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Follicular Lymphoma Presenting With Monoclonal IgM And MYD88 Mutation: A Case Report And Review Of The Literature.

ABSTRACT: MYD88 mutation has been reported in various lymphomas, specifically in lymphoplasmacytic lymphoma. Yet, the mutation has not been reported in primary follicular lymphoma. Here, we present a 62-year-old male with follicular lymphoma who had an MYD88 L265P somatic mutation and monoclonal IgM gammopathy. He received four cycles of R-CHOP immunochemotherapy. Interim PET/CT evaluation indicated a state of stable disease (SD). Neither did serum IgM remarkably drop. He was then given a bortezomib-contained regimen which significantly reduced the level of serum IgM. To the best of our knowledge, this is the first report of follicular lymphoma with monoclonal IgM and MYD88 L265P mutation. The present case indicated bortezomib may benefit these patients.

SUBMITTER: Xu L

PROVIDER: S-EPMC6767481 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Follicular Lymphoma Presenting With Monoclonal IgM And MYD88 Mutation: A Case Report And Review Of The Literature.

Xu Linglong L Ding Xiaoxiao X Ying Limei L Zhang Xiaochang X Lu Nina N

OncoTargets and therapy 20190923

MYD88 mutation has been reported in various lymphomas, specifically in lymphoplasmacytic lymphoma. Yet, the mutation has not been reported in primary follicular lymphoma. Here, we present a 62-year-old male with follicular lymphoma who had an MYD88 L265P somatic mutation and monoclonal IgM gammopathy. He received four cycles of R-CHOP immunochemotherapy. Interim PET/CT evaluation indicated a state of stable disease (SD). Neither did serum IgM remarkably drop. He was then given a bortezomib-conta ...[more]

PMID: 31576141

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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.

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.

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Follicular Lymphoma Presenting With Monoclonal IgM And MYD88 Mutation: A Case Report And Review Of The Literature.

Publications

Follicular Lymphoma Presenting With Monoclonal IgM And MYD88 Mutation: A Case Report And Review Of The Literature.

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