*OD, FAAO Nova Southeastern University, Fort Lauderdale, Florida.
Lymphoma is the most common malignancy of the ocular adnexa. Most of the ocular adnexa lymphomas are non-Hodgkin B-cell lymphomas. The most common type of ocular adnexa lymphoma is primary extranodal marginal zone B-cell lymphoma of the MALT (mucosa-associated lymphoid tissue). Most of these neoplasms are primary extranodal lymphomas, although 10% to 32% are secondary tumors from disseminated disease.
A 58-year-old woman presented for a comprehensive examination, with the chief complaint of ocular discomfort in both eyes. Anterior segment examination revealed bilateral salmon-colored lesions of the inferior and superior conjunctivae. The patient was referred for systemic evaluation and histopathology of the conjunctival lesions. She was diagnosed as having marginalzonelymphoma of the MALT and underwent radiation therapy (RT).
Ocular lymphoma may present on routine examination or with mild symptoms. Although most commonly a primary extranodal neoplasm, the condition may be associated with disseminated lymphoma and requires thorough evaluation and staging of the disease for determination of appropriate treatment. The primary eye care provider plays an important role in the identification and staging of the disease, as well as managing complications from RT. It is also important to recognize that concurrent conditions requiring treatment with topical medications, such as glaucoma, may be complicated after treatment because of the inflammation and ocular surface irritation after RT. The necessity and benefit of the addition of intraocular pressure medications during that time should be measured on a case-by-case basis. Patients should be followed closely after treatment for relapse of disease and identification of complications from ocular RT.
Medical Genomics Research Group, Molecular Medicine Program, CEITEC/Central European Institute of Technology, Masaryk University, Brno, Czech Republic.
Recent research has revealed the existence of subsets (clusters) of patients with different types of B-cell lymphomas and leukemias with restricted, "stereotyped" immunoglobulin (IG) variable heavy complementarity-determining region 3 (VH CDR3) sequences within their B cell receptors (BcR), suggesting selection by common epitopes or classes of structurally similar epitopes. BcR stereotypy was initially described in chronic lymphocytic leukemia (CLL), where it constitutes a remarkably frequent feature of the IG repertoire, and subsequently identified in other malignancies, including mantle cell lymphoma and splenic marginal-zonelymphoma. Of note, at least in CLL, emerging evidence indicates that the grouping of cases into distinct clusters with stereotyped BcR is functionally and prognostically relevant. Hence, the reliable identification of BcR stereotypy may assist in the investigation of the nature of the selecting antigens and immune pathways leading to lymphom adevelopment, and also potentially pave the way for tailored treatment strategies applicable to each major stereotyped subset. In this chapter, we provide an overview of BcR stereotypy in human B-cell malignancies, and outline previous and current methodological approaches used for its identification.
Nuclear Medicine Department, Clinic University Hospital, Barcelona, Spain, email@example.com.
Interim (18)F-FDG PET performed early during the course of therapy in diffuse large B-cell lymphoma (DLBCL) is a good predictor of outcome. However, interpretation criteria for interim PET for the evaluation of tumour response are still not clearly defined. The study aim was to assess whether interim PET can predict overall survival (OS) and progression-free survival (PFS) in DLBCL patients following three different sets of parameters, two qualitative (visual) methods and one semiquantitative.
A total of 50 newly diagnosed DLBCL patients were prospectively enrolled in this study. All patients had a PET/CT scan at diagnosis and an interim PET/CT scan after the second or third cycle of chemotherapy. Three methods of evaluation for the interim PET/CT were used: a qualitative three-point scoring (3-PS) method, a qualitative 5-PS method and a semiquantitative method (ΔSUV(max)). The degree of correlation between therapy response seen on FDG PET and PFS and OS was determined.
The analysis of the visual 3-PS method showed no statistically significant difference in PFS and OS. The estimated 5-year PFS and OS were 79 % and 92 %, respectively, in patients with an interim PET scan showing uptake not greater than in the liver versus 50 % in patients with uptake greater than in the liver, and this difference was statistically significant. The optimal cut-off value of ΔSUV(max) that could predict the PFS and OS difference in patients with DLBCL was 76 % (95 % CI 62.7-89.2 %) and 75 % (95 % CI, 54.6-95.4 %), respectively.
Our results support the use of liver uptake as an indicator in the qualitative evaluation of interim PET, or a ΔSUV(max) greater than 75 % in semiquantitative analysis. Interim PET may predict PFS and OS and could be considered in the prognostic evaluation of DLBCL.
Hospital das Clínicas da Faculdade de Medicina, Universidade de São Paulo - USP, São Paulo, SP, Brazil.
Diffuse large B-Cell lymphoma is the most common subtype of non-Hodgkin lymphoma in the West. In Brazil, it is the fifth cause of cancer, with more than 55,000 cases and 26,000 deaths per year. At Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo - HCFMUSP, diffuse large B-Cell lymphoma represents 49.7% of all non-Hodgkinlymphoma cases. Initially, the classification of non-Hodgkin lymphoma was based on morphology, but advances in immunology and molecular medicine allowed the introduction of a biological classification for these diseases. As for other cancers, non-Hodgkin lymphoma involves patterns of multifactorial pathogenesis with environmental factors, as well as genetic, occupational and dietary factors, contributing to its development. Multiple lesions involving molecular pathways of B-cell proliferation and differentiation may result in the activation of oncogenes such as the BCL2, BCL6, and MYC genes and the inactivation of tumor suppressor genes such as p53 and INK4, as well as other important transcription factors such as OCT-1 and OCT-2. A dramatic improvement in survival was seen after the recent introduction of the anti-CD20 monoclonal antibody. The association of this antibody to the cyclophosphamide, hydroxydaunorubicin, oncovin and prednisolone (CHOP) regimen has increased overall survival of diffuse large B-Cell lymphoma and follicular lymphoma patients by 20%. However, 50% of all diffuse large B-Cell lymphoma patients remain incurable, creating a demand for more research with new advances in treatment. Thus, it is important to know and understand the key factors and molecular pathways involved in the pathogenesis of diffuse large B-Cell lymphoma.
Department of Radiology, Hazrat Rasoul-e-Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Primary non-Hodgkin's lymphoma of the cranial vault is extremely rare. This case report presents a 42-year-old man with a painless subcutaneous scalp mass which extended intracranially associated with recent mild headache. Initial computed tomography and magnetic resonance imaging revealed two lesions emanating from the skull. Biopsy revealed a diagnosis of diffuse large B cell lymphoma (DLBCL). A thorough work-up revealed no other point of involvement. This case is concerned about considering lymphoma in the differential diagnosis of calvarial lesions with both intra- and extra cranial extensions but without obvious intervening bony destruction.
A 59-year-old man presented with acalculous cholecystitis and hepatomegaly. Routine laboratory tests were remarkable for thrombocytopenia (125 × 109/L), alanine aminotransferase 95 U/L, aspartate aminotransferase 760 U/L, alkaline phosphatase 223 U/L, and lactate dehydrogenase 3755 U/L. A peripheral smear showed morphologically normal red blood cells and large atypical lymphoid cells (panel A and inset). Cholecystectomy, liver biopsy, and bone marrow biopsy were performed. Flow cytometry on bone marrow and peripheral blood demonstrated a population of λ-restricted B cells that were positive for CD19, CD20, and CD22, and coexpressed CD5 and CD10. Histologic sections showed large atypical lymphoid cells within small subserosal vessels of the gallbladder, as well as within hepatic (panel C) and bone marrow sinusoids (panel B). Immunohistochemistry showed strong positivity for CD20, PAX-5, CD5, CD10, and Ki-67. Fluorescent in situ hybridization revealed a distal deletion of BCL6 and 3 to 5 copies of BCL2. Cerebrospinal fluid examination was unremarkable.
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal large B-cell lymphoma characterized by intraluminal growth of lymphoma cells within small vessels. Circulating lymphoma cells in the peripheral blood happens occasionally. Once called the oncologist's “great imitator,” IVLBCL can mimic, because of its intrinsically widespread nature, diseases in almost any organ and diagnosis requires high vigilance.
Department of Otolaryngology-Head and Neck Surgery, University of Rochester Medical Center, 601 Elmwood Ave., Box 629, Rochester, NY 14642, USA. RyanD_Walker@urmc.rochester.edu.
Uvular enlargement may occur acutely as a result of infection, allergy, or trauma. Squamous cell carcinoma may present as a progressively enlarging uvular mass. Primary MALT (mucosa-associated lymphoid tissue) lymphoma of the uvula and a neuroendocrine tumor of the parapharyngeal space presenting as a uvular mass have each been previously described in the literature. Here we present a case of low-grade B-cell lymphoma presenting as a uvular mass in a 55-year-old patient with progressive throat swelling and dysphagia.
Am old enough to understand the difference between the Bay of Pigs - and roasting a pig at a epicurian feast. Been thru the hippy, yippie and yuppie years - always remaining who I am.
Very much believe in "Sing your own song - weave your own tapestry"
Am young enough to still know the thrill of new discoveries, the beauty of the evening, to celebrate the joy of another tommorow.
Survived these many decades with a severe medical problems. Sorting out the maze of now having two lymphomas and all their nasty little companions, but I continue.
Besides, being a simple iconoclastic eclectic, have been called many things. An incurable romanticist - with a strong touch of reality. Thinker, intellectual (God, how I hate that term) - been told I am a lion with the heart of the poet.
Know how to wage war and conquer my foes - but would rather be known as one who brings hope and life. To bring hope into anothers life is the ultimate of joys.
Life should be about bringing hope, peace, vision... a sense of purpose beyond yourself.