cell lymphoma
Striking association with celiac disease
Histology and immunomorphology
Anaplastic/pleomorphic (80%)
Cel.+, enteropathy +, CD56-Monomorphic (20%)
Cel.-, enteropathy+/-, CD56+
Half of the patients die soon after the manifestation
TCR gamma - PCR
TGGE
ABI PRISM
Enteropathy assoc. T-cell lymphoma
CGH marker: 9q gain (70%; Zettl 2007)