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Plasmablastic lymphoma in the ano-rectal junction presenting in an immunocompetent man: a case report.

ABSTRACT: INTRODUCTION: Plasmablastic lymphoma is an aggressive non-Hodgkin lymphoma classically occurring in individuals infected with HIV. Plasmablastic lymphoma has a predilection for the oral cavity and jaw. However, recent case reports have shown lymphoma in the stomach, lung, nasal cavity, cervical lymph nodes and jejunum in HIV-negative individuals. We report what is, to the best of our knowledge, the first case of plasmablastic lymphoma occurring in the ano-rectal junction of an HIV-negative man. CASE PRESENTATION: A previously healthy 59-year-old Caucasian man presented with painless rectal bleeding. Colonoscopy revealed a lesion in the ano-rectal junction, with pathological examination demonstrating atypical lymphoid cells consisting primarily of plasmablasts with rounded nuclei, coarse chromatin, small nucleoli and multiple mitotic figures. Immunohistochemical analysis showed the atypical cells were negative for CD45, CD20, CD79a and immunoglobulin light chains, but were strongly positive for CD138 and EBV-encoded RNA. The results were consistent with a diagnosis of plasmablastic lymphoma. Aggressive systemic chemotherapy and involved field radiation therapy resulted in complete clinical and pathological remission. CONCLUSION: Increasing awareness of plasmablastic lymphoma in HIV-negative individuals and in this location is warranted.

SUBMITTER: Brahmania M

PROVIDER: S-EPMC3107799 | biostudies-other | 2011

REPOSITORIES: biostudies-other

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Plasmablastic lymphoma in the ano-rectal junction presenting in an immunocompetent man: a case report.

Brahmania Mayur M Sylwesterowic Thomas T Leitch Heather H

Journal of medical case reports 20110503

<h4>Introduction</h4>Plasmablastic lymphoma is an aggressive non-Hodgkin lymphoma classically occurring in individuals infected with HIV. Plasmablastic lymphoma has a predilection for the oral cavity and jaw. However, recent case reports have shown lymphoma in the stomach, lung, nasal cavity, cervical lymph nodes and jejunum in HIV-negative individuals. We report what is, to the best of our knowledge, the first case of plasmablastic lymphoma occurring in the ano-rectal junction of an HIV-negativ ...[more]

PMID: 21539737

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Project description:IntroductionParaneoplastic pruritus is defined as pruritus that occurs before or during the natural evolution of a hematologic disease. The reported prevalence is 30% in patients with Hodgkin's lymphoma. The severity of this pruritus has a very negative impact on patients' quality of life. Very few studies have been made to examine the efficacy of pharmacological treatments for this type of pruritus. One drug that appears to be effective in this respect is off-label aprepitant, a neurokinin 1 receptor antagonist.Case presentationA 20-year-old Caucasian woman presented with lateral neck nodes, sweating, and pruritus and was diagnosed with stage IIB nodular sclerosis Hodgkin's lymphoma. Throughout this period during the disease the pruritus was ever-present. Improvement was achieved with some of the chemotherapy treatments, but the symptom returned when the various treatments were withdrawn due to disease progression or poor tolerance. In the middle of the seventh year, she was admitted to our hospital with uncontrolled pruritus that resulted in severe lesions due to scratching. In response, aprepitant (off-label) 80 mg/day was added to the chemotherapic treatment of the pruritus, after studying the various treatment options. She reported a score of 9 on a visual analogue scale for the pruritus, and a score of 3 on the Eastern Cooperative Oncology Group performance status scale of performance status. After two weeks of treatment with aprepitant, she reported a score of 5 on the visual analogue scale for the pruritus, and this improved to a score of 4 in a month, which allowed her to lead a better quality of life, with an Eastern Cooperative Oncology Group performance status score between 1 and 2.ConclusionsSeveral cases and case series have been reported on the use of aprepitant for paraneoplastic pruritus, but none have referred to its use for Hodgkin's lymphoma. A prospective study was carried out to evaluate the efficacy of this drug in refractory pruritus secondary to Sezary syndrome, and other authors have studied the effectiveness of aprepitant against pruritus, secondary to biological therapy with erlotinib. In our case report, treatment was started with daily doses of aprepitant 80 mg. Pruritus improvement appeared to be attributable exclusively to the administration of aprepitant.

Project description:Acute abdominal syndrome can be caused by several possible reasons. The most common causes are perforation of a gastroduodenal ulcer, peritonitis, intestinal obstructions, and perforation of an appendix or fallopian tube. Fever and pain can be caused by an appendicitis or sigmoiditis. Appendiceal lymphoma is a rare disease that is usually found incidentally during appendectomy. Most of the cases are non-Hodgkin's lymphomas. Mantle cell lymphoma is an aggressive B-cell non-Hodgkin's lymphoma with a poorer prognosis than other B-cell lymphomas; thus, a definitive diagnosis is essential. A 60-year-old man presented with right lower quadrant pain. He denied any nausea, vomiting or anorexia and was afebrile. The physical examination revealed right lower quadrant abdomen tenderness. The computed tomography scan revealed periappendiceal fatty stranding with a swollen appendix, approximately 2 cm in diameter and prominent paraaortic, portacaval and mesenteric lymph nodes. A diagnosis of acute appendicitis was made, and laparoscopic appendectomy was performed immediately. The subsequent pathological examination revealed severe congestion with lymphoid hyperplasia. The immunohistochemistry stains revealed positive staining for cluster of differentiation (CD) CD20, B-cell lymphoma-2 (Bcl-2), cyclin D1, SRY-box transcription factor-11 (SOX-11), immunoglobulin D (IgD) and immunoglobulin M (IgM) but negative staining for CD3, CD5, CD10 and CD23. 18F-FDG positron emission tomography showed peripheral lymph node involvement, while the bone marrow biopsy showed negative findings. Therefore, a diagnosis of mantle cell lymphoma, Ann Arbor stage IVA, was made. The patient received postoperative combination chemotherapy and remained in a stable condition over a 1-year follow-up period. We report an uncommon case that initially presented as acute appendicitis, for which a final diagnosis of mantle cell lymphoma was made. In comparison with other B-cell lymphomas, mantle cell lymphoma has a poorer prognosis, and positive immunochemical staining of cyclin D1 and SOX-11 is useful for differentiating mantle cell lymphoma from other appendiceal lymphomas and treating patients appropriately. Physicians and nursing staff should be also aware of the associated complications and management in these patients.

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Plasmablastic lymphoma in the ano-rectal junction presenting in an immunocompetent man: a case report.

Publications

Plasmablastic lymphoma in the ano-rectal junction presenting in an immunocompetent man: a case report.

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