Pediatric-type follicular lymphoma in a Crohn’s disease patient receiving anti-α4β7-integrin therapy:A case report

BACKGROUND Patients with autoimmune conditions receiving immunosuppressants are at risk of non-Hodgkin lymphomas(NHL).Vedolizumab(anti-α4β7-integrin antibody),a treatment-of-choice for Crohn’s disease(CD),reduces inflammatory lymphocyte trafficking into the intestinal mucosa.This effect is believed to be confined to the colon.CASE SUMMARY We report the case of a CD patient on vedolizumab for five years who developed pediatric-type follicular lymphoma.Work-up prior to therapy revealed a reduction in circulating T-lymphocytes and their suppressed response to mitogens.Rituximab,cyclophosphamide,vincristine,and prednisone chemoimmunotherapy resulted in durable lymphoma remission,and vedolizumab treatment was continued.While the patient’s T-lymphocyte population and immunoglobulin production recovered,the T-lymphocyte mitogen response remained suppressed.CONCLUSION This patient’s NHL may be linked to receiving anti-α4β7 therapy.Further research could be beneficial to determine if proactive surveillance for NHL and other systemic diseases is indicated in patients on vedolizumab.

Epstein Barr virus-positive mucocutaneous ulcer of thecolon associated Hodgkin lymphoma in Crohn＇s disease

Epstein Barr virus （EBV） positive mucocutaneous ulcers（EBVMCU） form part of a spectrum of EBV-associatedlymphoproliferative disease. They have been reportedin the setting of immunosenescence and iatrogenicimmunosuppression, affecting the oropharyngeal mucosa,skin and gastrointestinal tract （GIT）. Case reports andseries to date suggest a benign natural history respondingto conservative management, particularly in the GIT. Wereport an unusual case of EBVMCU in the colon, arisingin the setting of immunosuppression in the treatment ofCrohn＇s disease, with progression to Hodgkin lymphoma18 mo after cessation of infliximab. The patient presentedwith multiple areas of segmental colonic ulceration,histologically showing a polymorphous infiltrate withEBV positive Reed-Sternberg-like cells. A diagnosisof EBVMCU was made. The ulcers failed to regressupon cessation of infliximab and methotrexate for 18mo. Following commencement of prednisolone for herCrohn＇s disease, the patient developed widespreadHodgkin lymphoma which ultimately presented as alife-threatening lower GIT bleed requiring emergencycolectomy. This is the first report of progression ofEBVMCU to Hodgkin lymphoma, in the setting of ongoingiatrogenic immunosuppression and inflammatory boweldisease.

Febrile cholestatic disease as an initial presentation of nodular lymphocyte-predominant Hodgkin lymphoma

Febrile cholestatic liver disease is an extremely unusual presentation of Hodgkin lymphoma(HL).The liver biopsy of a 40-year-old man with febrile episodes and cholestatic laboratory pattern disclosed an uncommon subtype of HL,a nodular lymphocyte-predominant HL(NLPHL).Liver involvement in the early stage of the usually indolent NLPHL's clinical course suggests an aggressiveness and unfavorable outcome.Emphasizing a liver biopsy early in the diagnostic algorithm enables accurate diagnosis and appropriate treatment.Although rare,HL should be considered in the differential diagnosis of cholestasis.

Risks and diagnosis of coronary artery disease in Hodgkin lymphoma survivors

Higher mortality rates are reported because of cardiovascular diseases in individuals living in industrialized areas of the World.In cancer patients,cardiotoxic chemotherapeutic agents and/or mediastinal radiotherapy are additional risk factors for the development of coronary artery disease.An improved survival rate for patients with Hodgkin lymphoma was reported in recent decades.Determining and handling the long-term effects of cancer treatment have become more important nowadays,parallel to the good results reached in survival rates.Mediastinal radiotherapy and cardiotoxic chemotherapeutic agents are routinely used to treat Hodgkin lymphoma but are commonly associated with a variety of cardiovascular complications.Drugs used in cancer treatment and radiotherapy may cause deleterious effects on contractile capacity and conduction system of the heart.Approximately ten years after the completion of all therapies,the cardiovascular disease risk peaks in patients who survived from Hodgkin lymphoma.The value of coronary computed tomography angiography as a diagnostic tool in determining coronary artery disease as early as possible is underlined in this review,in patients who are in remission and carry the risk of coronary artery disease probably because of chemo/radiotherapy used in their treatment.Survivors of Hodgkin lymphoma especially treated with combined chemoradiotherapy at younger ages are candidates for coronary computed tomography angiography.

NATIONAL MULTICENTER COHORT HISTOPATHOLOGICAL RETROSPECTIVE STUDY OF HODGKIN' S DISEASE (HD)

Retrospective and histopathological study on 1096 cases of Hodgkin' s disease (HD) showed that the incidence of HD in lymph node biopsy was 3. 87%, the ratio between HD and NHL in 1960s, 1970s and 1980s were quite similar. Among 1083 cases of HD with sex record, 787 were male, and 296 were female. The ratio of male to female was 2. 65: 1, and the mean age was 31 yean. According to Rye' s classification 274 were LP, 145 were NS, 511 were MC, 135 were LD and 19 were unclassified. The distribution of the four subtypes in this series showed that accounted LP for 25. 3%, NS for 13. 3%. MC for 47. 1% and LD for 12. 4%. There were three major differences: 1. NS was much less, 2. LD was higher, and 3. NS-G2 was much more, when compared to the figures of other representative studies in western countries. In regard to the pathological diagnosis, residue follicles could be obtained in 42. 8% of the HD lesions, and therefore, the presence of residue follicles would not exclude the positive diagnosis of HD. Typical R-S cells could be found only in 39. 2% of these cases. The recently reported ' syncytial variant' change was not a special feature of NS in our study. Primary extranodal HD was extremely rare in this study, and the mortality of HD in China seemed much higher than that in the advanced countries.

Kikuchi-Fujimoto disease:A comprehensive review

Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss,splenomegaly,leucopenia,and elevated erythrocyte sedimentation rate feature in severely affected patients.Cutaneous involvement occurs in about 30%-40%of cases as facial erythema and nonspecific erythematous papules,plaques,acneiform or morbilliform lesions of great histologic heterogeneity.Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede,develop subsequently,or sometimes be associated concurrently with Kikuchi-Fujimoto disease.It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis,cat-scratch disease,Sweet’s syndrome,Still’s disease,drug eruptions,infectious mononucleosis,and viral or tubercular lymphadenitis are other common differentials.Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value.Since its diagnosis is exclusively from histopathology,it needs to be evaluated more carefully;an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials.Its treatment with systemic corticosteroids,hydroxychloroquine,or antimicrobial agents mostly remains empirical.The article reviews clinicoepidemiological,diagnostic,and management aspects of KFD from the perspective of practicing clinicians.

A case of rapid growing colonic NK/T cell lymphoma complicated by Crohn's disease

A 37-year-old man developed abdominal pain and bloody diarrhea 11 months before admission. The colonoscopy revealed multifocal ulcers in the colon. Histology showed active chronic inflammation. Although anti-tuberculosis medication was effective, his symptoms repeated 2 months later. The subsequent colonoscopy revealed more extensive irregular ulcers than before, and he was clinically suspected with intestinal malignant lymphoma. He underwent subtotal colectomy and was histologically suggested Crohn＇s disease, then 5-aminosalicylic and a combination of prednisone and azathioprine were administered in succession postoperatively, but they achieved minimal relief of symptoms for a period of 7 months. The third colonoscopy showed a large irregular ulcer in the ileocolon stomas, and primary colonic NK/T cell lymphoma was diagnosed through histological and immunophenotypic studies. Malignant lymphoma should be taken into consideration when clinically diagnosed Crohn＇s disease was refractory to medication or when its clinical course became aggressive.

Nodal peripheral T-cell lymphomas in the new classification systems

Peripheral T-cell lymphomas(PTCLs)encompass a biologically diverse group of non-Hodgkin lymphomas derived from mature T-lymphocytes.Most PTCLs present as nodal diseases and include several subtypes characterized by distinct clinical and pathologic features,and will be the focus of this editorial.The PTCL group presenting as rare distinctive extranodal diseases will not be discussed.While T-cell neoplasms,like B-cell lymphomas,recapitulate stages of normal differentiation,the biology is notably intricate and exhibits remarkable plasticity.

Diffuse large B-cell lymphoma successfully treated with amplified natural killer therapy alone: A case report

BACKGROUND The prognosis of patients with advanced diffuse large B-cell lymphoma(DLBCL)is poor,with a 5-year survival rate of approximately 50%.The mainstay of treatment is multidrug combination chemotherapy,which has been associated with serious side effects.Amplified natural killer(ANK)cell therapy amplifies and activates natural killer(NK)cells to attack only malignant tumors.As ANK cells attack programmed death ligand 1(PD-L1)-positive tumor cells,ANK therapy is considered effective against adult T-cell lymphoma and malignant lymphoma.CASE SUMMARY Herein,we report a case of an older patient with advanced DLBCL who was successfully treated with ANK immunotherapy.A 91-year-old female visited our hospital with sudden swelling of the right axillary lymph node in April 2022.The patient was diagnosed with stage II disease,given the absence of splenic involvement or contralateral lymphadenopathy.ANK therapy was administered.Six rounds of lymphocyte sampling were performed on July 28,2022.To reduce the occurrence of side effects,the six samples were diluted by half to obtain 12 samples.Cultured NK cells were administered twice weekly.The treatment efficacy was evaluated by performing computed tomography and serological tests every 1 or 2 mo.The treatment suppressed lesion growth,and the antitumor effect persisted for several months.The patient experienced mild side effects.PD-L1 immunostaining was positive,indicating that the treatment was highly effective.CONCLUSION ANK therapy can be used as a first-line treatment for malignant lymphoma;the PD-L1 positivity rate can predict treatment efficacy.

Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma masquerading as Behcet’s disease: A case report and review of literature

BACKGROUND Anaplastic large cell lymphoma(ALCL)is a CD30-positive T cell lymphoma,a rare type of non-Hodgkin lymphoma.The current World Health Organization classification system divides ALCLs into anaplastic lymphoma kinase(ALK)-positive and ALK-negative groups.ALCL rarely presents in the gastrointestinal tract.CASE SUMMARY A 54-year-old male was admitted to the department of gastroenterology for abdominal pain.He presented with lower abdominal pain,diarrhea and recurrent oral and penile ulcers.He was misdiagnosed with Behcet's disease and treated with prednisone.But after one month,he was hospitalized in another hospital for reexamination.This time,the lesion on the penis was biopsied for histological examination.The final pathological diagnosis was ALCL,ALKnegative.The patient was treated with cyclophosphamide,doxorubicin,vincristine,prednisolone chemotherapy.However,he died within one month.CONCLUSION Gastrointestinal ALCL needs to be considered in the differential diagnosis to avoid delaying treatment.Repeated biopsy is the most important for early diagnosis and treatment.

Pediatric Non-Hodgkin Lymphoma: A Retrospective 7-Year Experience in Children &Adolescents with Non-Hodgkin Lymphoma Treated in King Fahad Medical City (KFMC)

Background: Non-Hodgkin’s lymphoma is an aggressive malignant disease in children and adolescents. Although it is the fourth most common malignancy in Saudi children as reported in Saudi cancer registry, less information is available about pediatric Non-Hodgkin lymphoma and its outcome in Saudi Arabia. Study Objectives: To provide demographic data, disease characteristics, treatment protocol, toxicity and outcome of treatment in children & adolescents with Non-Hodgkin’s lymphoma treated at KFMC. This study will form base line for future studies about pediatric Non-Hodgkin’s lymphoma in KFMC, which may help to improve outcome for children with cancer in Saudi Arabia. Study Patients and Method: We retrospectively analyzed 28 children and adolescents diagnosed to have Non-Hodgkin’s lymphoma at KFMC between December 2006 and December 2013, followed-up through June 2014. Results: Of the 28 patients, 10 (35.7%) girls and 18 (64.3%) boys, the male-to-female ratio was 1.8;1. The median age at time of diagnosis was 6.4 years old (range 2.0 to 13.0 years old). The majority of patients (64.3%) were aged between 5 and 12 years old. Burkitt’s lymphoma BL/BLL was the most common pathological subtype (60.7%), and DLBCL was the second most common subtype (21.4%). Abdominal and Retroperitoneal involvement was the most common primary site (78.6%) including the ileocaecal region. Most of the children presented with advanced Stage III and IV (75%), Cytogenetic study which screens specifically for the t (8;14) (q24;q32) a characteristic genetic feature of Burkitt’s Lymphoma was obtained from 21 patients, variant rearrangement was observed in 3/21 samples and complex chromosomes karyotype in addition to IGH/MYC rearrangement was observed in 2/21 samples. Those patients presented with very aggressive lymphoma and combined BM and CNS involvement. We use the French-American-British Mature B-Cell Lymphoma 96 Protocol (FAB LMB 96) for treatment fornewly diagnosed Mature B-Cell type NHL and high risk ALL CCG 1961 Protocol for lymphoblastic lymphoma and international Anaplastic Large Cell Lymphoma 99 Study Protocol for ALCL. The median follow-up in patients not experiencing an adverse event was 53.1 months. The estimated 3-year EFE and OS rates in the entire cohort of patients with newly diagnosed NHL treated in the KFMC were 85.2% and 89.2% respectively;Overall survival (OS) rate of patients with mature B-cell-NHL was 88.9%. Conclusion: The outcomes and survival in our small series appeared to be excellent compared with those reported in other international trials even though most of our patients presented in advanced stage of the disease. We feel that the importance of the current study is to document the relative distribution of various types of pediatric non-Hodgkin’s lymphomas and age-specific distribution in different Histological subtypes.

Kawasaki disease, <i>Mycoplasma pneumoniae</i>infection and anaplastic large cell lymphoma: An uncommon association

Kawasaki disease (KD) is an acute febrile systemic vasculitis occurring predominantly in young children less than 5 years of age. Although imperfectly known, the aetiopathogenesis of KD would be secondary to immunological abnormalities that could constitute a favourable terrain for neoplasms. We report on a case in a 2-year-old girl who presented clinical manifestations compatible with Kawasaki disease complicated by coronary aneurysm. Aetiopathological investigations revealed M. pneumoniae infection as specific IgM were present in the serum (Elisa). The patient was initially treated by intravenous immunoglobulins (IVIG) and aspirin to anti-inflammatory dose. Following a few days of desquamation, resolution of the symptomatology occurred. Four weeks later she developed disseminated tumorous syndrome. Lymph node biopsy revealed massive infiltration by large cells lymphomatous proliferation. Histologic and immunophenotypic findings were characteristic of ALK-1+ anaplastic large cell lymphoma. Four weeks later, the patient died from a severe nosocomial infection complicated by septic shock. Our observation is the first cases describing the association between anaplastic large cell lymphoma, KD and M. pneumoniae. Immunologic disorder due to KD and M. pneumoniae infection may play probably a central role for malignancy.

CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease: multi-institutional case series

AIM:To report CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease(IgG4-MALT lymphoma),a rare but clinically important complication of ocular adnexal IgG4-related disease.METHODS:We retrospectively reviewed all cases of histologically confirmed ocular adnexal IgG4-related disease at three ter tiary and one secondary referral centers,between February 2003 and December 2016.Seven cases of histopathologically diagnosed IgG4-MALT lymphoma were identified.CT and MR images were analyzed by consensus of two experienced head and neck radiologists.RESULTS:Lacrimal glands were the main site of involvement in all seven patients.The lesions typically showed well-demarcated margins,iso-to hyperattenuation on precontrast CT,T2 hypo-to isointensity,T1 isointensity,and homogenous internal architecture with homogenous enhancement pattern.Lesions were mostly hyperdense and isointense to normal extraocular muscles on postcontrast CT and MR images,respectively.CONCLUSION:Unlike in typical ocular adnexal IgG4-related disease,T2 isointensity and hyperattenuation on precontrast CT images were noted in some IgG4-MALT lymphoma cases.Although the findings may be nonspecific,the possibility of accompanying MALT lymphoma may need to be considered,when ocular adnexal lesions in patients clinically suspected of having IgG4-related disease are refractory to glucocorticoids and show T2 isointensity and hyperattenuation on precontrast CT for the optimal management of the patients.However,this is a case series of a very rare complication of ocular adnexal IgG4-related disease,and thus caution is warranted to generalize the conclusion.

Potential model for differential diagnosis between Crohn's disease and primary intestinal lymphoma

AIM To evaluate the usefulness of different parameters to differentiate Crohn's disease(CD) from primary intestinal lymphoma(PIL).METHODS The medical records of 85 patients with CD and 56 patients with PIL were reviewed retrospectively. Demographic, clinical, laboratory, endoscopic, and computed tomographic enterography(CTE) parameters were collected. The univariate value of each parameter was analyzed. A differentiation model was established by pooling all the valuable parameters. Diagnostic efficacy was analyzed, and a receiver operating characteristic(ROC) curve was plotted.RESULTS The demographic and clinical parameters that showed significant values for differentiating CD from PIL included age of onset, symptom duration, presence of diarrhea, abdominal mass, and perianal lesions(P < 0.05). Elevated lactate dehydrogenase and serum β2-microglobulin levels suggested a PIL diagnosis(P < 0.05). The endoscopic parameters that showed significant values for differentiating CD from PIL included multiple-site lesions, longitudinal ulcer, irregular ulcer,and intraluminal proliferative mass(P < 0.05). The CTE parameters that were useful in the identification of the two conditions included involvement of ≤ 3 segments, circular thickening of the bowel wall, wall thickness > 8 mm, aneurysmal dilation, stricture with proximal dilation, "comb sign", mass showing the "sandwich sign", and intussusceptions(P < 0.05). The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of the differentiation model were 91.8%, 96.4%, 93.6%, 97.5%, and 88.5%, respectively. The cutoff value was 0.5. The area under the ROC curve was 0.989.CONCLUSION The differentiation model that integrated the various parameters together may yield a high diagnostic efficacy in the differential diagnosis between CD and PIL.

An uncommon clinical presentation of retroperitoneal non-Hodgkin lymphoma successfully treated with chemotherapy: A case report

We report the case of a patient affected by an extra-nodal non-Hodgkin lymphoma presenting as a unique, large retroperitoneal mass with an unusual clinical presentation mimicking gastric peptic or neoplastic disease. The patientwas successfully treated with a first generation therapy,CHOP modified regimen (cyclophosphamide 600mg/m^2 intravenously on d 1, epirubicin 55mg/m^2 intravenously on d 1, vincristine 1.2 mg/m^2 intravenously on d 1,prednisone 60 mg/m^2 on d 1-5), and a complete response was achieved. The (18)F-fluorodeoxyglucose positron emission tomography was used to assess the therapy outcome. A brief review of literature is provided.

STUDIES OF THE CHARACTERISTIC FEATURES OF KI-1 POSITIVE NON-HODGKIN'S LYMPHOMA

The clinical histopathological and immuno-phenotypic features in 5 patients with Ki-1 positive non-Hodgkin's lymphoma (NHL) were studied. When firstseen, 4 patients presented enlargement of superficiallymph nodes, with skin lesions in 2 patients. Two patientsin stage Ⅳ with fever, hepato-splenomegaly and bonemarrow invasion, died. Histologically, the tumor cellsshowed diffused or patchy hyperplasia. The cells wererelatively large in size, rich in basophilic or slightlyeosinophilic cytoplasm with irregular-shaped nuclei,prominent nucleoli, and distinct anaplasia andpleomorphism. Some of the cells looked very much likethe Reed-Sternberg cells. Multinucleated giant cells wereseen. Immunophenotypically, all the cells were CD30 (Ki-1) and CD25 (IL-2 receptor) positive but CD15 (Leu M1)negative. Thus, the 5 patients with Ki-1 positive NHLwere all of T cell type.

Primary effusion lymphoma-like lymphoma in a patient with inflammatory bowel disease

A 77-year-old man with inflammatory bowel disease(IBD)and who was treated with anti-tumor necrosis factor(TNF),6-mercaptopurine and corticosteroids,presented with primary effusion lymphoma-like lymphoma(PEL-like lymphoma)with massive ascites.The patient’s clinical course was complicated by acute renal insufficiency and hypotension,which led to death within 2 wk.In general,patients with IBD may have an increased risk for development of lymphoma,which is frequently associated with immunosuppressive and/or anti-TNF antibody therapies.PEL is a rare subset of lymphoma localized to serous body cavities,lacks tumor mass or nodal involvement,and is associated with infection by human herpes virus 8(HHV-8).Primary neoplastic effusion may also be present in patients with large B-cell lymphoma without evidence of human immunodeficiency virus or HHV-8 infections.This type of lymphoma is classified as PEL-like lymphoma.Both PEL and PEL-like lymphoma types have been reported in patients undergoing immunosuppressive therapy,but to the best of our knowledge,the case described herein represents the first PEL-like lymphoma occurring in a patient with IBD.

Lymphoma and cerebral vasculitis in association with X-linked lymphoproliferative disease

Lymphoma is seen in up to 30%of patients with X-linked lymphoproliferative disease(XLP),but cerebral vasculitis related with XLP after cure of Burkitt lymphoma is rarely reported.We describe a case of a 5-year-old boy with XLP who developed cerebral vasculitis two years after cure of Burkitt lymphoma.He had Burkitt lymphoma at the age of 3 years and received chemotherapy(non-Hodgkin’s lymphomaBerlin-Frankfurt-Milan-90 protocol plus rituximab),which induced complete remission over the following two years.At the age of 5 years,the patient first developed headache,vomiting,and then intellectual and motorial retrogression.His condition was not improved after anti-infection,dehydration,or dexamethasone therapy.No tumor cells were found in his cerebrospinal fluid.Magnetic resonance imaging showed multiple non-homogeneous,hypodense masses along the bilateral cortex.Pathology after biopsy revealed hyperplasia of neurogliocytes and vessels,accompanied by lymphocyte infiltration but no tumor cell infiltration.Despite aggressive treatment,his cognition and motor functions deteriorated in response to progressive cerebral changes.The patient is presently in a vegetative state.We present this case to inform clinicians of association between lymphoma and immunodeficiency and explore an optimal treatment for lymphoma patients with compromised immune system.

Abdominal computed tomography in refractory coeliac disease and enteropathy associated T-cell lymphoma

AIM: To evaluate computed tomography (CT) findings, useful to suggest the presence of refractory celiac disease (RCD) and enteropathy associated T cell lymphoma (EATL). METHODS: Coeliac disease (CD) patients were divided into two groups. GroupⅠ: uncomplicated CD (n = 14) and RCD typeⅠ(n = 10). Group Ⅱ: RCD type Ⅱ (n = 15) and EATL (n = 7). RESULTS: Both groups showed classic signs of CD on CT. Intussusception was seen in 1 patient in groupⅠvs 5 in group Ⅱ (P = 0.06). Lymphadenopathy was seen in 5 patients in group Ⅱ vs no patients in groupⅠ(P = 0.01). Increased number of small mesenteric vessels was noted in 20 patients in groupⅠvs 11 in group Ⅱ (P = 0.02). Eleven patients (50%) in group Ⅱ had a splenic volume < 122 cm3 vs 4 in groupⅠ(14%), 10 patients in groupⅠ had a splenic volume > 196 cm3 (66.7%) vs 5 in group Ⅱ (33.3%) P = 0.028. CONCLUSION: CT scan is a useful tool in discriminating between CD and (Pre) EATL. RCD Ⅱ and EATL showed more bowel wall thickening, lymphadenopathy and intussusception, less increase in number of small mesenteric vessels and a smaller splenic volume compared with CD and RCDⅠ.

Primary non hodgkin's lymphoma of lacrimal sac presented as recurrent acute dacryocystitis: A case report

Primary non Hodgkin's lymphoma of the lacrimal sac is uncommon but potentially delay in diagnosis as it may mimic the presentation of primary post saccal nasolacrimal duct obstruction. In this article, we reported a case of primary non Hodgkin's lymphoma of the lacrimal sac presented with recurrent acute dacryocystitis in a young lady. A 28 years old Malay lady had history of persistent epiphora on left eye for 4 years. Prior to presentation to our clinic, it was preceded by progressive recurrent painful medial canthal swelling for 6 months duration. Clinical diagnosis of post saccal naso lacrimal duct obstruction was made and otorhinolaryngology assessment revealed intranasal mass. Endoscopic excision was done showed diffuse large B cell lymphoma on histology. The patient was started on 6 cycles of chemotherapy which subsequently result in no recurrence of the tumour post chemotherapy. Any case of post saccal nasolacrimal duct obstruction should be investigated thoroughly as it may become as a presentation of other sinister pathology.