Atypical presentation of Sjogren's syndrome with MALT lymphoma mimicking chronic venous ulcers / Apresentação atípica da síndrome de Sjogren com linfoma MALT que mimetiza úlceras crônicas venosas

Abstract Chronic ulcerations of the lower extremities are quite a common condition amongst adults, most often caused by chronic venous insufficiency. Irrespective of the main underlying cause, chronic limb ulcerations are usually associated with significant symptoms, impairing daily functioning. Improper or delayed diagnosis and inadequate treatment increase the risk of serious complications, including limb amputations. Malignancies can develop secondary to chronic leg ulcers. About 2.4% of ulcers arising from chronic venous stasis undergo malignant transformation. Squamous cell carcinoma is the most common type of malignancy found in chronic leg ulceration biopsies. Basal cell carcinoma, sarcoma, and melanoma have all been documented infrequently. In the case described here, we found lymphoma of the marginal zone of mucosa-associated lymphoid tissue (MALT), which is an extremely rare cutaneous neoplasm of the lower extremities, but one that may have an association with autoimmune diseases.

Resumo Úlceras crônicas dos membros inferiores são uma condição bastante comum entre adultos, na maioria das vezes causadas por insuficiência venosa crônica. Independente da sua causa principal, úlceras crônicas dos membros estão geralmente associadas a sintomas significativos, prejudicando o funcionamento diário. O diagnóstico inadequado ou tardio e o tratamento inadequado aumentam o risco de complicações graves, inclusive o risco de amputação do membro. Malignidades podem se desenvolver secundariamente em úlceras crônicas da perna. Em torno de 2,4% das úlceras decorrentes de estase venosa crônica sofrem transformação maligna. O carcinoma de células escomosas é o tipo de malignidade mais comum que pode ser encontrada em biópsias de úlceras crônicas da perna. Carcinoma basocelular, sarcoma e melanoma foram raramente documentados. No caso aqui descrito, encontramos linfoma da zona marginal do tecido linfoide associado à mucosa (mucosa-associated lymphoid tissue, MALT), o qual é uma neoplasia cutânea dos membros inferiores extremamente rara mas que pode estar associada a doenças autoimunes.

Management of Suspicious Mucosa-Associated Lymphoid Tissue Lymphoma in Gastric Biopsy Specimens Obtained during Screening Endoscopy

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.

Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report / Miosite do músculo reto medial como forma atípica de apresentação de linfoma tipo MALT: relato de caso

Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT). Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

Descrevemos um raro caso de miosite do músculo reto medial como forma atípica de apresentação de linfoma não-Hodgkin de células B tipo MALT. A anatomia patológica e imuno-histoquímica do músculo afetado confirmaram o diagnóstico definitivo do caráter neoplásico da doença. As miosites orbitárias têm como principal etiologia a oftalmopatia de Graves, porém diversas outras causas podem apresentar-se dessa forma. Sendo assim, as causas neoplásicas devem ser descartadas. O linfoma não-Hodgkin de células B tipo MALT é o tipo histológico mais comum de linfoma orbitário, as regiões mais frequentemente acometidas são a conjuntiva e glândula lacrimal. No entanto, pode apresentar-se com formas clínicas atípicas, acometendo outras regiões e tecidos.

Treatment Outcome for Gastric Mucosa-Associated Lymphoid Tissue Lymphoma according to Helicobacter pylori Infection Status: A Single-Center Experience

BACKGROUND/AIMS: Helicobacter pylori eradication therapy has been used as a first-line treatment for H. pylori-positive gastric mucosa-associated lymphoid tissue (MALT) lymphoma. However, the management strategy for H. pylori-negative MALT lymphoma remains controversial. Therefore, the aim of this study was to examine the success rate of each treatment option for H. pylori-positive and H. pylori-negative gastric MALT lymphomas. METHODS: In total, 57 patients with gastric MALT lymphoma diagnosed between December 2000 and June 2012 were enrolled in the study. The treatment responses were compared between H. pylori-positive and H. pylori-negative gastric MALT lymphomas. RESULTS: Of the 57 patients, 43 (75%) had H. pylori infection. Forty-eight patients received H. pylori eradication as a first-line treatment, and complete remission was achieved in 31 of the 39 patients (80%) with H. pylori-positive MALT lymphoma and in five (56%) of the nine patients with H. pylori-negative MALT lymphoma; no significant difference was observed between the groups (p=0.135). The other treatment modalities, including radiation therapy, chemotherapy, and surgery, were effective irrespective of H. pylori infection status, with no significant difference in the treatment response between H. pylori-positive and H. pylori-negative MALT lymphomas. CONCLUSIONS: H. pylori eradication therapy may be considered as a first-line treatment regardless of H. pylori infection status.

New Guidelines for Helicobacter pylori Treatment: Comparisons between Korea and Japan / 대한소화기학회지

Korea and Japan show the highest incidence of gastric cancer and Helicobacter pylori infection. New 2013 guidelines on H. pylori infection differ between the two countries with regard to the indications for H. pylori eradication, diagnostic methods, and treatment regimens. Indications for eradication in Korean guideline focus on specific diseases such as peptic ulcer disease, low-grade gastric mucosa-associated lymphoid tissue lymphoma, and after resection of early gastric cancer, while Japanese guideline includes all H. pylori-associated gastritis for the prevention of dissemination. With regard to the diagnosis, either noninvasive or invasive method (except for bacterial culture) is recommended in Korea, while two noninvasive tests including serum anti-H. pylori IgG antibody level are preferred in Japan. As for the treatment regimens, second-line treatment (quadruple bismuth-containing regimen) is recommended without first-line triple therapy in areas of high clarithromycin resistance in Korea. However, there is no bismuth-based second-line treatment in Japan, and the Japanese regimen consists of a lower dose of antibiotics for a shorter duration (7 days). Such discrepancies between the two countries are based not only on the differences in the literature search and interpretation, but also on the different approvals granted by the national health insurance system, manufacturing process of the antibiotics, and diagnostic techniques in each country. Collaborations are required to minimize the discrepancies between the two countries based on cost-effectiveness.

Limited Role of Bone Marrow Aspiration and Biopsy in the Initial Staging Work-up of Gastric Mucosa-Associated Lymphoid Tissue Lymphoma in Korea

BACKGROUND/AIMS: The aim of this study was to investigate the frequency of disseminated gastric mucosa-associated lymphoid tissue (MALT) lymphoma and the role of bone marrow study in the initial staging work-up. METHODS: A total of 194 patients with gastric MALT lymphoma was enrolled. The incidence of disseminated disease was evaluated in the initial staging work-up. The demographic data and tumor characteristics were compared according to Helicobacter pylori infection status. RESULTS: Localized disease of Lugano stage I accounted for 97.4% of the enrolled cases. Abdominal computed tomography revealed abdominal lymph node metastasis in five patients (2.6%). Bone marrow (BM) involvement was found in only one patient without H. pylori infection (0.5%). No patient showed positive findings on chest computed tomography or positron emission tomography. H. pylori-negative cases showed a significantly higher frequency of advanced-stage disease than H. pylori-positive cases (10.0% vs 0.6%). In patients achieving complete remission, no extragastric recurrence occurred during follow-up. CONCLUSIONS: The incidence of disseminated disease, including BM involvement, was very low in Korean gastric MALT lymphoma patients. It might be beneficial to perform BM aspiration and biopsy as a part of staging work-up only in patients with risk factors for advanced disease such as H. pylori negativity.

Guidelines for the Diagnosis and Treatment of Helicobacter pylori Infection in Korea, 2013 Revised Edition / 대한소화기학회지

Since the Korean College of Helicobacter and Upper Gastrointestinal Research has first developed the guideline for the diagnosis and treatment of Helicobacter pylori infection in 1998, the revised guideline was proposed in 2009 by the same group. Although the revised guideline was made by comprehensive review of previous articles and consensus of authoritative expert opinions, the evidence-based developmental process was not applied in the revision of the guideline. This new guideline has been revised especially in terms of changes in the indication and treatment of H. pylori infection in Korea, and developed by the adaptation process as evidence-based method; 6 guidelines were retrieved by systematic review and the Appraisal of Guidelines for Research and Evaluation (AGREE) II process, 21 statements were made with grading system and revised by modified Delphi method. After revision, 11 statements for the indication of test and treatment, 4 statements for the diagnosis and 4 statements for the treatment have been developed, respectively. The revised guideline has been reviewed by external experts before the official endorsement, and will be disseminated for usual clinical practice in Korea. Also, the scheduled update and revision of the guideline will be made periodically.

Linfoma esplénico de zona marginal como causa de esplenomegalia masiva: reporte de caso clínico y revisión de la literatura / Splenic marginal zone lymphoma as cause for massive splenomegaly: a clinical case and literature review

Massive splenomegaly is in the which the growth of the spleen has spread to other quadrants of the abdomen. It is produced by a limited number of pathologies, both benign and malignant. It is presented a case of a 62 year-old woman who is consulting for four years of progressive increase in her abdominal volume, associated to the feeling of abdominal fullness, dyspnea on moderate exertion and lower extremities edema. At the physical examination was observed massive splenomegaly and jaundice. The hemogram showed pancytopenia and a lymphocyte count of80 percent. The myelogram revealed marrow infiltration by lymphocytes of mature appearance. Flow cytometry of peripheral blood showed 70 percent of lymphocytes, which expressed B cells markers CD19, CD20, CD23and FMC7 in addition to Kappa light chain restriction, suggesting marginal splenic zone lymphoma. The bone marrow biopsy showed lymphoid small cells infiltrate with positive markers CD20, CD5,CD23 and negative cyclin D1 study. BCL-2 was also positive. It was considered unfit to receive chemotherapy and was treated with 4 cycles of rituximab, with significant decrease of splenic size.

Primary cutaneous marginal zone lymphoma (immunocytoma like) with lymphoepithelioid or Lennert's lymphoma like involvement of nodes.

Primary cutaneous marginal zone lymphomas (PCMZL) have a wide range of morphology from tumors with monocytoid B cells to those composed entirely of plasma cells and the T-cell rich variants. We report a 60-year-old male with a PCMZL rich in plasma cells of the foot with a lymphoepithelioid-like pattern of dissemination to the lymph nodes posing problems in the diagnosis. The patient had a lesion on the dorsum of the foot which histologically revealed dense perivascular collections of lymphoid cells and plasma cells amidst fibrous tissue. Though the plasma cells did show light chain restriction, CD20 and CD3 did not reveal an overwhelming B/T-cell population and hence a diagnosis of a reactive process was offered. Subsequently the patient developed inguinal nodes with diffuse loss of architecture and replacement by epithelioid histiocytes and reactive T cells with few large B cells (lymphoepithelioid-like pattern). On pathology review it was realized that the two lesions may be related and clonality studies were asked for. The skin lesion showed clonally rearranged IgH receptor while the T-cell receptor rearrangement was negative. The patient developed disseminated disease and received six cycles of chemotherapy with partial response and 6 years after the initial presentation was alive with nonprogressive disease. Thus, the polymorphous background in PCMZL is evolving and an immunocytoma-like tumor can show a T-cell rich or Lennert's like growth pattern of spread and early recognition these odd patterns may aid in appropriate management of patients.

Linfoma MALT gástrico, gastritis linfocitaria y enfermedad celíaca: caso clínico con seguimiento a largo plazo, post erradicación de Helicobacter pylori y regresión del MALT. Correlación Clínico-Endoscópica-Patológica / Gastric MALT lymphoma, lymphocytic gastritis and celiac disease: case report with long-term follow-up post helicobacter pylori eradication and regression of MALT: clinical-pathological and endoscopic correlation

A case report of 61 year old female patient with diarrhea syndrome, whose study showed the presence of low-grade MALT gastric lymphoma associated with lymphocytic gastritis and celiac disease. During a six-year period of follow-up, the eradication of Helicobacter pylori determined the remission of the gastric lymphoma and lymphocytic gastritis; celiac disease showed variable activity according to their adherence to dietary treatment. We discuss possible associations and etiology of MALT lymphoma and lymphocytic gastritis, and of the latter with celiac disease.

Se presenta caso de paciente mujer de 61 años con cuadro de síndrome diarreico, cuyo estudio demuestra la presencia de linfoma MALT de bajo grado asociado a gastritis linfocitaria y enfermedad celíaca. Durante su seguimiento de 6 años, la erradicación de Helicobacter pylori, determinó la remisión del linfoma y gastritis linfocitaria, mientras la enfermedad celíaca mostró actividad variable de acuerdo con su adherencia al tratamiento dietético. Se discuten las posibles asociaciones y etiología de linfoma MALT y gastritis linfocitaria, y de esta última con enfermedad celíaca.

Could Antibiotic Resistance Rate of Helicobacter pylori be Different according to Gastroduodenal Diseases? / 대한소화기학회지

No abstract available.

Helicobacter pylori Eradication Therapy in Korea / 대한소화기학회지

Helicobacter pylori (H. pylori) is known to be associated with many gastrointestinal diseases including peptic ulcer. In Korea, eradication of H. pylori is recommended for peptic ulcer disease, low grade gastric mucosa-associated lymphoid tissue lymphoma, and early gastric cancer. Standard triple therapy using proton pump inhibitor, clarithromycin, and amoxicillin and bismuth-containing quadruple therapy have been the main first-line and second-line therapy for H. pylori in Korea. Although eradication rate of second-line quadruple therapy remains similar to that of the past, the success rate of eradication with triple therapy has decreased with increasing antimicrobial resistance to H. pylori. There is no standard third-line therapy, and some regimens that incorporate levofloxacin, moxifloxacin, and rifabutin can be used. New regimens such as sequential or concomitant therapy are suggested as alternative treatment for H. pylori. We need more well designed randomized controlled studies to choose proper treatment for H. pylori infection.

Diagnosis and Treatment of Gastric MALT Lymphoma / 대한소화기학회지

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma represents approximately 40% of gastric lymphomas, and its incidence is increasing. An early diagnosis for gastric MALT lymphoma is important, but not easy due to non-specific symptoms and endoscopic findings. Diagnosis is based on the histopathologic evaluation of multiple, deep and repeated biopsies taken from normal and any abnormal appearing sites of the stomach. In addition, the presence of Helicobacter pylori (H. pylori) infection must be determined to determine therapeutic approach. Endoscopic ultrasonography (EUS) is essential for the evaluation of regional lymph nodes and the depth of tumor invasion in the gastric wall, for predicting response to H. pylori eradication, and for monitoring tumor regression or recurrence. The eradication of H. pylori is recommended as an initial treatment for low-grade gastric MALT lymphoma with H. pylori infection. Both radiation therapy and chemotherapy are suitable alternative options for H. pylori-negative, refractory, or high-grade gastric MALT lymphoma. But, the role of surgery is diminishing. After treatment, strict endoscopic regular follow-up including EUS is recommended with multiple biopsies. However, controversy remains regarding the best diagnosis, treatment and follow-up strategy for this disease.

Marginal Zone B-cell Lymphoma of MALT in Small Intestine Associated with Amyloidosis: A Rare Association

A 62-yr-old man presented with a 5-yr history of intermittent abdominal distention and pain. These symptoms persisted for several months and subsided without treatment. A diagnosis of suspected small bowel lymphoma was made based on plain radiograph and computerized tomogram findings, and he was referred to our institution for further evaluation. Segmental resection of the small intestine was performed and the diagnosis of marginal zone B-cell lymphoma associated with amyloidosis was made. This is the first case of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in the small intestine associated with amyloidosis in Korea.

Unusual Cause of Acute Right Ventricular Dysfunction: Rapid Progression of Superior Vena Cava Aneurysm Complicated by Thrombosis and Pulmonary Thromboembolism

Aneurysms of the major thoracic veins are rare. They are usually asymptomatic and thus treated conservatively. We report an extremely rare case of rapidly progressing superior vena cava (SVC) aneurysm complicated by thrombosis and acute pulmonary thromboembolism (PTE) with right ventricular dysfunction. Thrombolytic therapy for hemodynamically significant acute PTE was harmful to the patient in the present case, because it induced further thrombosis and mobilization of the thrombi within the aneurysm, subsequently causing de novo PTE. Surgical aneurysmectomy combined with pulmonary artery embolectomy would be a treatment of choice in patients with SVC aneurysm complicated by acute PTE.

A Case of Monoclonal Gammopathy in Extranodal Marginal Zone B-cell Lymphoma of the Small Intestine / 대한진단검사의학회지

Monoclonal gammopathy occurs in one-third of the patients with mucosa-associated lymphoid tissue lymphoma (MALT lymphoma). However, monoclonal gammopathy has been rarely reported in Korea. Paraprotenemia accompanying MALT lymphoma is strongly correlated with involvement of the bone marrow, and this involvement leads to the progression of the disease. Here, we present a case of a 66-yr-old man diagnosed with IgM monoclonal gammopathy and stage IV extranodal marginal zone lymphoma of the small intestine, with the involvement of the bone marrow.

Helicobacter pylori Eradication for Stage IE1 Gastric Mucosa-associated Lymphoid Tissue Lymphoma: Predictive Factors of Complete Remission / 대한소화기학회지

BACKGROUND/AIMS: Eradication of Helicobacter pylori (H. pylori) is accepted as initial treatment of stage IE1 gastric mucosa associated lymphoid tissue (MALT) lymphoma. However, 10-20% of gastric low grade MALT lymphomas are unresponsive to H. pylori eradication treatment. The aim of this study was to find out the predictive factors of complete remission of gastric MALT lymphoma after H. pylori eradication. METHODS: From 1995 to 2006, consecutive 95 patients with modified Ann Arbor stage I(E1) gastric MALT lymphoma were enrolled, and their medical records were reviewed. The patients were initially treated by H. pylori eradication. The complete remission was determined by endoscopic and histologic finding. RESULTS: Eighty eight patients (92.6%) achieved complete remission after H. pylori eradication therapy. Mean follow up time for these patients was 40+/-25 months. Seven patients (7.4%) failed to achieve complete remission. There was no significant difference in the age, sex, endoscopic appearance, and large cell component between the remission group and failure group. Among 66 patients with distal tumor, 65 patients (98.5%) achieved complete remission. On the other hand, among 13 patient with proximal tumor, 9 patients (69.2%) achieved complete remission (p=0.001). The odds ratio of proximal tumor for H. pylori eradication failure was 28.9 (95% CI=2.9-288.0). CONCLUSIONS: The proximally location of MALT lymphoma is a risk factor of the H. pylori eradication treatment failure. Therefore, the proximally located gastric MALT lymphoma should be carefully treated and followed.

Elevada prevalencia de infección por helicobacter pylori en el linfoma gástrico de tipo MALT: [revisión] / Prevalence of helicobacter pylori infection in gastric MALT lymphoma: [review]

Objetivo: Realizar una revisión sistemática de los estudios que evalúan la prevalencia de Helicobacter pylori en los pacientes con linfoma MALT, así como analizar los factores de los que depende. Métodos: Se efectuó una búsqueda bibliográfica en Pubmed y se seleccionaron los artículos en los que se estudiaba la prevalencia de H. pylori en pacientes con linfoma MALT. Resultados: Se identificaron 38 estudios que incluían un total de 1 844 pacientes. La prevalencia media global de infección por H. pylori fue del 79%. En pacientes en los que se utilizaron dos o más métodos para el diagnóstico de H. pylori la prevalencia fue del 85%, frente al 77% cuando se empleó un método diagnóstico (p < 0.0001). La prevalencia de H. pylori en pacientes diagnosticados mediante histología fue del 75% frente al 85% cuando se utilizó serología (p < 0.0001). La prevalencia de H. pylori en los linfomas MALT de alto grado fue del 60%, frente al 79% en los de bajo grado (p < 0.0001). Se detectó la infección en el 74% de los linfomas MALT confinados a la submucosa, y sólo en el 44% de aquellos que sobrepasaban la submucosa (p < 0.0001). Conclusiones: La prevalencia de la infección por H. pylori en pacientes con linfoma MALT gástrico parece depender del número y tipo de técnicas diagnósticas utilizadas para detectar la infección, del grado histológico y de la profundidad de la invasión tumoral. Si se utilizan los métodos diagnósticos adecuados y si se consideran únicamente los linfomas de bajo grado la prevalencia de infección por H. pylori es muy elevada, cercana al 90%.

Primary Biliary Lymphoma Mimicking Cholangiocarcinoma: A Characteristic Feature of Discrepant CT and Direct Cholangiography Findings

Primary non-Hodgkin's lymphoma arising from the bile duct is extremely rare and the reported imaging features do not differ from those of cholangiocarcinoma of the bile duct. We report a case of a patient with extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue (MALT), who presented with obstructive jaundice and describe the distinctive radiologic features that may suggest the correct preoperative diagnosis of primary lymphoma of the bile duct. Primary MALT lymphoma of the extrahepatic bile duct should be considered in the differential diagnosis when there is a mismatch in imaging findings on computed tomography or magnetic resonance imaging and cholangiography.

Small Intestinal Perforation Caused by Primary Jejunal MALT Lymphoma / 대한소화기학회지

No abstract available.