Marginal zone lymphoma Flashcards
Subtypes
Extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT lymphoma)
Nodal marginal zone lymphoma
Splenic marginal zone lymphoma
Chromosomal abnormalities
Trisomy 3 (60%) t(11;18)(q21;q21) (25-40%)- Predicts lack of response to HP Tx t(14;18)(q32;q21) t(1;14)(p22;q32) t(3;14)(p13;q32)
Median age
66
Gender diffrences
Male- stomach, small intestine, skin, and kidney
Female- salivary gland, soft tissue, and thyroid
Disseminated disease %
33%
Related autoimmune diseases
Sjogren- x6.6NHL, x30 MZL. x1000 parotid EMZL
SLE- x2.7 NHL, x7.5 MZL
Hashimoto thyroiditis
Relapsing polychondritis
Bactrial infections
HP- gastric MALT
Chlamydia psittaci- Ocular
Campylobacter jejuni- Immunoproliferative small intestinal disease
Borrelia afzelii- solitary cutaneous MZL?
Achromobacter xylosoxidans- pulmonary?
CD
Light chain restriction
B cell-associated antigens (CD19, CD20, CD22, CD79aת BCL2)
Complement receptors (CD21 and CD35)
Negative for CD5, CD10, CD23, BCL6, and cyclin D1
Types of primary cutanous MZL
I switched imuunoglobulin type (IgG. IgA, IgE) CXCR3 negative Predominance of T cells IgG4 positive in 40%
II Diffuse proliferation or large nodules of neoplastic B cells IgM CXCR3 MYD88 50% More likely have extracutaneous disease
Evaluation
CBC, Chem, PEP, beta-2-microglobulin
Borrelia serology in endemic areas
TBCT or PET-CT
BM not indicated
Tx of primary cutanous MZL
Radiation is prefferd option for limited disease- 99% CR
Surgical resection is an option if radiation is not possible- if clean margins- 99% CR
Rituximab for more systemic disease
Gastric MALT pretreatment avaluation
ECOG and Karnofsky Endoscopy with multiple biopsies HIV HCV HBV PETCT BM biopsy Fertility-preserving measures
Tx of stage I/II HP positive gastric MALT
HP eradication
If failure- RT
Post treatment monitoring of stage I/II HP positive gastric MALT
HP eradication confirmation
Endoscopy every 3 months
B symptoms
< 5%
