Low-grade Malignant Lymphoma Research Articles

The Impact of Anemia on the Survival of Patients Diagnosed With Low-Grade Malignant B-cell Lymphomas.

B-cell lymphomas with a low degree of malignancy represent a heterogeneous group of diseases, that evolve slowly, butpresent particularities in terms of long-term survival. We investigated the impact of anemia from the time of diagnosis in 249 patients with malignant B-cell lymphomas, diagnosed between January 2011 and December 2015, in the Hematology Department of the Sibiu County Emergency Hospital, Romania. We included 126(50.6%) male and 123 (49.4%) female patients with the average age being 68.2 years. Among all patients, 106 (42.6%) were diagnosed with chronic lymphocytic leukemia (CLL), 61 (24.5%) with marginal zone lymphoma (MZL), 53 (21.3%) with multiple myeloma (MM), 16 (6.4%) with follicular lymphoma (FL), nine (3.6%) with plasmacytoma, and four cases with hairy cell leukemia (HCL). The serum Hb value in the subject group varied between 2.6 g/dL and 17 g/dL.At diagnosis, 18 (7.2%) patients had severe anemia, 32 (12.9%)had moderate anemia, 58 (23.3%) had mild anemia, and 141 (56.6%) had no anemia at all at the time of diagnosis. In our group, the higher degree of anemia was correlated with a more advanced stage of the disease but not with the older age of the patients. Our study's highest median value of LDH corresponded to moderate anemiaand the lowest value to patients who did not have anemia. Patients who did not have anemia at diagnosis had the best survival at five years, followed by those with mild anemia, then those with moderate anemia. In our cohort, subjects with the lowest Hb value at diagnosis had the worst survival. The results of our study conclude that anemia represents a negative impact factor not only on the patient's quality of life but also on their survival.

Phenotypic heterogeneity of mycosis fungoides cells leads to the difficulties in determining tumour origin.

Mycosis fungoides (MF) is a low-grade malignant cutaneous T-cell lymphoma that originates from memory T cells. It typically follows a unique and relatively indolent disease course. MF is used to be characterized by a tissue-resident memory T cell (TRM) phenotype, although recent molecular research has revealed its complexity, casting doubt on the cell of origin and the TRM-MF paradigm. Recent clonal heterogeneity studies suggest that MF may originate from immature early precursor T cells. During development, the tumour microenvironment (TME) influences tumour cell phenotype. The exact origin and development trajectory of MF remains elusive. Clarifying the origin of MF cells is vital for accurate diagnosis and effective treatment.

Cutaneous lymphoproliferative disorders: Back to the future.

In the 1980s, immunohistochemistry and clonality analyses became instrumental in the recognition and definition of new types of cutaneous T-cell lymphoma (CTCL) and cutaneous B-cell lymphoma (CBCL) and the development of new classifications. By accepting loss of pan-T-cell antigens and clonal T-cell receptor gene rearrangements as important criteria to differentiate between benign and malignant T-cell proliferations, and monotypic immunoglobulin light-chain expression and clonal immunoglobulin gene rearrangements as crucial criteria to distinguish between benign and malignant B-cell proliferations, many cases, until then diagnosed as cutaneous lymphoid hyperplasia or pseudolymphoma, were reclassified as primary cutaneous CD4+ small/medium T-cell lymphoma (PCSM-TCL) or primary cutaneous marginal zone lymphoma (PCMZL), respectively. However, in recent years there is growing awareness that neither these immunohistochemical criteria nor demonstration of T-cell or B-cell clonality is specific for malignant lymphomas. In addition, many studies have reported that these low-grade malignant CTCL and CBCL have an indolent clinical behavior and an excellent prognosis with disease-specific survival rates of or close to 100%. As a result, recent classifications have downgraded several low-grade malignant cutaneous lymphomas to lymphoproliferative disorder (LPD). Both the 5th edition of the WHO classification (2022) and the 2022 International Consensus Classification (ICC) of mature lymphoid neoplasms reclassified PCSM-TCL as primary cutaneous CD4+ small/medium T-cell LPD and primary cutaneous acral CD8+ T-cell lymphoma as primary cutaneous acral CD8+ T cell LPD. While the 2022 ICC introduced the term "primary cutaneous marginal zone LPD," in the 5th edition of the WHO classification PCMZL is maintained. In this review we describe the background and rationale of the continually changing terminology of these conditions and discuss the clinical consequences of downgrading malignant lymphomas to LPDs.

Fine-Needle Aspiration Biopsy as a Diagnostic Modality for Orbital Adnexal Lymphoma

Introduction: The aim of this study was to evaluate fine-needle aspiration biopsy (FNAB) as a diagnostic tool for lymphoproliferative orbital lesions in light of recent improvements in cytomorphological and immunologic analyses. Method: Retrospective case series including all orbital FNABs with a lymphoproliferative outcome at Karolinska University Hospital, Stockholm, Sweden during the period 2005–2015. Results: Of the 38 patients included, 31 (82%) were conclusively diagnosed as having lymphoma according to the first FNAB. Disease in 20 patients (65%) could be subclassified. The diagnosis in 7 patients (18%) was either inconclusive, suggestive of lymphoma, or reactive lymphatic infiltrate. These 7 patients were re-investigated, and the initial suspected diagnosis of malignant lymphoma was confirmed in four. Two of the remaining 3 patients were initially diagnosed as having non-lymphoproliferative disease; however, this was later changed to a lymphoproliferative diagnosis following reinvestigation, while the results of both reFNAB and incisional biopsy were inconclusive in the third. Conclusion: In the majority of the 38 patients, a definitive diagnosis of lymphoma could be made based on FNAB alone, using cytomorphological and immunological workup, and subclassification was possible in 20 patients (65%). Primary low-grade malignant orbital lymphomas are traditionally treated with low-dose radiotherapy regardless of subtype, and incisional biopsy was not needed to initiate treatment. Our findings suggest that FNAB is a valid first option for the diagnosis of suspected orbital lymphomas due to the minimal risk of complications compared to incisional biopsy, and the fact that it can be performed as an outpatient procedure with no anesthesia.

A Case of Subglottic Marginal B-Cell Lymphoma With Laryngeal Amyloidosis.

Laryngeal mucosa-associated lymphoid tissue (MALT) is an extra-nodal margin zone B-cell lymphoma (MALT lymphoma) and a low-grade malignant lymphoma with a low incidence, the etiology of the condition remains obscure, and the process of differential diagnosis poses a significant challenge, so it is easy to miss diagnosis and misdiagnosis clinically. The present article presents a clinical case study of a patient who was diagnosed with subglottic MALT lymphoma, which was associated with laryngeal amyloidosis. The patient underwent a successful treatment regimen comprising carbon dioxide laser and radiotherapy. In addition, the article provides an overview of relevant literature that can aid in the diagnosis and management of this rare disease. The study is expected to contribute to the existing body of knowledge on the treatment of subglottic MALT lymphoma and laryngeal amyloidosis.

Pseudolymphoma to Lymphoma: A Case of Chronic Reactive Lymphoid Hyperplasia Transforming to Primary Cutaneous Marginal Zone Lymphoma.

Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade malignant B-cell lymphoma that originates from the skin. It often presents as erythematous solitary or multiple papules, nodules, and/or plaques. It is one of the 3 main subtypes of primary cutaneous B-cell lymphomas. PCMZLs are believed to develop from chronic antigenic stimulation such as from tick-borne bacteria, vaccines, tattoo pigment, or other foreign body. In addition, cutaneous lymphoid hyperplasia, a documented precursor to malignant PCMZL, often presents in response to areas of chronic inflammation. Cutaneous lymphoid hyperplasia and PCMZL share several clinical and histological similarities that require clinicopathologic suspicion, immunohistochemical ancillary studies, and histopathologic analysis to accurately differentiate the 2 entities. Although gene rearrangement studies have historically been of limited value in the diagnosis of PCMZL, recent studies investigating molecular markers have identified the presence of multiple genetic abnormalities that have helped to better characterize the disease and aid in diagnosis. In addition, newer studies have found associations between PCMZL and gastrointestinal disorders, including Helicobacter pylori and inflammatory bowel disorders. In this article, we describe a case of a 56-year-old patient with a history of ulcerative colitis presenting with chronic reactive lymphoid hyperplasia that transformed to primary cutaneous marginal zone lymphoma.

Primary non-Hodgkin lymphoma of the chiasm and optic tract in a nonimmunocompromised patient: illustrative case.

Chiasmatic and optic track lymphoma as the primary lesion of the central nervous system (CNS) is extremely rare. The authors report a case of a previously healthy 62-year-old woman who presented with quick and progressive visual impairment leading to bilateral blindness. Brain imaging studies suggested glioma or lymphoma of the chiasm and the posterior visual pathway. Postoperative examination revealed low-grade malignant B-cell lymphoma. No evidence of extracranial lymphoma was found, so a final diagnosis of primary CNS lymphoma (PCNSL) was made. To the authors' knowledge, PCNSL confined to the optic chiasm has rarely been reported in nonimmunocompromised patients. The present case of lymphoma affecting the optic chiasm and optic tract is extremely rare.

Characterization of molecular genetics and clinicopathology in thymic MALT lymphoma

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma is a type of low-grade malignant B-cell lymphoma. The aim of this study was to investigate the clinicopathological characteristics of thymic MALT lymphoma. We analyzed the clinical, morphological, immunophenotypical, cytogenetic, and molecular characteristics of 11 cases of thymic MALT lymphoma. The relevant literature was also reviewed. The median age of the 11 patients was 50 (range: 33-60). There was a female predominance with a female-to-male ratio of 10:1. Three patients presented with Sjögren syndrome, autoimmune thrombocytopenia purpura, and type B1 thymoma, respectively. Microscopically, thymic MALT lymphoma was characterized by epithelium-lined cysts that were surrounded by small lymphocytes, centrocyte-like cells, and monocytoid B-cells. Plasmacytic differentiation was observed in two cases. The tumor cells expressed CD20, CD79α, and BCL2. Clonal immunoglobulin genes were detected in all 8 examined cases. Fluorescence in situ hybridization (FISH) for 18q21 was performed in 7 cases, and no translocations involving 18q21 were found. Targeted gene sequencing was performed in five cases with available DNA samples, and TNFAIP3, CARD11, IGLL5, and CCND3 mutations were identified. Thymic MALT lymphoma is a rare type of B cell malignancy with a female predominance and excellent clinical outcomes. Molecular aberrations involving the NF-κB pathway are frequent in thymic MALT lymphoma, suggesting that dysregulation of the NF-κB pathway is an important mechanism underlying the pathogenesis of thymic MALT lymphoma.

A case of Barrett\u2019s esophageal cancer with gastric mucosa-associated lymphoma

BackgroundAlthough the first-line therapy for early-stage gastric mucosa-associated lymphoid tissue lymphoma is the eradication of Helicobacter pylori, the effect of eradication in Helicobacter pylori-negative cases is unclear. In this case report, we describe a surgical option for a case of Barrett’s esophageal cancer with concurrent gastric mucosa-associated lymphoid tissue lymphoma.Case presentationA 79-year-old man was admitted to our hospital with Barrett’s esophageal cancer and gastric mucosa-associated lymphoid tissue lymphoma. Initially, we performed endoscopic submucosal dissection for Barrett’s esophageal cancer. Since residual tumor was observed after the endoscopic submucosal dissection, we performed an esophagectomy with two-field lymph node dissection, which was followed by placement of a gastric conduit via the posterior mediastinal route. He was discharged 14 days after surgery. Although no additional treatment exists for mucosa-associated lymphoid tissue lymphoma, no recurrent disease has been detected to date.ConclusionAn option to use a portion of the stomach with low-grade malignant mucosa-associated lymphoid tissue lymphoma as a conduit after esophagectomy was suggested.

Targeted Metabolomics Analysis in Follow-up of Classic and Mild PKU using LC-ESI-MS/MS: Assessment of Metabolic Control through PHE/TYR ratio

MALT lymphoma, also referred to as extra nodal marginal zone B-cell lymphoma, is a low-grade malignant non-Hodgkin lymphoma...

Hyperpigmented mycosis fungoides: A rare entity

Dear Editor, A 67-year-old man of Kosovar-Albanian ethnic origin (skin phenotype IV) presented to our dermatology clinic with generalized hyperpigmented patches and plaques all over the body, so-called melanoerythroderma (Figure 1). The lesions, which first appeared nearly six years ago, developed gradually; they were diagnosed as mycosis fungoides (MF), and were subsequently treated only with topical corticosteroids. We performed further examinations upon admission to our department. Relevant laboratory parameters - blood cell count, LDH, urinalysis, and serum chemistry - were within normal limits. Endocrinological examination excluded Addison disease, and the patient was not receiving any drugs that could cause skin hyperpigmentation. Chest-abdomen-pelvis computed tomography (CT) scan and sternal puncture were normal. Flow cytometric immunophenotyping revealed less than 5% aberrant T-cells. Histopathology and immunohistochemistry of skin specimens revealed lichenoid infiltration of small- to medium-sized atypical T-lymphocytes within the upper dermis, epidermotropic lymphocytes with several Pautrier's microabscesses (Darier's nests), pigment incontinence, abundant melanophages in the papillary dermis (Figure 2, a, b), and the T-cell CD4+CD7-CD8+ phenotype (Figure 2, c, d). Based on the clinical picture, histopathology, and immunohistochemistry the diagnosis of hyperpigmented mycosis fungoides (MF) stage IIIA (T4N0M0B0) was established. Skin-oriented therapy (retinoids-PUVA) resulted in slight improvement. Hyperpigmented MF is a rare, uncommon, clinical variant of MF, with a predilection for dark-skinned people (1). Only a few cases of hyperpigmented MF have been reported so far, and our case being one of them (2-5). Hyperpigmented patches or/and plaques dominate the clinical picture. Hyperpigmented MF is characterized by a predominantly CD8+ epidermotropic T-cell phenotype, although different phenotypes have been reported (CD4+ or CD4-CD8-) (2). Histopathologically, interface changes, pigment incontinence and melanophages are usually found in addition to the classical findings of early MF (1). The exact mechanism of hyperpigmentation is not well understood. Hyperpigmented MF had an indolent course in most reported cases, and skin-directed therapy is therefore the treatment of choice. Although MF and its hyperpigmented variant is a lymphoma of low-grade malignancy, large-cell transformation (CD30+) of hyperpigmented MF can occur (1). These rare cases of special clinical MF variants are extremely valuable and can help us investigate and understand the pathophysiology of the disease. Treatment and close follow-up is mandatory in the hyperpigmented variant of MF.

Cutaneous pseudolymphoma: An enigma

Cutaneous pseudolymphoma, also called lymphoid infiltrates of the skin mimicking lymphomas, is defined as reactive polyclonal benign lymphoproliferative process predominantly composed of either B-cells or T-cells, localized or disseminated. A 62-year-old male presented with multiple asymptomatic swellings over the posterior aspect of the left ear of 1-year duration. On examination, multiple nodules were present over the left retroauricular area, 2 of which were skin colored, firm, and nontender. Histopathology revealed pseudolymphoma with the features of a dense diffuse and nodular infiltrate of small and large lymphocytes and histiocytoid cells involving the whole of reticular dermis and extending to subcutis. A patch test was done using Indian standard battery series showed positive reaction - 2+ for paraphenylenediamine. A diagnosis of cutaneous pseudolymphoma was made. The lesions were treated with intralesional Triamcinolone acetonide injection 10 mg/ml for 2 sittings, 3 weeks apart following which there was remission of the nodules. Pseudolymphomas are benign but persistent lymphoid proliferations in the dermis, which may be difficult to distinguish from a low-grade malignant lymphoma. Pseudolymphomas are classified according to the histological components into B-cell and T-cell variants. So far no case of cutaneous pseudolymphoma suggestive of insect bite with coincidental paraphenylenediamine allergy has been reported. Hence, this case is reported as its histopathology was suggestive of pseudolymphoma secondary to insect bite reaction.

Ocular Adnexal Lymphoma: Clinical Presentation, Diagnosis, Treatment and Prognosis

Ocular adnexal lymphoma (OAL) is a rare tumor with an incidence of 0.2 per 1,00,000 individuals. However, it constitutes the most common orbital tumor especially in older population with an incidence ranging from 11% to 24%. OAL can involve conjunctiva, orbital soft tissues, eyelid, or adnexal structures such as lacrimal gland and lacrimal drainage system. The most common primary OAL is low-grade malignant extranodal marginal zone B-cell lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT) type. Though various classification and staging systems have been proposed for OAL, currently, American joint committee on cancer (AJCC) TNM (tumor node metastasis) staging is most commonly used. Most commonly, conjunctival lymphoma typically presents as ‘salmon-colored patch’ and orbital lymphoma presents as proptosis, which on imaging shows characteristic appearance of molding around the globe without any globe indentation or bone erosion. Orbital radiotherapy and systemic chemotherapy forms the mainstay of treatment. Newer treatment modalities include immunotherapy with interferon and anti-CD20 antibody rituximab that have been shown to be useful either when used alone or in combination with chemotherapy or as radio-immunotherapy.

Abstract B195: Serum levels of CXCL10 and CXCL11 elevate after skin rash induction, the commonest adverse event by bendamustine in the treatment of malignant lymphoma

Abstract Introduction: Bendamustine has been recently approved for the treatment of low-grade malignant lymphoma. In spite of the better efficacy, skin rash is the commonest adverse event by bendamustine in the treatment of malignant lymphoma. To understand the mechanism of the skin rash, we prospectively examine the relationship between skin rash and cytokines/chemokines. Methods: After informed consent to the patients, who would received bendamustine (B) or bendmustine+rituximab (BR), the sera were collected before and after the treatment of B or BR. The Cytokine/Chemokine array was performed by Bio-Plex system. Results: 20 patients with FL (n = 18), MCL (n = 1), and low-grade B-cell lymphoma (n = 1) were enrolled in this study. All patients wee treated with B or BR regimen. Nine patients showed skin rash after the treatment of bendamustine. In Cytokine/Chemokine array, serum levels of CCL22 and CCL25 before the treatment were significantly higher in skin rash group (p = 0.010738476 and p = 0.030366028, respectively), and serum levels of CXCL10 and CXCL11 after skin rash were higher significantly (p = 0.000923527 and p = 0.014890795, respectively). Conclusion: The commonest adverse event by bendamustine and CXCL10 and CXCL11might contribute to induction of skin rash by bendamustine, and inhibition of their receptor, CXCR3 may be helpful for the treatment of skin rash by bendamustine Citation Format: Yasuhito Terui, Yuji Mishima, Kiyohiko Hatake. Serum levels of CXCL10 and CXCL11 elevate after skin rash induction, the commonest adverse event by bendamustine in the treatment of malignant lymphoma. [abstract]. In: Proceedings of the AACR-NCI-EORTC International Conference: Molecular Targets and Cancer Therapeutics; 2015 Nov 5-9; Boston, MA. Philadelphia (PA): AACR; Mol Cancer Ther 2015;14(12 Suppl 2):Abstract nr B195.

Diagnosis and treatment of lymphomatoid papulosis

Lymphomatoid papulosis (LyP), a low-grade malignant T-cell lymphoma, belongs to the spectrum of cutaneous CD30+ lymphoproliferative diseases. It clinically manifests as necrotic papules or nodules, and superficial scar may be left after regression in some cases. Since lesions tend to recur and heal spontaneously, the lesions at different stages of evolution usually coexist. The histologic manifestations of LyP are various, and usually present as a spectrum. Pathologically, LyP is characterized by the presence of CD30 + neoplastic cells, and can easily be mistaken for other benign or malignant diseases. At present, no specific treatments for LyP are available, and no treatments can alter the natural history of LyP. In general, active treatments are not recommended. LyP has a good prognosis, but may be followed by other malignant lymphomas, so a long-term follow-up is warranted. Key words: Lymphomatoid papulosis; Lymphoma, T-Cell; Diagnosis; Pathology, clinical; Immunophenotyping

Treatment advances in mucosa-associated lymphoid tissue lymphoma

Mucosa-associated lymphoid tissue (MALT) lymphoma originates from outside of the lymph nodes, which is a kind of low-grade malignant lymphoma and the most type of marginal zone lymphoma (MZL). It accounts for about 30 %-50 % of the indolent NHL. The onsets and therapies of MALT lymphoma are diverse. This article focuses on the recent therapeutic advances of MALT lymphoma. Key words: Lymphoma, non-Hodgkin; Mucosa-associated lymphoid tissue lymphoma; Drug therapy; Radiotherapy; Surgical procedures, operative

Medical thoracoscopy in MALT lymphoma causing pleural effusion: A case report.

Mucosa-associated lymphoid tissue (MALT) lymphoma is a form of low-grade malignant B-cell extranodal non-Hodgkin's lymphoma. It is classified as marginal-zone lymphoma and represents less than 1% of all lung cancer. We describe a case of MALT lymphoma limited exclusively to the lung that came to our attention with infective pleural effusion and concomitant lung consolidation of the left lower lobe. Our case demonstrates that MALT can begin with an acute clinical presentation. The clinical scenario, with fever, parietal chest pain, and leukocytosis, suggested an infective process. Radiological and sonographic examinations and the endoscopic aspect during medical thoracoscopy (MT) were typical of an infective etiology. The histological outcome of non-specific inflammatory pleuritis confirmed our suppositions. However, the missing resolution of lung consolidation after several weeks led us to an alternative diagnosis. Parenchymal biopsies obtained by bronchoscopy allowed us to reach the correct diagnosis: MALT lymphoma limited to the lung.

A case report of primary cutaneous marginal zone lymphoma treated with intralesional steroids

Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade malignant B-cell lymphoma that appears in the skin without any extracutaneous manifestations. Therapeutic mainstays for PCMZL have primarily included radiotherapy and surgery. Intralesional steroids have been found to resolve the lesions caused by PCMZL, but there is a dearth of literature regarding this therapy indicating that this is not a commonly favored treatment option. We present a case of 60-year-old woman who presented with PCMZL on her right eyebrow. Three years later, after two courses of radiation and one relapse, the patient presented with new lesions on both arms, consistent once again with PCMZL. At this time, therapy with nine rounds of intralesional triamcinolone was attempted. After each round of triamcinolone, gradual improvement of the lesions was noted until complete resolution occurred. This case reminds practitioners that intralesional corticosteroids can be effectively and safely used to treat localized PCMZL. Intralesional steroids are less invasive, cheaper, and easier to administer than the majority of other recommended therapies for PCMZL. Thus, although they are often overlooked, intralesional corticosteroids should be more often considered when developing a treatment plan for localized PCMZL.

Serum Levels of CCL22 and CCL25 Might Predict Skin Rash Induction、the Common Adverse Event By Bendamustine Therapy in Malignant Lymphoma

Introduction: Bendamustine has been recently approved for the treatment of low-grade malignant lymphoma. In spite of the better efficacy, skin rash is the commonest adverse event by bendamustine in the treatment of malignant lymphoma. To understand the mechanism of the skin rash, we prospectively examine the relationship between skin rash and chemokines. Methods: After informed consent to the patients, who would received bendamustine (B) or bendmustine+rituximab (BR), the sera were collected before and after the treatment of B or BR. Cytokine/Chemokine array was performed by Bio-Plex system. Results: 20 patients with FL (n=18), MCL (n=1), and low-grade B-cell lymphoma (n=1) were enrolled in this study. All patients wee treated with B or BR regimen. 9 patients showed skin rash after the treatment of bendamustine. In Cytokine/Chemokine array, serum levels of CCL22 and CCL25 before the treatment were significantly higher in skin rash group (p=0.010738476 and p=0.030366028, respectively) (Figure 1 and 2), and serum levels of CXCL10 and CXCL11 after skin rash were higher significantly (p=0.000923527 and p=0.014890795, respectively). Conclusion: Serum levels of CCL22 and CCL25 might predict skin rash inductionAthe commonest adverse event by bendamustine and CXCL10 and CXCL11might contribute to induction of skin rash by bendamustine. [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Clinical Analysis of Primary Conjunctival Malignant Lymphoma

Purpose: To investigate clinical outcomes, response to treatment, and the related factors of recurrence and complication, following treatment of primary conjunctival malignant lymphoma. Methods: The medical records of 39 patients diagnosed with primary conjunctival malignant lymphoma between January 2005 and June 2013 were retrospectively reviewed. Results: The mean age of patients was 51.1 years old. The most common presenting symptom was hyperemia (33.3%). The most common anatomical location of the mass was the fornix (38.5%) and 25.6% patients had bilateral involvement. Histopathologically, mucosa-associated lymphoid tissue (MALT) lymphoma (92.3%) was the most common subtype. Every patient underwent radiotherapy (92.3%) or chemotherapy (7.7%) after surgical excision and had 100% complete remission. Local or systemic recurrence was observed in 15.4% of patients after treatment (mean 8.0 ± 3.3 months), but was completely remitted after additional radiation or chemotherapy. International prognostic index and location of tumor were significantly related factors for predicting tumor recurrence (p < 0.01, p = 0.02, respectively). Dry eye disease (DED) was the most common ocular complication (44.4%) after radiotherapy. Total radiation dosage and location of tumor were significantly associated factors for developing DED after radiotherapy (both p = 0.04). Conclusions: Most conjunctival malignant lymphomas were low grade malignant MALT lymphomas that responded well to treatment but recurrence of tumor and development of DED after radiotherapy were common, therefore predicting and preparing these lymphomas is important. J Korean Ophthalmol Soc 2014;55(9):1298-1306