Cell Lymphoma Presenting Research Articles

ALK‐positive large B‐cell lymphoma presenting with jejunal intussusception

European Journal of HaematologyVolume 90, Issue 3 p. 261-261 Clinical Picture ALK-positive large B-cell lymphoma presenting with jejunal intussusception Ya-Ping Chen, Ya-Ping Chen Department of Internal Medicine, National Cheng Kung University and Hospital, Tainan, TaiwanSearch for more papers by this authorLiang-Yi Hung, Liang-Yi Hung Institute of Bioinformatics and Biosignal Transduction, National Cheng Kung University, Tainan, TaiwanSearch for more papers by this authorYan-Shen Shan, Yan-Shen Shan Department of Surgery, National Cheng Kung University and Hospital, Tainan, TaiwanSearch for more papers by this authorKung-Chao Chang, Corresponding Author Kung-Chao Chang Department of Pathology, National Cheng Kung University and Hospital, Tainan, TaiwanCorrespondence Kung-Chao Chang, MD, PhD, Department of Pathology, National Cheng Kung University Hospital, 138 Sheng-Li Road, Tainan 704, Taiwan. Tel: 886 6 235 3535 ext. 2636; Fax: 886 6 276 6195; e-mail: [email protected]Search for more papers by this author Ya-Ping Chen, Ya-Ping Chen Department of Internal Medicine, National Cheng Kung University and Hospital, Tainan, TaiwanSearch for more papers by this authorLiang-Yi Hung, Liang-Yi Hung Institute of Bioinformatics and Biosignal Transduction, National Cheng Kung University, Tainan, TaiwanSearch for more papers by this authorYan-Shen Shan, Yan-Shen Shan Department of Surgery, National Cheng Kung University and Hospital, Tainan, TaiwanSearch for more papers by this authorKung-Chao Chang, Corresponding Author Kung-Chao Chang Department of Pathology, National Cheng Kung University and Hospital, Tainan, TaiwanCorrespondence Kung-Chao Chang, MD, PhD, Department of Pathology, National Cheng Kung University Hospital, 138 Sheng-Li Road, Tainan 704, Taiwan. Tel: 886 6 235 3535 ext. 2636; Fax: 886 6 276 6195; e-mail: [email protected]Search for more papers by this author First published: 22 November 2012 https://doi.org/10.1111/ejh.12031Citations: 4Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume90, Issue3March 2013Pages 261-261 RelatedInformation

MulticentricT‐cell lymphoma presenting as inferior palpebral swelling in a Standardbred mare

A 6-year-old Standardbred mare that presented with bilateral palpebral swelling and a left corneal ulcer was diagnosed with a multicentric T-cell lymphoma. Other clinical findings included submandibular lymphadenopathy and oedema, pharyngeal oedema and rhinitis. Prior to euthanasia the horse developed keratomalacia, the onset of which was coincidental with the use of high doses of topical and systemic corticosteroids. Although an unusual presentation, palpebral swelling should be considered as a clinical sign of lymphoma.

ALK‐positive anaplastic large cell lymphoma presenting as intradural spinal mass: First reported case and review of literature

Anaplastic large cell lymphoma (ALCL) is characterized by large anaplastic cells of T-cell or null-cell phenotype expressing CD30 (Ki-1 antigen). In most cases this neoplasm expresses the anaplastic lymphoma kinase (ALK), a chimeric protein resulting from the t(2;5)(p23;q35) translocation. ALK-positive anaplastic large cell lymphoma is most frequent in the first three decades of life and shows a male predominance, involving both nodal and extranodal sites, but rarely the CNS. We report a 21-year-old patient with a previous history of nodal ALK-positive ALCL, lymphohistiocytic subtype, who was admitted for recent occurrence of left-sided anesthesia with pain and progressive motor weakness of both legs. An MRI of the spine documented an intradural extramedullary mass dislocating the thoracic cord, suggesting a meningioma and the patient underwent surgical decompression. Histological examination revealed a lymphoproliferative neoplasm with morphology and immunophenotype of ALK-positive anaplastic large cell lymphoma. After surgery, all preoperative symptoms disappeared. To our knowledge, no cases of ALCL presenting as secondary localization with an intradural extramedullary spinal mass have been reported in the literature.

Mantle Cell Lymphoma Presenting as Chronic Polyposis

Purpose: A 71-year-old male presented for screening colonoscopy. Last colonoscopy, 10 years ago was negative for polyps, dysplasia or cancer. Past medical history was significant for thromobocytopenia, diagnosed in 2003. Family history was negative for GI cancer and polyps. Physical examination was negative. Colonoscopy from rectum to cecum was negative for any polyps, dysplasia or cancer. In Feb 2011, patient presented for his colonoscopy with no current acute GI symptoms. Colonoscopy revealed numerous 3-6 mm sessile polyps of benign appearance diffusely throughout the colon. Polypectomies showed extensive involvement by a markedly atypical lymphoid infiltrate with a nodular architectural pattern. The cells were monomorphous small to intermediate in size with uniform stippled chromatin, absence of nucleoli and mildly irregular nuclear contours. A panel of immunostains was performed showing atypical lymphoid infiltrate that were overwhelmingly B cell immunophenotype with CD 20 immunostain. There was also co expression of CD 5 and nuclear positivity for cyclin D-1. Based on the morphological findings and immunoprofile, the lymphoid infiltrate represents a neoplastic B cell lymphoproliferative disorder best classified as Mantle Cell lymphoma. In this clinical setting the B cell neoplasm was presenting as lymphomatous polyposis. CT and PET scans revealed metastases involving the jugular chain, supraclavicular, axillary, mediastinal, hilar and subcarinal lymph nodes. The spleen was mildly enlarged. After six cycles of Bendamustine and Rituximab therapy, repeat colonoscopy 9 months later revealed complete resolution of polyposis.Figure 1: Polyp at the transverse colon.Figure 2: Cyclin D immunostain.

Intravascular large B cell lymphoma presenting as anasarca: an unusual presentation of a rare lymphoma

Dear Editor, An 82-year-old male presented with progressive generalized edema over a month without associated fever, angina, dyspnea, rash, lymphadenopathy, and neurological or gastrointestinal symptoms. He was found to be profoundly hypotensive requiring vasopressor support. Initial blood work revealed severe hypoalbuminemia of 1.6 g/dL and a markedly elevated LDH of 1891 U/L. Complete blood count revealed normocytic anemia with hemoglobin of 9.8 g/dL and normal platelet and leukocyte count. Peripheral smear showed no abnormal cells. Liver function tests, urinalysis, chest X-ray, and echocardiogram were unremarkable. Random skin biopsies were subsequently performed revealing intravascular infiltration of CD5and CD20-positive malignant cells compatible with intravascular B cell lymphoma (IVLBCL; Fig. 1). IVLBCL is a rare malignancy with estimated incidence of less than one per million per year [1]. It was first described as endothelial cell tumor [2]. Subsequent immunohistochemical studies confirmed its lymphoidal nature [3]. In 2008, WHO classified IVLBCL as a subtype of extranodal diffuse large B cell lymphoma characterized by limited proliferation of lymphoma cell within lumina of small blood vessels [4]. Diagnosis of IVLBCL can be challenging given its bewildering presentation. Virtually, any organ can be affected as a result of small vessel occlusion. Fever, night sweat, and weight loss are the most common presentation. In western countries, these constitutional symptoms are frequently accompanied by neurological and cutaneous symptoms, whereas hemophagocytic syndrome is more predominant in Asian population [1, 5]. Anarsarca is an unusual initial presentation and was found in less than 5 % in one case series [5]. Biopsy is mandatory for the diagnosis. Involved tissues demonstrate large lymphoid cells with prominent nucleoli and frequent mitotic figures in the capillaries and venules. B cell-associated antigens, particularly CD20, CD5, and CD10, are typically positive. Selection of organ for biopsy should be guided by patient's manifestation as identification of tumor cells is relatively easy when an affected organ is biopsied [6]. In case of no apparent specific organ involvement, as seen in our patient, random skin biopsy from normal-appearing skin is an alternative diagnostic approach with a high sensitivity of 83.3 % [7]. Prognosis of a patient with IVLBCL is unfavorable although recent retrospective studies suggest more promising outcome with the use of rituximab in conjunction with anthracycline-based chemotherapy [1, 8]. Our patient, with unstable hemodynamics despite of vigorous resuscitation, was not a suitable candidate for instituting chemotherapy. Unfortunately, his clinical course deteriorated and he passed away after a week of hospitalization.

Characteristics and outcomes of diffuse large B‐cell lymphoma presenting in leukaemic phase

Diffuse large B-cell lymphoma (DLBCL) occasionally presents with circulating malignant cells. The clinical characteristics and long-term outcomes of these patients have not been described. Twenty-nine newly diagnosed DLBCL presenting in leukaemic phase were identified between 1996 and 2010, at two institutions. Median age was 48years, and patients presented with leucocytosis, high lactate dehydrogenase levels, B symptoms, and high International Prognostic Index score. Extra nodal site involvement was observed in all patients and affected the bone marrow (100%), spleen (62%), pleura/lung (41%), liver (21%), bone (17%), bowels (7%) and cerebrospinal fluid (14%). Blood lymphomatous cells co-expressed CD19, CD20, CD22, CD38, CD45, HLA-DR and FMC7 in >90%, and kappa or lambda light chain restriction in >50%. Ninety per cent received rituximab and anthracycline-based chemotherapy. Overall, remission was complete in 54% and partial in 31%; 15% had resistant disease. Median follow-up was 47months; 13 (45%) patients remain alive in complete remission. Median progression-free and overall survivals were 11·5 and 46·7months, respectively. In summary, patients with DLBCL in leukaemic phase present with high tumour burden and frequent involvement of extra nodal sites. In this uncommon DLBCL subgroup, anthracycline-based regimens with rituximab are associated with early morbidity and mortality, but yield approximately 50% 4-year survival.

Fatal ALK-negative systemic anaplastic large cell lymphoma presenting with disseminated cutaneous dome-shaped papules and nodules

Anaplastic large cell lymphoma (ALCL) is classified as a CD30 positive non-Hodgkin lymphoma. Systemic ALCL (S-ALCL) is further subdivided into two subgroups based on anaplastic lymphoma kinase (ALK) expression. In systemic ALCL, positive ALK expression correlates with a favorable prognosis, whereas negative ALK expression correlates with poorer overall survival. By definition, primary cutaneous ALCL (cut-ALCL) is limited to the skin and is uniformly ALK-negative. Cut-ALCL closely resembles LyP with regards to its benign clinical course and CD30 positivity. We describe a unique case of ALK-negative (ALK-) S-ALCL presenting with cutaneous disseminated dome-shaped papules.

A case of primary cutaneous anaplastic large cell lymphoma presenting with numerous patches, papules and nodules in a child

A case of primary cutaneous anaplastic large cell lymphoma presenting with numerous patches, papules and nodules in a child

Left Atrial Primary B Cell Lymphoma Presenting with Mitral Regurgitation

Primary cardiac lymphoma (PCL) is an extremely rare diagnosis. We present a case of a 38-year-old immunocompetent female who presented with dyspnea on exertion and chest pain and the echocardiography revealed a mass in the left atrium (LA) causing moderate mitral regurgitation and mimicking a left atrial myxoma. The patient was managed with excision of the mass and mitral valve replacement followed by chemotherapy. The histopathology revealed B-cell non-Hodgkin lymphoma (NHL) with tumor cells showing immunoreactivity with CD20 and negative for CD45RO.

Diffuse large B cell lymphoma presenting as Horner's syndrome in a patient diagnosed with neurofibromatosis type 1: a case report and review of the literature

IntroductionHorner's syndrome has a variety of etiologies ranging from benign to serious life-threatening conditions and has been infrequently reported as a presenting symptom of patients with lymphoid neoplasms. Only one case of Burkitt's lymphoma presenting with toothache, paresthesia, and Horner's syndrome has been described and no case reports of diffuse large B-cell lymphoma as the etiology of Horner's syndrome currently exist in the literature. In addition, lymphoid neoplasms have rarely been reported to occur in patients with neurofibromatosis type 1 despite an increased risk of many types of cancer in such cases.Case presentationA 28-year-old Thai man presented with a progressively enlarged left supraclavicular mass together with a significant weight loss and night sweating for four months. He also noticed hoarseness and ptosis of his left eye associated with double vision for two months. Physical examination revealed large supraclavicular lymphadenopathy and Horner's syndrome (ptosis, miosis, and anhydrosis) on the left side of his face. A large mediastinal mass was clearly detected by chest X-ray and computed tomography and subsequent lymph node biopsy provided a diagnosis of diffuse large B-cell lymphoma. Interestingly, the patient was also definitely diagnosed with neurofibromatosis type 1 from multiple café au lait macules, axillary freckles, three neurofibromas, multiple Lisch nodules, and a history of affected family members. He subsequently received chemotherapy with a good response. Twenty-seven cases of various types of lymphoid neoplasms previously reported to occur in neurofibromatosis type 1 patients were also extracted from the literature. All cases were non-Hodgkin lymphoma and the major subtype was T-cell. Only nine cases were B-cell lymphoma. The majority of cases were young with a median age at lymphoma diagnosis of 9.4 years (range 1.1 to 77 years). Two-thirds of the cases were boys or men. Other concomitant malignancies were brain tumor, colorectal cancer, pheochromocytoma, and acute lymphoblastic leukemia.ConclusionsWe describe for the first time a case of diffuse large B-cell lymphoma that occurred in a neurofibromatosis type 1 patient with Horner's syndrome. Horner's syndrome can be an initial manifestation of diffuse large B-cell lymphoma. Patients who present with a classical triad of Horner's syndrome should always be fully investigated for lymphomatous involvement, especially in the thorax. The exact molecular mechanism for diffuse large B-cell lymphoma development in neurofibromatosis type 1 cases remains to be elucidated.

A Case of Mantle Cell Lymphoma Presenting as a Small Intestinal Stricture

Jae Bum Park, M.D., Joong Goo Kwon, M.D., Jae Young Oh, M.D., Ji-Min Han, M.D., Jin Tae Jung, M.D., Eun Young Kim, M.D., Ho Gak Kim, M.D., Hun Mo Ryoo, M.D., Hyun Dong Chae, M.D., Chang Ho Cho, M.D. Departments of Internal Medicine, Surgery, and Pathology, Catholic University of Daegu School of Medicine, Daegu, Korea 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏 Mantle cell lymphoma (MCL) is a subtype of B-cell non-Hodgkin’s lymphoma (NHL), accounting for 3-10% of NHL. MCL involves the gastrointestinal (GI) tract in 10-30% of patients and common sites of MCL GI tract involvement are the colorectum and stomach, but any region of the GI tract may be involved. GI tract involvement by MCL usually presents in the form of multiple lymphomatous polyposis involving several segments of the GI tract. A few cases of MCL presenting with a GI tract stricture have been reported. Here, we present a rare case of a small intestinal stricture caused by MCL and review the literature of this disease. (Intest Res 2012;10:300-304) 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏

First Isolation of Cryptococcus uzbekistanensis from an Immunocompromised Patient with Lymphoma

Cryptococcus species are known agents of opportunistic infections in healthy and immunocompromised hosts. Here we describe the first case of Cryptococcus uzbekistanensis causing bone marrow infection in an elderly Asian man with undiagnosed T cell lymphoma presenting with fever of unknown origin, pancytopenia, and exposure to chicken manure.

Mantle Cell Lymphoma Presenting As Multiple Colonic Polyp: Case Report and Review of the Literature

Mantle Cell Lymphoma Presenting As Multiple Colonic Polyp: Case Report and Review of the Literature

Unusual Presentation of Mantle Cell Lymphoma of Gastrointestinal Tract

Purpose: A 65 year old man presented to the hospital because of abdominal pain. The patient developed abdominal pain two days prior to presentation, most severe in right lower quadrant which was intermittent, spasmodic and non radiating in nature. The patient also had night sweats, weight loss of about 15 pounds associated with loose stools over last 3 months. The abdominal examination revealed mild tenderness in the right lower quadrant but no organomegaly and guarding. Computed tomography (CT) of the abdomen revealed mesenteric lymphadenopathy and radiological findings suggestive of Ileo colonic intussusception. Subsequently, a colonoscopy done showed a diffuse area of severely nodular mucosa in the entire colon and a small bowel lesion appeared to be causing intussusception of the ileum into the ascending colon (Figure). Biopsy of this mass showed immuno phenotypic features consistent with CD5-negative mantle cell lymphoma. He was treated With Rituximab and hyper CVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone) for eight cycles alternating with a cycle of methotrexate and Cytarabine. CT abdomen and pelvis done at six weeks confirmed resolution of the entire disease and a colonoscopy at eight weeks showed normal appearing ileum. Discussion: Involvement of the gastrointestinal tract in MCL is seen up to 20% to 30% of the cases. MCL can involve any region of the gastrointestinal tract. The most commonly seen endoscopic finding of mantle cell lymphoma involving the gastrointestinal tract is “multiple lymphomatous polyposis”, characterized by multiple polypoid lesions involving the gastrointestinal tract. This case presents with a rare complication that occurred secondary to mantle cell lymphoma involving the gastrointestinal tract. So far, a very few cases of mantle cell lymphoma presenting with intussusception have been reported.Figure: Ileo colonic intussusception.

Successful treatment with tandem consolidation using 90yttrium-ibritumomab tiuxetan (Zevalin) and high-dose therapy with autologous PBSCT in a patient with relapsed mantle cell lymphoma presenting as multiple lymphomatous polyposis

Successful treatment with tandem consolidation using 90 yttrium-ibritumomab tiuxetan (Zevalin) and high-dose therapy with autologous PBSCT in a patient with relapsed mantle cell lymphoma presenting as multiple lymphomatous polyposis

Recurrent mantle cell lymphoma presenting as a solitary rectal mass

Recurrent mantle cell lymphoma presenting as a solitary rectal mass

Breast implant related Anaplastic Large Cell Lymphoma presenting as late onset peri-implant effusion

In the past 10 years there has been a number of case reports of lymphoma associated with implants. In January 2011 the US FDA (United States Food and Drug Administration) produced a review of the medical literature reporting an association between breast implants and Anaplastic Large Cell Lymphoma (ALCL). A common presenting feature is late onset effusion around the implant.1 We report the first case in New Zealand and add to the worldwide total of 34 reported cases.

CD8-positive primary cutaneous anaplastic large cell lymphoma presenting as multiple scrotal nodules and plaques

ejd.2011.1375 Auteur(s) : Hongjun Xu1, Jiali Qian1, Jin Wei1, Yan Zhao1, Cheng Zhou1, Dingbao Chen2, Jianzhong Zhang1 rmzjz@126.com 1 Department of Dermatology, Peking University People's hospital, 11# Xizhimen South Street, 100044 Beijing, China 2 Department of Pathology, Peking University People's hospital, 11# Xizhimen South Street, 100044 Beijing, China A 53-year-old man presented with multiple nodules and plaques on the scrotum for six months. The lesion began with several asymptomatic rice-sized [...]

Mantle Cell Lymphoma Presenting as a Saccular Cyst

Mantle Cell Lymphoma Presenting as a Saccular Cyst

Anaplastic lymphoma kinase–positive anaplastic large cell lymphoma presenting as retroperitoneal fibrosis

Retroperitoneal fibrosis is a rare fibroinflammatory condition involving the abdominal aorta, iliac vessels, and ureters that carries an association with several other autoimmune conditions. Most cases of retroperitoneal fibrosis are thought to be idiopathic. The disorder can affect all age groups but is most common in persons between the ages of 50 and 70 years. A subset of cases is associated with an underlying immunohematologic abnormality including lymphoma. We describe in this case report a highly unusual presentation of a young woman who died with a diagnosis of "idiopathic retroperitoneal fibrosis" based on multiple biopsy procedures. Postmortem examination, however, revealed disseminated anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. The clinical and histopathologic importance of this very unusual presentation of anaplastic lymphoma kinase-positive anaplastic large cell lymphoma with retroperitoneal fibrosis is discussed.