MALT-type Lymphoma Research Articles

Non-hodgkin Lymphoma of Lymphoid Tissue Associated with Extranodal Mucosa of the Cecum and Acute Appendicitis. Report of a Case

Objective: Presentation of an acute appendicitis and non-Hodgkin lymphoma of lymphoid tissue associated with extranodal mucosa from the cecum clinical case. Introduction: The cecal appendix can be affected by Non-Hodgkin Lymphomas (NHL) by extension of the neoplasm; however primary appendicular lymphoproliferative processes are uncommon. The NHL of Mucosal-Associated Lymphoid Tissue (MALT) is exceptional. To establish the diagnosis of MALT, a morphological, molecular and immunohistochemical diagnosis is required, including molecular techniques that reflect B cell clones, PCR analysis or the identification of chromosomal abnormalities. Methods: 40 year old male, with 15 days of evolution of abdominal pain located in Right Lower Quadrant (RLQ) and associated symptoms like decreased appetite, nausea, and adynamia, who was firstly diagnosticated as an urinary tract infection treated as outpatient; upon his readmission, he was performed CT scan evidencing mesenteric panniculitis and thickening of the appendicular wall; he was surgically intervened finding edematous and congestive vermiform appendix with lower third perforation, and indurated cecum with 2 cm perforation from the appendicular base, performing right hemicolectomy with ileo-transverse mechanical anastomosis; he was discharged home 7 days after the postoperative period; the histopathological study and immunohistochemistry panel was compatible with MALT-type lymphoma, with lesion-free edges. Extension CT scans with no evidence of distant disease. He was sent to oncology for follow-up. Conclusion: It is important to get an established diagnosis in this rare pathology with molecular techniques and not only with histopathological cuts, since 30% of patients relapse.

Tumor Cell Microenvironment and Microvessel Density Analysis in MALT Type Lymphoma.

MALT type lymphoma belongs to marginal zone lymphoma (MZL). MALT lymphomas' inflammatory microenvironment contributes to the pathogenesis of this cancer. In this study, we analyzed and quantified the tumor inflammatory microenvironment in MALT lymphoma samples and in healthy controls, and the microvessel content by immunohistochemistry and morphometric estimation. Biopsy specimens from MALT type lymphoma patients and from non-metastatic axillary lymph nodes (CTRL) were analyzed by immunochemistry in order to quantify CD3, CD4, CD8, CD68, CD163, tryptase, CD34, and Ki67 positive cells. A substantial increase in the number of cells that were positive for the above markers and microvascular density (MVD) were observed in the MALT group. We also found a positive correlation between microvessels and CD8+ cells and between CD8+ cells and M2 type macrophages, while tryptase+ mast cells correlated with CD4+ cells. The mitotic proliferation index Ki67 was higher in MALT samples. The interactions between inflammatory cells in the tumor microenvironment and their correlation with angiogenesis is a useful tool in the development of new immunotherapy strategies.

Radiosugery with CyberKnife in Primary Orbital MALT Lymphoma: A Case Report

MALT lymphoma constitutes one half of all orbital malignancies. Ocular adnexal lymphoma of MALT-type (OAML) may affect critical structures in the orbit and can become therapeutic challenge. Surgery, radiotherapy or chemotherapy, alone or in combined modalities can be used as treatment options based on the individual patient variables as well as the site, stage and surgical accessibility of the OAML. This case confirms that radiosurgery with CyberKnife is an effective and safe therapeutic option in patients with OALM reducing the collateral damage to a minimal rate.

Helicobacter pylori and skin disorders: a comprehensive review of the available literature.

Helicobacter pylori is a Gram-negative bacterium identified for the first time about 30 years ago and commonly considered as the main pathogenic factor of gastritis and peptic ulcer. Since then, it was found to be associated with several gastrointestinal and extra-gastrointestinal diseases. Helicobacter pylori is also associated with many skin disorders including, but not limited to, chronic urticaria, rosacea, lichen planus, atopic dermatitis, psoriasis, pemphigus vulgaris, vitiligo, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, primary cutaneous marginal zone B-cell lymphomas and cutaneous T-cell pseudolymphoma. Literature up to September 2020 shows that clear evidence exists only for some of the mentioned associations, while in the majority of cases, data appear contrasting. The aim of this review is to summarize the available studies on the topic and draw possible conclusions. Further clinical and laboratory studies are needed to assess the real plausibility and relevance of these associations, as well as the possible role of Helicobacter pylori with the underlying pathogenic mechanisms.

Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Intraocular Involvement: Clinicopathologic Characteristics and the Treatment Outcome of Intravitreal Injection of Methotrexate

Background：Primary intraocular and periocular mucosa-associated lymphoid tissue lymphoma (MALT-lymphoma) was a rare type of non-Hodgkin lymphoma (NHL) with marked differences from other indolent B-cell lymphomas. Transformation to large-cell aggressive lymphomas could occur in a few cases. The initial therapeutic modality has not been fully established for the disease. Methotrexate (MTX), as the most efficient cytotoxic drug for patients with vitreoretinal involvement in primary intraocular lymphoma (PIOL), through intravitreal injection of MTX. Here we reported long-term activity and treatment outcomes of the MALT-lymphoma intraocular and periocular involvement. Patients and Methods: Adult patients with histologically/cytologically confirmed unilateral primary intraocular and periocular B-cell MALT-lymphoma at Beijing Tongren hospital were enrolled. Patients were randomly assigned 1:1 to receive intravitreal injection of MTX (IV-MTX group, 400 µg/0.1 ml) according to a standard Intensive-Consolidation-Maintenance regimen, or Wait-and-See management as a control group. Primary end points were progression-free-survival (PFS), best-corrected visual acuity (BCVA), clinical response to intravitreal chemotherapy, complications during the study period. Results: A total of 10 patients were enrolled (IV-MTX, n=5; Wait-and-See, n=5). Median follow-up was 5.5 years (QIR, 2.0-9.0 years). At the end follow-up, no death and systemic metastasis occurred in all patients. PSF at 5 years was excellent (70% and 80%, respectively) and did not differ between two groups. For the IV-MTX group, the BCVA was not obviously improved and the tumor tissues were unchanged clinically in size after a median 7 times of vitreous injection of MTX in all 5 eyes (P>0.05) and had lower rates of treatment-related complications. For the control group, one of five eyes had worsening BCVA and four remained stable. The volume of tumor tissue increased slowly, and the tumor basal and thickness increased 1.08±1.63 mm and 0.32±0.80 mm on average, respectively. Conclusions: According to our current sample size and the duration of follow-up, it suggests that no clinical benefit of IV-MTX has been found to primary intraocular and periocular MALT-lymphoma. The observing management for these patients with primary intraocular and periocular lymphoma of MALT-type was reasonable, with relatively excellent long-term outcomes. Funding Statement: The National Natural Science Foundation of China (Nr. 81272981), the Beijing Natural Science Foundation (Nr. 7151003), and the priming scientific research foundation for the junior research in Beijing Tongren Hospital Capital Medical University (2017-YJJ-ZZL-009) provided financial support. Declaration of Interests: All authors declare that they have no conflict of interest in this manuscript. Ethics Approval Statement: This study followed the tenets of the Declaration of Helsinki and its ethical standards of 1964. The study was approved by the Medical Ethics Committee of the Beijing Tongren Hospital, and written informed consent was obtained from all participants. All methods were performed in accordance with the relevant guidelines and regulations.

PNEUMONIA OR LYMPHOMA? DO NOT BE FOOLED!

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Mucosa-associated lymphoid tissue (MALT) Lymphoma, also known as Extranodal Marginal Zone B cell lymphoma (EMZL), is a rare type of non-Hodgkin’s lymphoma with an incidence rate of 18.3 cases per 1 million in the United States. The objective of this case is to describe a challenging case of EMZL presenting as persistent localized lung consolidation that was mistaken for pneumonia. CASE PRESENTATION: 77-year-old male, former smoker who presented with complains of nonproductive cough for several weeks, chest-x-ray (CXR) showed a 4.3 cm rounded hazy right parahilar mass. A follow up computed tomography (CT) of the chest revealed 4.7 x2.8 cm consolidative opacity along the interlobar fissure in the right upper lobe (RUL). Reviewing back a year ago, CT scan done for nonspecific chest pain, showed the mass size was unchanged but appeared to be less solid compared to the current scan. Patient underwent a Fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan which showed elevated FDG activity SUV 3.0 associated with a consolidative pulmonary opacity in the posterior portion of the right upper lobe silhouetting the major fissure. The patient subsequently underwent a CT guided percutaneous biopsy of the right upper lobe airspace consolidation. Histologic sections showed interstitial expansion and bronchial epithelium involvement by small monocytoid- appearing lymphocytes and significant amount of plasma cells (Figure 1). Immunohistochemistry was positive for CD20 and BCL-2 and negative for BCL-6 and CD10. CD138 which highlights plasma cell component was positive. Immunostaining for kappa and lambda showed findings most suggestive of monotypic lambda expression. Fluorescence in situ hybridization (FISH) studies for MALT1 (18q21) rearrangement identified a MALT1 gene rearrangement and B cell clonality studies done by PCR were positive for a clonal rearrangement. Therefore, the final diagnosis was small B-cell non-hodgkin lymphoma consistent with extranodal MALT. In retrospect the lymphoma was likely present a year ago however mistaken as a pneumonia. Given the lack of symptoms and minimal progression of over one year, was consistent with the indolent clinical behavior of the tumor. Decision was made not to treat at this point by the physician and patient. Till date patient remain asymptomatic. DISCUSSION: EMZL has been associated with persistent inflammatory state, either infectious or autoimmune in nature. EMZL can present in various ways from an incidental finding on imaging to ambiguous symptoms such as dyspnea, chest pain, cough, and rarely hemoptysis. Prognosis in MALT-type lymphomas is generally good with mean survival of more than 10 years. CONCLUSIONS: In conclusion, EMZL of the lung is uncommon and unique therefore a collaborative effort addressing early diagnosis and less invasive diagnostic testing for these high-risk EMZL patients are needed. Reference #1: Khalil MO, Morton LM, Devesa SS, et al. Incidence of marginal zone lymphoma in the United States, 2001 to 2009 with a focus on primary anatomic stie. Br J Haematol 2014; 165:67 Reference #2: Santos IG, Marchiori E, Zanetti G, Mano CM, SarcielliLuz B, Vianna FG. Primary pulmonary mucosaasociated lymphoid tissue lymphoma computed tomography findings: A case report. Cases J. 2009;2:6329. [PMCID:PMC2740136] [PubMed: 19829788] DISCLOSURES: No relevant relationships by Hussein Hussein, source=Web Response No relevant relationships by Ahmad JABRI, source=Web Response No relevant relationships by Sonya Joshi, source=Web Response No relevant relationships by Sujith Modugula, source=Web Response No relevant relationships by Elizabeth Verghese, source=Web Response

Primary Breast Lymphoma: Case Report Of A Rare Pathological Entity

Primary breast lymphoma is a rare disease, which accounts for 0.4% of all malignancies seen in the breast and 2% of extranodal lymphomas. The majority of cases are B-cell lymphomas, with diffuse large cell (B) lymphoma the predominant histological type. A variety of treatment modalities have been suggested, with radiation therapy recommended for stage I patients and chemotherapy recommended for stage II patients. Outcome following treatment is predicted by the pathological grade of the tumor and the presence of lymph node involvement. Radical surgical resection including mastectomy has not been shown to affect the outcome of this entity.
 A 61-year-old woman presented with a one month history of a painful right breast lump. Examination identified the presence of two lumps which were biopsied, confirming the presence of a lymphocytic lymphoma expressing a B-cell phenotype with co-expression of CD 43 (MT-1) consistent with MALT-type lymphoma.
 She underwent further excision for recurrent tumor but declined adjuvant therapy. She has remained disease free during a 7-year follow up period.
 Primary breast lymphoma is a rare malignancy of the breast representing less than 2% of non-Hodgkin lymphoma. The features on clinical breast examination and imaging are not characteristic and cannot be differentiated from breast carcinoma. Treatment involves surgical resection with adjuvant chemotherapy and radiotherapy administered in selected cases.
 A 61-year-old woman presented with a one month history of a painful right breast lump. Examination identified the presence of two lumps which were biopsied, confirming the presence of a lymphocytic lymphoma expressing a B-cell phenotype with co-expression of CD 43 (MT-1) consistent with MALT-type lymphoma.
 She underwent further excision for recurrent tumor but declined adjuvant therapy. She has remained disease free during a 7-year follow up period.
 Primary breast lymphoma is a rare malignancy of the breast representing less than 2% of non-Hodgkin lymphoma. The features on clinical breast examination and imaging are not characteristic and cannot be differentiated from breast carcinoma. Treatment involves surgical resection with adjuvant chemotherapy and radiotherapy administered in selected cases.

MON-441 Primary Thyroid Maltoma: A Rare Non-hodgkin Malignancy

Introduction: Thyroid lymphoma is a rare malignancy; only 2% of extranodal lymphomas arise within the thyroid gland. Furthermore, lymphomas represent no more than 2% of all malignant thyroid tumors and almost always non-Hodgkin type. Case: A 58-year-old female with a medical history of rheumatoid arthritis treated with Golimumab for years. Exam revealed a right thyroid lobe enlargement with bilateral palpable nodules. Labs include TSH of 1.4 mU/L, Free T4 of 1.76 ng/dL, normal anti-thyroid peroxidase antibodies, but elevated anti-thyroglobulin. US of the thyroid showed right-sided, hyperechoic nodule measuring 1.3cm, and left-sided solid hypoechoic nodule measuring 2.2cm. US-guided FNA of the right nodule yielded atypical cells of undetermined significance (2017 Bethesda category III). FNA of the left nodule showed severe, chronic thyroiditis, with small clonal population of cells and suspicion for lymphoma. She underwent total thyroidectomy with left central lymph node dissection. Microscopic evaluation showed a predominant lymphocytic population with plasmacytoid/plasma cells. The histological finding was consistent with extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT lymphoma or MALToma). However, thyroid tissues were negative for clonal immunoglobulin gene rearrangements (both immunoglobulin heavy and light chains). Immunohistochemistry demonstrated that the tumor cells were CD19, CD 20, CD 22, PAX5, CD78A positive, highlighting predominantly small to medium-sized B- cell lymphocytes, along with plasmacytoid/plasma cells (MUM1 and CD 75A positive). Furthermore, occasional reactive germinal centers (CD10, BCL6 positive, BCL2 negative) were noted. In conclusion, these features were consistent with a low grade, non-Hodgkin’s lymphoma of MALT type. Discussion: EMZL is a clinically indolent non-Hodgkin lymphoma, and growing evidence suggests that numerous cases originate in the background of chronic immune stimulation. MALToma is associated with Hashimoto’s thyroiditis or other immune diseases. Whereas half of EMZL can still arise without a Hashimoto thyroiditis background. Characteristics of the MALToma include reactive follicles, with the neoplastic cells invading the marginal zone (small lymphocytes, marginal zone B-cells, and plasma cells). The key findings are the presence of clonality (light chain restriction), and confirmation of B-cell origin (presence of B-cell markers). The immunophenotype is confirmed by immunohistochemistry and flow cytometry. Localized EMZL of the thyroid can be effectively treated with radiation alone. Surgery is usually used for diagnostic biopsy only. Chemotherapy is usually reserved for those with advanced stages

A RARE PRESENTATION OF PRIMARY PULMONARY EXTRANODAL DIFFUSE LARGE B CELL LYMPHOMA

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Diffuse large B cell lymphoma (DLBL) is a common subtype of non-Hodgkin’s lymphoma (NHL) that usually presents with rapidly expanding lymphadenopathy. Here we present an unusual case of DLBL with a primary extra-nodal pulmonary presentation. CASE PRESENTATION: A 91-year-old female presented to her PMD for evaluation of a non-productive cough of several weeks’ duration. She had no prior history of pulmonary disease, was a never smoker, and was independent. She denied recent travel, pets, or known sick contacts and was up-to-date with pneumonia vaccinations. Complete blood counts and routine serum chemistries were unremarkable. She returned three months later with continued non-productive cough as well as generalized fatigue and intermittent chills. She was prescribed benzonatate and doxycycline for management of bronchitis. A CXR was ordered which showed a 4.5 cm right hilar opacity. CXR performed one year prior was unremarkable. She was referred for pulmonary consultation. CT chest showed a right upper lobe 4.8 x 5.9 cm mass, a left lower lobe 2.1 x 2.3 cm mass, right pleural thickening, and right cardiophrenic adenopathy measuring 2.0 x 1.2 cm. A navigational bronchoscopy with biopsy demonstrated a mass partially occluding right upper lobe bronchus and biopsy was consistent with DLBL. PET-CT revealed multiple areas of hypermetabolic activity in the abdomen, pelvis, and chest with multiple pulmonary masses, the most superior of which occluded the right upper lobe bronchus, as well as extensive pleural disease. She was started on a non-anthracycline chemotherapy regimen within two weeks of diagnosis. DISCUSSION: Primary NHL of the lung is rare, accounting for 0.4% of all malignant lymphomas. DLBL is an uncommon subtype within that group. Symptoms are non-specific, as are associated radiologic findings, making initial diagnosis difficult. Fine needle aspiration of DLBL usually obtains insufficient material to evaluate the architecture of the lymphoma for diagnosis. The majority of patients require invasive diagnostic biopsies to provide adequate tissue for evaluation. Several studies have shown that cell marker studies using bronchoalveolar lavage may be useful. Prompt initiation of treatment is necessary as DLBL of the lung has a worse prognosis than the more common MALT lymphoma of the lung. However, some studies report survival rates as high as 86% at three years for primary NHL of the lung. CONCLUSIONS: DLBL is a common NHL subtype that rarely manifests with primary pulmonary presentation masquerading as a primary lung malignancy. Given the non-specific clinical presentation, non-specific radiologic findings, and overall rarity, initial diagnosis is difficult, making prompt treatment a challenge. Further research is warranted to better diagnose and prognosticate primary DLBL of the lung. Reference #1: Teras, L. R., DeSantis, C. E., Cerhan, J. R., Morton, L. M., Jemal, A. and Flowers, C. R. (2016), 2016 US lymphoid malignancy statistics by World Health Organization subtypes. CA: A Cancer Journal for Clinicians, 66: 443-459. https://doi.org/10.3322/caac.21357 Reference #2: Kandori S, Kojima T, Nishiyama H. The updated points of TNM classification of urological cancers in the 8th edition of AJCC and UICC. Jpn J Clin Oncol. 2019; Reference #3: Kurtin PJ, Myers JL, Adlakha H, et al. Pathologic and clinical features of primary pulmonary extranodal marginal zone B-cell lymphoma of MALT type. Am J Surg Pathol. 2001;25(8):997-1008. DISCLOSURES: No relevant relationships by Joseph Berman, source=Web Response No relevant relationships by Miguel Conde, source=Web Response No relevant relationships by Kristin Fless, source=Web Response No relevant relationships by Vagram Ovnanian, source=Web Response No relevant relationships by Killol Patel, source=Web Response

Glycan expression in placental villi histocultures and BeW0 cells: Differences between healthy pregnant vs. early-onset severely preeclamptic women and immunomodulatory effects on circulating Natural Killer Cells.

Primary NHL of the female genital tract are relatively uncommon, accounting for only 2% of all extranodal primary lymphomas, and for less than 0.5% of gynaecologic cancers. Primary endometrial lymphomas can be considered a rarity, with less than 50 instances reported in the literature. We describe a case of primary marginal zone B-cell MALT-type lymphoma of the endometrium, of which there were only other 5 case reports in the literature. We also present a review of the literature.

Linfoma MALT de localización colónica: a propósito de un caso y revisión de la literatura


 
 
 El linfoma MALT es una forma poco frecuente de linfoma no Hodking de células B de bajo grado, que se desarrolla a expensas del tejido linfoide de las membranas mucosas. La localización más frecuente a nivel gastrointestinal es el estómago relacionando con la infección por Helicobacter pylori. El linfoma MALT colónico es una entidad extremadamente rara y cuya incidencia no supera el 5% de los casos en países asiáticos, con series porcentualmente menores al 2,5%, siendo la ubicación de este tipo de linfoma la más inusual de todo el tracto digestivo en comparación con otras neoplasias malignas que afectan al colon. Los autores exponen el caso infrecuente de presentación de linfoma tipo MALT de localización colónica en un paciente varón de 51 años, describiendo la clínica, los resultados de pruebas complementarias y el tratamiento recibido por el paciente.
 
 

Clinical features of malignant orbital lymphoma

The occurrence of malignant lymphoma (ML) among patients with malignant orbital tumors ranges between 10 and 37.3% making early diagnosis clarification crucial for timely treatment. To study clinical features of ML with consideration of its morphological type and localization in the orbit. Clinical picture of primary malignant orbital lymphoma of 34 patients (36 orbits) was examined retrospectively with consideration of the tumor's morphotype. Median observation time was 6 years. Patients aged between 34 and 84 years, female to male ratio was 1.27:1. The final diagnoses were: small cell MALT-type ML (23 patients), large cell B-lymphoma (5 patients), diffuse malignant lymphoma (5 patients), T-cell lymphoma (1 female patient). Systemic changes developed in 29.41% of cases after 6.7 years on average. 23.53% of patients has died. Clinical signs of MALT-type ML were described with consideration of tumor's localization in the orbit, and clinical features of B-cell, large cell, diffuse and T-cell lymphoma were shown. Signs helping differentiate ML from false tumor, composite tear gland tumor and malignant orbital tumor were established. Despite the higher prevalence of malignant MALT-type lymphoma, primary malignant orbital lymphoma is characterized by unfavorable vital prognosis: in a third of all cases, signs of systemic lesion develop in 6.7 years on average. Clinical picture of MALT-type ML is defined by tumor's localization in the orbit. Recurrence of MALT-type ML after combined treatment (surgical, radiotherapy, polychemotherapy) happens in every fourth case among patients with tumor primarily localized in the orbital cellular tissues or in the tear gland. Describing clinical features of primary malignant orbital lymphoma allows faster diagnosis clarification reducing the number of orbital diseases that require differential diagnositcs.

Follicular gastritis associated with Helicobacter pylori.

In order to understand the pathogenesis of gastric lymphoma, we investigated the association of H.pylori infection with lymphoid follicular hyperplasia. Eighty-four gastric specimens removed for gastroduodenal ulcer were histologically examined. The distribution and prevalence of H. pylori, neutrophilic and lymphocytic infiltration, mucosal atrophy, intestinal metaplasia, and lymphoid follicles were scored. The lymphoid follicles were more frequently observed in H.pylori positive cases. They indicated a positive correlation with the score of H. pylori. When follicular gastritis (FG) was defined as a case in which the secondary lymphoid follicles (Lf2) numbered two or more per one centimeter of mucosa in the pyloric gland area of the lesser curvature, twenty specimens out of the 84 (24%) fit that definition. All of the FG cases were H.pylori positive, and they displayed high H. pylori scores. It was supposed that most FG cases would ultimately lead to atrophic gastritis, whereas H.pylori would gradationally decrease or disappear in accordance with the aging and progression of intestinal metaplasia. The histological features of the FG cases, however, were similar to the background mucosal state of early-stage MALT-type gastric lymphoma. We may conclude that H. pylori infection is one cause of the FG, which may be a high-risk condition that gives rise to MALT-type gastric lymphoma.

Differential somatostatin and CXCR4 chemokine receptor expression in MALT-type lymphoma of gastric and extragastric origin

Whereas the different somatostatin receptor (SSTR) subtypes and the chemokine receptor CXCR4 are known to be expressed in a wide variety of human malignancies, comprehensive data are still lacking for MALT-type lymphomas. Overall, 55 cases of MALT-type lymphoma of both gastric and extragastric origin were evaluated for the SSTR subtype and CXCR4 expression by means of immunohistochemistry using novel monoclonal rabbit antibodies. The stainings were rated by means of the immunoreactive score and correlated with clinical data. While the CXCR4 was detected in 92% of the cases investigated, the SSTR subtypes were much less frequently present. The SSTR5 was expressed in about 50% of the cases, followed by the SSTR3, the SSTR2A, the SSTR4 and the SSTR1, which were present in 35, 27, 18 or 2%, respectively, of the tumors only. Gastric lymphomas displayed a significantly higher SSTR3, SSTR4 and SSTR5 expression than extragastric tumors. A correlation between CXCR4 and Ki-67 expression was seen in gastric lymphomas, whereas primarily in extragastric tumors SSTR5 negativity was associated with poor patient outcome. The CXCR4 may serve as a promising target for diagnostics and therapy of MALT-type lymphomas, while the SSTRs appear not suitable in this respect.

Lymphome de type Mucosa-Associated Lymphoid Tissue, (MALT) pulmonaire, puis gastrique

Pulmonary Mucosa-Associated Lymphoid Tissue (MALT)-type lymphoma is the most frequent primary pulmonary lymphoma. We report the case of a patient who presented a pulmonary MALT-type lymphoma treated with chloraminophen, with a recurrence 5 years later characterized with pulmonary lesions associated with a gastric location. This observation underlines some anatomical and clinical aspects of pulmonary MALT-type lymphoma, and leads to discuss the evolution of its physiopathological and therapeutic concepts. In particular, the contributions of positron emission tomography and molecular biology allow the analysis of possible multifocal affections of this disease.

Endoscopic criteria for diagnostics of various macroscopic variants of non-Hodgkin gastric lymphomas

According to most investigators, stomach is the most common site of gastrointestinal lymphomas, but endoscopic semiotics and diagnostics of these lesions is not sufficiently developed. Aim : to develop criteria for the diagnosis of endoscopic non-Hodgkin lymphomas (NHL) of the stomach. Materials and methods : We studied endoscopic view of gastric lymphoma in 250 patients, in 160 (64%) patients lesions were regarded as the primary, and in 90 (36%) – as secondary. In our study all patients underwent standard videoendoscopic examination of the stomach, which included examination with magnification of 115 times, together with an analysis of microvascular picture with narrow spectrum analysis of mucosal and submucosal layers of the stomach wall, followed with chromogastroscopy using 0.4% solution of indigo carmine and endoscopic ultrasound. The diagnosis of lymphoma was verified by morphological and immunohistochemical methods. Results : According to our observations duration of the period of the presence of gastroenterological diseases until diagnosing of a tumor in the group of patients with primary lymphoma of the stomach is very variable: during the first 3 months diagnosis was established in 30.6% (n=49) of patients, during 6 months – in 18.1% (n=29) of patients, during period from 6 to 12 months in 15.6% (n=25) of patients and it should be noted that nearly a third of patients (27.5%, n=44) diagnosis of primary gastric lymphoma was established after one year. Macroscopic forms of gastric tumor was presented by the following variants: exophytic – in 22 patients (8.8%), infiltrative – in 54 (21.6%), ulcerative – in 37 (14.8%), infiltrative-ulcerous – in 71 (28.4% ), gastritis-like - in 36 (14.4%) and mixed - in 30 (12.0%).. Another direction of our study was to identify the characteristics of the macroscopic manifestations depending on the morphological variant of gastric lymphoma. According to presented data gastritis-like variant dominated in patients with MALT-lymphoma (in 34.3%), infiltrative-ulcerous variant dominated in patients with diffuse large B-cell lymphoma of MALT type, as in patients with diffuse large B-cell lymphoma - in 45.0% and 41.1% respectively. Infiltrative variant was most frequent in patients with follicular lymphoma (31.8%), and ulcerative variant was dominated in patients with Burkitt's lymphoma (60.0%). Conclusion : the most informative diagnostic criteria for endoscopic gastric NHL were established.

Multiple Intestinal Lymphoma

Gastrointestinal tract is the most common location for extralymphonodular lymphomas. The small intestine is affected only in 9% of the cases. Intestinal lymphoma may have single or multiple location. This paper describes a case of multiple location in the small intestine of a non-Hodgkin B-cell in a 53 years old patient, who was initially diagnosed with bilateral pneumonia with pleurisy with E. coli, steeper on the right side, but the persistence of symptoms as fever, malaise, despite appropriate treatment, required further investigation. The CT exam observed fluid collection in the hypogastrium around a digestive loop. The patient underwent surgery, the intraoperative foundings being: a large mesenteric tumor - 5 cm in diameter, a terminal ileal mesenteric tumor, a mesenteric tumor - 6 cm in diameter, omentum with nodular formations, a tumor - 3.3/2.5.1 cm in the abdominal wall, pseudotumoral appendix. Segmental. enterectomy with entero-enterostomy, excision of mesenteric tumors, appendectomy and omentectomy were performed. Pathological diagnosis was non-Hodgkin marginal zone B-cell MALT type lymphoma of the small intestine with extension to the appendix, meso, omentum and abdominal wall. Postoperatively, the patient received chemotherapy for remission.

Disseminated Gastric MALT Lymphoma with Monoclonal Gammopathy, t(11;18)(q21;q21), and Subsequent Development of T-Large Granular Lymphocytic Leukemia: A Case Report and Review of the Literature.

Background. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is a well-characterized entity that may share clinical and morphological findings with other low-grade non-Hodgkin's lymphomas. Dissemination of MALT-type lymphoma to bone marrow and peripheral blood simultaneously with the presence of T-large granular cell leukemia (T-LGL) has rarely been reported. Case Presentation. This is the case of a 42-year-old male who presented with a gastric MALT-type lymphoma, disseminated to the bone marrow and the peripheral blood with high serum IgM levels and t(11;18)(q21;q21). The morphological, immunophenotypical and, immunohistochemical studies of the successive bone marrow and peripheral blood samples had revealed the coexistence of two distinct lymphoma cell populations: a B-cell, marginal zone type population expressing CD19, CD20, CD22, CD79b, IgM, and kappa light chain, and a T-large granular cell population, developed after treatment with rituximab expressing CD3, CD8, CD5, CD7, and CD45. Conclusion. Based on the analysis of this unusual case we performed an extensive review of the literature to elucidate the relationship between T-LGL and B-cell lymphomas and to emphasize the importance of paraprotein analysis at diagnosis of gastric MALT lymphoma.

シェーグレン症候群を合併した皮膚原発marginal zone B-cell lymphom of MALT typeの1例

シェーグレン症候群を合併した皮膚原発marginal zone B-cell lymphom of MALT typeの1例

Folliculotropic T‐cell infiltrates associated with B‐cell chronic lymphocytic leukaemia or MALT lymphoma may reveal either true mycosis fungoides or pseudolymphomatous reaction: seven cases and review of the literature

Mycosis fungoides (MF) and pseudo-MF (or MF simulant) can be associated with B-cell malignancies, but distinction between a true neoplasm and a reactive process may be difficult. To report seven patients with B-cell malignancy and folliculotropic MF or pseudo-MF and emphasize on criteria allowing distinction between the two conditions. We retrospectively and prospectively included seven patients with B-cell malignancy who presented skin lesions histologically consisting in a folliculotropic T-cell infiltrate and reviewed the literature on the topic. Four men and three women had a chronic lymphocytic leukaemia (n=6) or a MALT-type lymphoma (n=1). Five patients had localized papules, and two had patches and plaques. Histological examination showed in all cases a diffuse dermal T-cell infiltrate with folliculotropic involvement and follicular mucinosis associated with clusters of the B-cell lymphoma, without significant expression of follicular helper T-cell markers. T-cell rearrangement studies showed a polyclonal pattern in the patients with papules and a monoclonal pattern in the cases of patches and plaques. Papular lesions had an indolent evolution, whereas patches and plaques persisted or worsened into transformed MF. Folliculotropic T-cell infiltrates associated with B-cell malignancies can be either a true folliculotropic MF or a pseudo-MF. The distinction between both conditions cannot rely only on the histopathological aspect, but needs both a clinical pathological correlation and the search for a dominant T-cell clone. Whether the neoplastic T and B cells derive from a common ancestor or the T-cell proliferation is promoted by the underlying B-cell lymphoma remains unsolved, but interaction between B and T cell in the skin does not appear to be dependent on a TFH differentiation of the T-cell infiltrate.