OBJECTIVE: To analyze the clinical characteristics of the patients with B-cell chronic lymphoproliferative disease（B-CLPD） in the new drug era and the effect of new drug treatment on efficacy and survival. METHODS: The clinical and laboratory data of 200 cases B-CLPD patients diagnosed between April 2015 and August 2021 were analyzed retrospectively. The clinical efficacy and survival of the patients under different treatments including Bruton tyrosine kinase（BTK） inhibitors， rituximab， and chemotherapy alone were analyzed. The prognostic factors affecting the survival of patients were analyzed by univarite analysis and multivariate analysis. RESULTS: There were 119 male（59.5％） and 81 female（40.5%） in 200 cases B-CLPD patients， the sex ratio(male/female) was 1.5∶1 with median age of 61（30- 91） years old. The distribution of subtypes were as fallows: 51 cases (25.5%) of chronic lymphocytic leukemia/small lymphocytic lymphoma（CLL/SLL）， 64（32.0%） cases of follicular lymphoma（FL）， 40（20.0%） cases mantle cell lymphoma（MCL）， 30（15.0%） cases of marginal zone lymphoma（MZL）， 10（5%） cases of lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia（LPL/WM）， 5（2.5%） cases of B cell chronic lymphoproliferative disorders unclassified（B-CLPD-U） . The main clinical manifestation of 102 patients was lymph node enlargement, 32 cases were complicated with B symptoms. Among CLL/SLL patients， there were 12（23.5%） cases in Binet A and 39（76.5%） cases in Binet B/C. There were 29 patients（20.9%） in Ann Arbor or Lugano stage I-II and 110 cases（79.1%） in stage III-IV of other subtypes. The complete remission（CR） rate was 43.1%（25/58）， 40.2%（39/97）， 7.1%（1/14）， and overaIl response rate（ORR） was 87.9%（51/58）， 62.9%（61/97）， 28.6%（4/14） in the groups of BTK inhibitors， rituximab-based therapy， and chemotherapy alone. The 3-year OS rate and PFS rate in all patients was 79.2% and 72.4% respectively. The 3-year OS rate of patient with MZL, CLL/SLL, FL,WM was 94.7%, 87.7%, 86.8% and 83.3% respectively, while the 3-year OS rate of MCL was only 40.6%, which was significantly lower than other subtypes. The median OS of patients treated with BTK inhibitors and rituximab-based therapy was 20.5 and 18.5 months respectively， and the 3-year OS rate was 97.4% and 90.7%. However， the median PFS of patients receiving chemotherapy alone was 4 months， and the 1-year OS rate was 52.7%， which was statistically significant compared with the other two groups（P<0.05）. Univarite analysis showed that anemia， elevated lactate dehydrogenase， elevated β2-microglobulin， and splenomegaly were the poor prognostic factors for OS（P<0.05）， elevated lactate dehydrogenase was also poor prognostic factors for PFS（P<0.05）. Multifactor analysis showed that anemia and elevated lactate dehydrogenase were the independent poor prognostic factors for survival（P<0.05）. CONCLUSION The clinical features of B-CLPD was various， anemia and elevated lactate dehydrogenase are the prognostic factors for poor survival. BTK inhibitors and new immunotherapy can improve the survival and prognosis of patients in the new drug era.
Humans ; Adult ; Female ; Male ; Middle Aged ; Aged ; Aged, 80 and over ; Rituximab/therapeutic use* ; Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy* ; Retrospective Studies ; Lymphoma, Mantle-Cell ; Prognosis ; Lymphoma, B-Cell, Marginal Zone ; Lactate Dehydrogenases

Hepatitis C virus (HCV) is one of the leading causes of chronic liver disease. HCV is not only related to hepatic malignancies but may also promote lymphoid neoplasms. Currently, research has confirmed HCV-related lymphoma, including marginal zone lymphoma (MZL), lymphoplasmacytic lymphoma (LPL), follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL), and Burkitt lymphoma (BL). Many types of research have shown that antiviral therapy can improve or even remission several HCV-related lymphomas. The direct-acting antiviral agents (DAAs) (such as NS5A protease inhibitors, NS4/4A protease inhibitors and viral polymerase inhibitors) have shown clinical advantages of high efficacy and low side effects for both virus elimination and tumor regression in several HCV-related lymphomas, which may make the selected HCV-related lymphoma patients treated without chemotherapy. In this review the research progress and development direction of antiviral therapy in treating HCV-related lymphoma has summarized briefly.
Antiviral Agents/therapeutic use* ; Hepacivirus ; Hepatitis C/drug therapy* ; Hepatitis C, Chronic/drug therapy* ; Humans ; Lymphoma, B-Cell, Marginal Zone/drug therapy* ; Lymphoma, Large B-Cell, Diffuse/drug therapy*

The stomach is the most common primary site of an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type, which is characterized by an indolent clinical course. A diagnosis of gastric MALT lymphoma requires an endoscopic biopsy that should be confirmed by an experienced pathologist. Gastric MALT lymphoma shows a variable endoscopic appearance, including erosion, erythema, discoloration, atrophy, ulcer, and subepithelial lesion. The distribution is often multifocal. Therefore, clinical suspicion and multiple biopsies are essential for an accurate diagnosis. Gastric MALT lymphoma is almost invariably associated with a Helicobacter pylori (H. pylori) infection. H. pylori eradication therapy is the mainstay of treatment, which must be delivered to all patients regardless of the H. pylori infection status or stage. For patients who have failed to achieve remission following eradication therapy, radiotherapy or chemotherapy can be considered. Radiotherapy is an effective treatment modality for a localized stage and shows excellent outcomes. In the presence of disseminated or advanced disease, chemotherapy and/or immunotherapy with the anti-CD20 monoclonal antibody, rituximab, can be applied. Treatment should be individualized according to the stage and symptoms, as well as the patients' preference. Given that the clinical course of gastric MALT lymphoma is usually indolent, watchful waiting may be an adequate strategy in selected cases where scheduled follow-up is guaranteed.
Atrophy ; Biopsy ; Diagnosis ; Drug Therapy ; Erythema ; Follow-Up Studies ; Helicobacter pylori ; Humans ; Immunotherapy ; Lymphoma, B-Cell, Marginal Zone ; Radiotherapy ; Rituximab ; Stomach ; Ulcer ; Watchful Waiting

BACKGROUND/AIMS: The eradication of Helicobacter pylori (H. pylori) is an effective treatment in gastric mucosa-associated lymphoid tissue (MALT) lymphoma associated with H. pylori infection. However, the treatment strategy in gastric MALT lymphoma patients who are H. pylori-negative or unresponsive to H. pylori eradication therapy remains controversial. In this study, we investigated the clinical efficacy of treatments other than H. pylori eradication therapy in these groups of patients. METHODS: This was a retrospective single-center study based on the medical records of patients diagnosed with gastric MALT lymphoma at Yeungnam University Medical Center between January 2005 and December 2016. Patients were treated with H. pylori eradication therapy, chemotherapy, or radiotherapy according to their H. pylori infection status and stage of gastric MALT lymphoma. RESULTS: Of the 68 eligible patients, 50 were enrolled in the study. Of the 42 patients with H. pylori-positive gastric MALT lymphoma, 36 (81.7%) were treated with H. pylori eradication therapy as primary treatment and 25 (69.4%) achieved a complete response (CR). Patients without a CR after H. pylori eradication therapy (n=11, 30.6%) received radiotherapy as a secondary treatment. Two patients with H. pylori-positive gastric MALT lymphoma and eight with H. pylori-negative gastric MALT lymphoma received radiotherapy as the primary treatment. CR was achieved in all 21 patients treated with radiotherapy as primary or secondary treatment. The 5-year progression-free survival rate after radiotherapy was 92.9%. CONCLUSIONS: Radiotherapy may be a worthwhile treatment option in patients with H. pylori-negative MALT lymphoma or H. pylori-positive MALT lymphoma that is not responsive to H. pylori eradication therapy.
Academic Medical Centers ; Disease-Free Survival ; Drug Therapy ; Helicobacter pylori* ; Helicobacter* ; Humans ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Medical Records ; Radiotherapy* ; Retrospective Studies ; Stomach Neoplasms ; Treatment Outcome*

PURPOSE: We report a case of a young female patient who was diagnosed with a marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) of the lacrimal sac which mimicked dacrocystitis. CASE SUMMARY: A 23-year-old female suffered from epiphora for 3 years in the right eye. She had swelling and a painful lesion at the lacrimal sac 4 months prior and was referred to our hospital due to nasolacrimal duct obstruction and dacryocystitis. Lacrimal irrigation was performed with no passing and regurgitation with mucoid discharge. We performed orbital computed tomography and magnetic resonance imaging which showed a suspected tumor of the lacrimal sac in the right eye. We then performed excision and biopsy of the tumor through the skin approach. The patient was diagnosed with a MALT lymphoma with no systemic involvement. After six cycles of chemotherapy involving rituximab with cyclophosphamide, vincristine, and prednisone, the lesion of the mass and the painful symptoms decreased, which was regarded as a complete response. However, epiphora and eye discharge persisted, showing a nasolacrimal duct obstruction, so we performed endoscopic dacryocystorhinostomy with a lacrimal sac biopsy. The histological examination showed chronic inflammation, but not lymphoma. There was no recurrence of lymphoma at one year postoperatively. CONCLUSIONS: If there is an epiphora and palpable lesion in the lacrimal sac area, MALT lymphoma mimicking dacyocystitis may be suspected. After the lymphoma is first diagnosed by excision and biopsy, systemic chemotherapy with dacryocystorhinostomy could be an effective treatment.
Biopsy ; Cyclophosphamide ; Dacryocystitis ; Dacryocystorhinostomy ; Drug Therapy ; Female ; Humans ; Inflammation ; Lacrimal Apparatus Diseases ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone ; Magnetic Resonance Imaging ; Nasolacrimal Duct ; Orbit ; Prednisone ; Recurrence ; Rituximab ; Skin ; Vincristine ; Young Adult

PURPOSE: To report a case where bilateral malignant retrobulbar lymphoma was diagnosed after repetitive intraocular lens dislocation to the anterior chamber. CASE SUMMARY: An 85-year-old male with a history of stroke who had undergone cataract surgery 10 years ago at another hospital presented with repeated intraocular lens (IOL) dislocations of both eyes into the anterior chamber. He had previously undergone IOL scleral fixation once in his left eye and twice in his right eye, but IOL dislocation was still repeatedly occurring. The best-corrected visual acuity was 0.4 in both eyes. Hertel exophthalmetry was 20 mm in his right eye and 18 mm in his left eye. Painless limitation of motion at supraduction was observed in the right eye. Funduscopy showed newly appeared choroidal folding in the right eye, so orbital computed tomography (CT) with contrast was performed. The CT scans showed bilateral homogenously enhancing retrobulbar masses. Biopsy of the masses showed a MALToma. After radiation therapy, the choroidal folds resolved and exophthalmetry improved to 10 mm in both eyes. No additional IOL dislocation occurred. During 2.5 years of follow-up, there was no evidence of recurrence or distant metastasis of the MALToma. CONCLUSIONS: Orbital lymphomas can cause lid edema, exophthalmos, strabismus, and diplopia, and can be diagnosed with imaging modalities such as CT. Final diagnosis involves biopsy and radiation therapy or chemotherapy. If IOL dislocation occurs repeatedly, it may result from an increase in retrobulbar pressure, and concurrent choroidal folding using funduscopy is strongly recommended for imaging to check for the presence of retrobulbar masses.
Aged, 80 and over ; Anterior Chamber ; Biopsy ; Cataract ; Choroid ; Diagnosis ; Diplopia ; Dislocations* ; Drug Therapy ; Edema ; Exophthalmos ; Follow-Up Studies ; Humans ; Lenses, Intraocular* ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Neoplasm Metastasis ; Orbit ; Recurrence ; Strabismus ; Stroke ; Tomography, X-Ray Computed ; Visual Acuity

Approximately 90% of gastric mucosa-associated lymphoid tissue (MALT) lymphomas are associated with Helicobacter pylori infection and 10% of gastric MALT lymphomas occur independent of H. pylori infection. The gene translocation t(11;18)(q21;q21) is frequently observed in cases presenting with H. pylori-negative gastric MALT lymphomas. H. pylori-negative gastric MALT lymphomas do not differ from H. pylori-positive gastric MALT lymphomas in terms of clinical features and endoscopic findings and show similar prognosis. H. pylori eradication therapy could be considered as first-line treatment for gastric MALT lymphomas regardless of their H. pylori infection status. In cases with H. pylori-negative gastric MALT lymphomas, the rate of response to eradication therapy varies from 0 to 83%. If gastric MALT lymphoma persists for more than a year, secondary treatment with radiotherapy, chemotherapy, and/or surgery should be considered. Of note, the comparative outcome is excellent.
B-Lymphocytes ; Drug Therapy ; Helicobacter pylori ; Helicobacter* ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Prognosis ; Radiotherapy

It is often difficult to differentiate gastric mucosa-associated lymphoid tissue (MALT) lymphoma from Helicobacter pylori-associated follicular gastritis, and thus, it becomes unclear how to manage these diseases. This study aimed to explore the management strategy for and the long-term outcomes of suspicious gastric MALT lymphoma detected by forceps biopsy during screening upper endoscopy. Between October 2003 and May 2013, consecutive subjects who were diagnosed with suspicious gastric MALT lymphomas by screening endoscopy in a health checkup program in Korea were retrospectively enrolled. Suspicious MALT lymphoma was defined as a Wotherspoon score of 3 or 4 upon pathological evaluation of the biopsy specimen. Of 105,164 subjects who underwent screening endoscopies, 49 patients with suspicious MALT lymphomas who underwent subsequent endoscopy were enrolled. Eight patients received a subsequent endoscopy without H. pylori eradication (subsequent endoscopy only group), and 41 patients received H. pylori eradication first followed by endoscopy (eradication first group). MALT lymphoma development was significantly lower in the eradication first group (2/41, 4.9%) than in the subsequent endoscopy only group (3/8, 37.5%, P = 0.026). Notably, among 35 patients with successful H. pylori eradication, there was only one MALT lymphoma patient (2.9%) in whom complete remission was achieved, and there was no recurrence during a median 45 months of endoscopic follow-up. H. pylori eradication with subsequent endoscopy would be a practical management option for suspicious MALT lymphoma detected in a forceps biopsy specimen obtained during screening upper endoscopy.
Adult ; Aged ; Anti-Bacterial Agents/therapeutic use ; Biopsy ; Female ; Follow-Up Studies ; Gastric Mucosa/*pathology ; Gastritis/diagnosis/etiology/microbiology ; Gastroscopy ; Helicobacter Infections/complications/*diagnosis/drug therapy ; Humans ; Lymphoma, B-Cell, Marginal Zone/complications/*diagnosis/pathology ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies

Primary rectal lymphoma is a rare disease among the gastrointestinal (GI) lymphoma. In particular, diffuse large B-cell lymphoma (DLBCL) transformed from mucosa-associated lymphoid tissue (MALT) lymphoma is often the primary type of GI lymphoma, mostly in stomach or duodenum, but has never been reported in rectum. Here we report an unusual case in which a 75-year-old male patient diagnosed with DLBCL transformed from MALT lymphoma in the rectum. The patient was diagnosed as rectal DLBCL transformed from MALT lymphoma as Lugano stage II2 and was treated with chemotherapy (R-CHOP) with CD-20 monoclonal antibody (rituxaimb). Complete remission of multiple lymphadenopathy and mass forming ulcer of the rectum was achieved after 6 cycles of R-CHOP. He has been free from disease for 12 months.
Aged ; B-Lymphocytes* ; Drug Therapy ; Duodenum ; Humans ; Lymphatic Diseases ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Rare Diseases ; Rectum ; Stomach ; Ulcer

Recent advances in chemotherapy have led to increased survival rates for patients with hematologic malignancies. However, standard chemotherapies, including alkylating agents for non-Hodgkin lymphoma, could induce therapy-related myeloid neoplasms (t-MNs), a group of disorders categorized by the World Health Organization in 2008. Here, we report a case of coexistence of bone marrow (BM)-involved refractory marginal zone B-cell lymphoma (MZL) and therapy-related myelodysplastic syndrome (t-MDS). Simultaneous presence of refractory lymphoma and t-MN in the BM is rare, and this is the first report in Korea. The patient received allogeneic hematopoietic stem cell transplantation (HSCT) to cure both the MZL and t-MDS. Since the HSCT, he has been stable for 21 months without any evidence of recurrence.
Alkylating Agents ; Bone Marrow ; Drug Therapy ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Lymphoma, Non-Hodgkin ; Myelodysplastic Syndromes* ; Recurrence ; Survival Rate ; World Health Organization