Retroperitoneal fibrosis due to B-cell non-Hodgkin lymphoma: Responding to rituximab!

Retroperitoneal fibrosis is a rare disease manifesting as chronic soft tissue fibrosis in the retroperitoneum, with potential anatomic and/or functional compromise of adjacent organs. It can be primary (idiopathic) or secondary to other conditions such as cancers, autoimmune disorders, or drugs. We report herein a 66-year-old patient with symptomatic retroperitoneal fibrosis leading to bilateral hydronephrosis and renal failure, in whom, after a complex diagnostic work-up and protracted clinical course, a B-cell non-Hodgkin lymphoma in the retroperitoneal space and several vertebral bodies was identified. The patient was treated with radiation therapy and weekly rituximab infusions, with resolution of hydronephrosis and lower back pain. We include a thorough literature review on etiopathogenesis, diagnosis, therapy, and prognosis of retroperitoneal fibrosis. A meticulous search for malignancy is necessary in this rare condition that, if positive, may have significant therapeutic and prognostic implications.

Coronary Vasospasm and Bowel Ischemia in a Patient with Metastatic Gastrointestinal Carcinoid.

We describe a case of a 60-year-old female with a history of metastatic carcinoid disease with liver involvement who developed coronary vasospasm and mesenteric ischemia. The carcinoid syndrome is known for its cardiac involvement most well characterized by fibrous tissue deposits on the endocardium.(1,2) Case reports of coronary artery vasospasm have been previously described and hypothesized to be mediated by vasoactive amines and polypeptides synthesized by the tumor.(3-9) Intestinal ischemia is another reported complication of the carcinoid syndrome and is hypothesized to have a similar mechanism to that of the coronary vasospasm.(10-17) We have reviewed the literature and describe a case of coronary vasospasm and mesenteric ischemia in a patient on octreotide therapy. This is the first case in which we have identified concurrent coronary vasospasm and mesenteric ischemia in a patient with carcinoid disease.

CD5 negative mantle cell lymphoma: a different clinical entity?

We report herein a 77-year-old patient with CD5 negative mantle cell lymphoma (MCL). We further review the existing literature on clinicolaboratory features of this rare MCL subtype. Although most of the patients in the literature (including ours) had advanced stage at diagnosis, splenomegaly, and bone marrow involvement, they displayed prompt and durable responses to conventional treatment. We postulate that CD5 surface antigen expression could have prognostic implications in MCL. Further research and a larger number of patients are necessary in order to validate these findings.

An unusual presentation of Kikuchi-Fujimoto disease.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limited disease of unknown etiology. This condition is most commonly encountered in Asian and Caucasian females of childbearing age who usually present with cervical lymphadenopathy and fever. Though rarely, KFD has been described in younger African-American females as well. It often mimics more serious conditions such as lymphoma, metastatic solid malignancy, HIV infection, tuberculosis, sarcoidosis, or systemic lupus erythematosus. Although its etiopathogenesis has not been fully elucidated, literature suggests viral or possibly autoimmune components to play a role. We describe a 34-year-old African-American female who presented with constitutional symptoms and polyadenopathy on clinical examination and imaging, of which the portacaval and portahepatis lymph nodes were most prominent. An extensive workup was otherwise unremarkable, and biopsy showed histiocytic necrotizing lymphadenitis. Initially, her clinical condition improved spontaneously, and she required only a short course of oral steroids. Three months later, she relapsed with bilateral cervical adenopathy and constitutional symptoms and was successfully managed again with steroids. Our case is unique with respect to (a) portahepatis and portacaval node enlargement as the dominant adenopathy and (b) her underlying conditions of fibromyalgia and chronic fatigue syndrome.