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A slow-growing MALT lymphoma of the tissues around the eye: the conjunctiva, the eye socket, the tear gland or the eyelid. It usually shows itself as a painless salmon-pink patch on the white of the eye or as an eye that has begun to bulge, and it is controlled in almost everybody, often by a short course of radiotherapy.
What it is. The tissues around the eye, the conjunctiva that lines the eyelids and covers the white of the eye, the orbit the eye sits in, the tear gland and the eyelids, are collectively the ocular adnexa. A marginal zone lymphoma arising there grows slowly and causes trouble by taking up space rather than by destroying anything. Three-quarters of lymphomas in this location are MALT lymphomas; the rest are follicular lymphoma, diffuse large B-cell lymphoma or others, which is why a biopsy is needed before anything is decided.
How it shows itself. A flat, salmon-pink patch under the conjunctiva that has been there for months; a painless swelling of the eyelid; an eye that has begun to bulge forward or to sit slightly out of line; double vision; a lump at the outer corner where the tear gland is. Pain, rapid growth and loss of vision are unusual and suggest a different, more aggressive lymphoma. Both sides are involved in a substantial minority, so the other eye is always examined.
How it differs from its parent and from its sibling in the stomach. WHO-HAEM5 sets out that marginal zone lymphomas differ genetically by the site they arise in, and that ocular adnexal disease has its own profile: mutation or deletion of TNFAIP3 is common, and gain of chromosome 6p with loss of 6q is recurrent here and not at other extranodal sites. The translocation that governs treatment in the stomach, t(11;18), is rare here.
The bacterium, and the honest version of the story. In Italian studies, the ocular adnexal lymphomas were associated with Chlamydia psittaci, the bacterium of psittacosis, and a course of doxycycline produced lymphoma regression in about two-thirds of patients in prospective trials. That association does not hold everywhere. A United States study tested 28 ocular adnexal lymphoma specimens with two different polymerase chain reaction assays and found no Chlamydia psittaci DNA in any of them, and noted that the association has been reported internationally with great variability. So whether an antibiotic is worth trying depends on where the patient lives and what the local series show, and a reader told that this lymphoma is caused by an infection should ask which population that finding came from.
How it is treated. For disease confined to the orbit, low-dose radiotherapy is the usual treatment and it is effective. In a series of 28 patients who were given doxycycline first and whose lymphoma relapsed or progressed, salvage radiotherapy at 30 to 36 Gy in 15 to 18 fractions produced a response in every patient and a complete response in 89 per cent, with 4-year progression-free survival of 74 per cent; the recorded toxicity was three cases of grade 2 cataract and three of grade 1 blepharitis, and all but one patient was alive at a median follow-up of 96 months from diagnosis. That paper also answers the question it was written to answer: delaying radiotherapy while an antibiotic is tried did not cost those patients their chance of cure. Very low doses of radiotherapy, around 4 Gy in two fractions, are used in some centres to spare the lens and the tear gland, with retreatment if needed. Disease that has spread beyond the orbit is treated systemically, like any other marginal zone lymphoma.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
A biopsy of the lesion is needed, because about a third of lymphomas at this site are not marginal zone lymphomas and the treatment differs. Imaging of the orbit, usually by magnetic resonance, maps the extent and the relationship to the optic nerve and the muscles. Staging of the rest of the body follows, because apparently localised ocular disease is sometimes part of a systemic marginal zone lymphoma. Both orbits are examined; bilateral disease is common and does not by itself mean the lymphoma has spread.
Radiotherapy to the orbit is the standard treatment for disease confined to the ocular adnexa and it controls it in almost everybody. In a series of 28 patients irradiated after a trial of doxycycline, 30 to 36 Gy in 15 to 18 fractions produced a response in all of them and a complete response in 89 per cent, with four-year progression-free survival of 74 per cent and little toxicity beyond three cases of grade 2 cataract and three of grade 1 blepharitis. Very low-dose schedules of about 4 Gy in two fractions are used to spare the lens and the tear gland, with retreatment if the lymphoma returns. Where local series have shown an association with Chlamydia psittaci, a course of doxycycline is a reasonable first step, and the same series shows that trying it first did not cost patients their response to radiotherapy afterwards.
Treated as any other marginal zone lymphoma: watched where it is causing no trouble, and treated with an anti-CD20 antibody alone or with chemotherapy when it is. The regimens, the thresholds for starting and the evidence are on the marginal zone and MALT lymphoma pages and in the treatment layer of this family.
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Query for this cancer: (TITLE:"Ocular adnexal MALT lymphoma" OR ABSTRACT:"Ocular adnexal MALT lymphoma" OR TITLE:"Ocular adnexal marginal zone lymphoma" OR ABSTRACT:"Ocular adnexal marginal zone lymphoma" OR TITLE:"OAMZL" OR ABSTRACT:"OAMZL" OR TITLE:"Orbital lymphoma" OR ABSTRACT:"Orbital lymphoma" OR TITLE:"Conjunctival lymphoma" OR ABSTRACT:"Conjunctival lymphoma" OR TITLE:"Lacrimal gland lymphoma" OR ABSTRACT:"Lacrimal gland lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Ocular adnexal MALT lymphoma, not a curated reading list.
Italian investigators reported the bacterium of psittacosis in ocular adnexal marginal zone lymphomas and showed that doxycycline produced regression in a proportion of patients.
A United States study tested 28 ocular adnexal lymphoma specimens with two polymerase chain reaction assays and found no Chlamydia psittaci DNA, and noted that the association has been reported internationally with great variability.
In 28 patients whose lymphoma relapsed or progressed after doxycycline, salvage radiotherapy produced a response in every one and a complete response in 89 per cent, with four-year progression-free survival of 74 per cent.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Anyone who has ever had hepatitis B can have the virus wake up when rituximab strips out their B cells, sometimes months later and sometimes fatally. A blood test before the first dose, and a tablet for those who need it, prevents almost all of it.
A shortage of antibodies because treatment has wiped out the B cells or plasma cells that make them, as CD19 CAR-T, CD20 antibodies and BCMA therapies deliberately do. It leaves patients prone to infections, sometimes for years, and is managed with immunoglobulin infusions.
Treatments that remove B cells also remove the antibodies B cells make, and some people never make them again. When repeated chest and sinus infections follow, donated antibody given monthly by drip or weekly under the skin prevents them.
Several lymphoma treatments knock out the part of the immune system that keeps a particular lung infection, Pneumocystis, at bay. A low-dose antibiotic three times a week prevents it, and a separate tablet prevents shingles.
Reactions around the moment a drug is given: chills, fever or breathlessness from antibodies (infusion reactions), true allergy (hypersensitivity, rarely anaphylaxis), and leakage of a damaging drug into tissue around the vein (extravasation).
See all on the product pages:BendamustineRituximab·Printable cards in the navigator
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