Related Subjects:
|Hodgkin Lymphoma
|Non Hodgkin Lymphoma
|Diffuse large B-cell lymphoma
|Intravascular large B-cell lymphoma
|Mantle cell lymphoma
|Marginal Zone Lymphoma
|Gastric (MALT) Lymphoma
|Primary CNS Lymphoma (PCNSL)
|Burkitt's lymphoma
|Follicular Lymphoma
๐งฌ Marginal Zone Lymphoma (MZL) is a rare B-cell non-Hodgkin lymphoma (NHL), arising from lymphocytes in the "marginal zone" around lymphoid tissues. It is indolent (slow-growing) but clinically important due to its link with infections and autoimmune disease.
โน๏ธ About
- Indolent (slow-growing) B-cell lymphoma
- A type of non-Hodgkin Lymphoma (NHL)
- Accounts for ~10% of all NHL cases
๐งฌ Aetiology
- Often infection-driven (chronic antigen stimulation):
- ๐ฝ๏ธ Helicobacter pylori โ gastric MALT lymphoma
- ๐ฅ Campylobacter jejuni โ small bowel lymphoma
- ๐ฆ Hepatitis C virus (HCV) โ splenic MZL
- Autoimmune links: Sjรถgrenโs syndrome, Hashimotoโs thyroiditis
๐ฉบ Clinical Features
- ๐ Nodal disease: painless lymphadenopathy
- โ ๏ธ B-symptoms: fatigue, weight loss, night sweats
- ๐ซ Extranodal (MALT): gastric pain, dyspepsia, organ-specific symptoms
- ๐ฉธ Splenic MZL: abdominal discomfort from splenomegaly, cytopenias
Types of MZL
- Extranodal (MALT) Lymphoma: most common, linked to chronic infection/autoimmunity
- Nodal MZL: rare, mainly lymph node involvement
- Splenic MZL: ~10% cases, linked with HCV, splenomegaly common
๐ Investigations
- ๐ฌ Biopsy: gold standard diagnosis
- ๐ฅ๏ธ Imaging: CT/PET, ultrasound (especially for spleen)
- ๐งช Bloods: FBC, LDH, viral serology (esp. HCV)
- ๐ฆด Bone marrow biopsy: useful in splenic subtype
๐ Management
- ๐ Watchful waiting: asymptomatic/indolent cases
- ๐ Antibiotics: eradicate H. pylori in gastric MALT โ can induce remission
- โข๏ธ Radiotherapy: for localized disease
- ๐ Chemo-immunotherapy: Rituximab ยฑ chemotherapy in advanced disease
- ๐ช Splenectomy: in splenic subtype with massive splenomegaly
Prognosis
- ๐ Generally favourable (indolent course)
- โณ Many patients live for years post-diagnosis
- โ๏ธ Prognosis depends on subtype, stage, and response to therapy