Peripheral T-cell Lymphoma Presenting Research Articles

TCL-089: Clinicopathological Presentation of Peripheral T-Cell Lymphoma and Their Prognostic Factors: A Single Center Experience

<h3>Background</h3> Peripheral T-cell lymphomas (PTCLs) represent an uncommon and heterogeneous group of diseases. They comprise 10-15% of NHL, with epidemiological majority in South American and Asian populations. Historically these groups of malignancies have presented a poor prognosis, however, with the incorporation of brentuximab and autologous stem cell transplantation (ASCT) as consolidation strategy, the overall survival has improved. <h3>Objectives</h3> Describe the clinicopathological presentation of patients with PTLCs and review their prognosis features. <h3>Design</h3> Retrospective, observational study that included patients over 18 years with diagnosis of PTLCs, between January 2010 and December 2019. All of them were followed up at the HIBA. <h3>Results</h3> We included 43 patients with PTLCs diagnosis. The median age was 58 years (range 20-90) and 65% were male. The most common histopathological subtype was PTLC-NOS in 42% (n=18) followed by ALCL ALK- and AITL with 23% (n=10) and 14% (n=6) respectively. The 81% presented III/IV stage at the diagnosis and 58% had evidence of extranodal disease. Considering IPI and PIT scores, high risk populations slightly differ from 53% in the first score to 44% in the other. Nearly 80% of the patients received protocols including anthracyclines such as CHOEP in 44% (n=19) or CHOP in 32% (n=14) as first line treatment. Another 7% used brentuximab + CHP and 41% (n=18) of the total underwent ASCT as consolidation therapy. Almost 58% achieved complete remission. The overall survival (OS) at 5 years was 37% (IC 95% 20-56) with a median of 36 months. Variables significantly associated with OS were age range (48% vs 26% in under 60 years; p=0,12) and IPI score (65% <i>vs</i> 25% in low-risk groups; p=0.0044). Moreover, ASCT was associated with better OS and PFS with 61% and 58% respectively; <i>versus</i> 26% and 28% in the group without transplant (p=0,0064). <h3>Conclusions</h3> In our experience, PTLCs represent an ominous diagnosis with high mortality in older people and high-risk groups. ASCT showed improvement in OS. Nevertheless, the implementation of brentuximab is still being studied because of the few patients receiving this therapy at the moment.

Poster: TCL-089: Clinicopathological Presentation of Peripheral T-Cell Lymphoma and Their Prognostic Factors: A Single Center Experience

Poster: TCL-089: Clinicopathological Presentation of Peripheral T-Cell Lymphoma and Their Prognostic Factors: A Single Center Experience

Peripheral T-cell lymphoma presenting with pleural effusion.

Peripheral T-cell lymphoma presenting with pleural effusion.

2676 An Extranodal Presentation of Peripheral T-Cell Lymphoma, Not Otherwise Specified as Primary Gastric Lymphoma, With Coexistence of HTLV-1

INTRODUCTION: Peripheral T- Cell Lymphoma, not otherwise specified ( PTCL-NOS) is a subtype of T-cell lymphoma that typically presents as primary nodal lymphoma, however can rarely present as primary extranodal lymphomas with stomach being the most common extranodal site. Here we present a unique case of PTCL-NOS gastric lymphoma with coexistence of HTLV-1, which is usually associated with Adult T-cell lymphoma/leukemia. CASE DESCRIPTION/METHODS: A 58 year old man with known hypertension and a chief complaint of abdominal discomfort, fatigue, occasional black stools and unintentional 10 lb weight loss of 2 months duration was seen in clinic. Vital signs, physical exam were normal except for conjunctival pallor. Labs revealed Hb of 7 gm/dL and elevated LDH. CT scan of the abdomen revealed an 11 × 12 × 12.6 cm poorly defined inseperable mass, in the left upper abdomen (Figure 1). Upper endoscopy (EGD) with Endoscopic ultrasound (EUS) revealed a large, ulcerated, nearly circumferencial, fungating mass that appeared to erode through the cardia of the stomach. Biopsy and immunohistochemical (IHC) staining revealed pleomorphic lymphocyte cells positive for CD2, CD3, CD5, CD4(70%), CD7 (20%), CD8 (30%), CD43 and CD45 (20%) (Figure 2). KI-67 index was 80% suggestive of high grade PTCL-NOS, with necrosis (Figure 3). H. pylori and HTLV-1 antibody were positive. Pretreatment PET scan and bone marrow biopsy did not reveal additional site involvement. He was subsequently started on CHOEP regimen and triple therapy for H.Pylori. Repeat imaging after the 4th cycle revealed an interval decrease in the size of the stomach mass with no new lesions. DISCUSSION: Primary Gastric lymphomas account for 3-4% of all the stomach malignancies, mostly B-cell (85-90%) in origin followed by PTCL-NOS. Biopsy is obtained by EGD in most cases and EUS is being increasingly used to evaluate for locoregional spread. Early diagnosis and initiation of treatment with CHOP/CHOEP regimen is crucial for better outcomes. Pan imaging, FDG PET, bone marrow biopsy are critical for staging, tumour activity and for followup to check for response. Radiation, surgery, bone marrow transplantation are used as adjuvant to chemotheraphy in select patients. Coexistence of HTLV-1 in a PTCL-NOS patient make our case unique and this gives scope for further research to verify if there is any association exists between PTCL-NOS and HTLV-1.

Chronic demyelinating polyneuropathy associated with anti-ganglioside GQ1b antibodies in peripheral T-cell lymphoma

Malignant lymphoma can cause peripheral neuropathy through various mechanisms. We report the case of 67-year-old man with chronic motor and sensory axonal demyelinating polyneuropathy associated with anti-ganglioside GQ1b antibodies in serum and cerebrospinal fluid (CSF) as an initial presentation of peripheral T-cell lymphoma, not otherwise specified. The patient was treated with chemotherapy for T-cell lymphoma, and achieved complete remission. Motor function recovered completely and sensory function improved. Neurological improvement was concurrent with the disappearance of serum and CSF anti-ganglioside GQ1b antibodies. This is the first report of chronic demyelinating polyneuropathy associated with anti-ganglioside GQ1b antibodies in peripheral T-cell lymphoma.

Primary sino-orbital peripheral T-cell lymphoma presenting as unilateral periorbital swelling: a case report.

Primary sino-orbital peripheral T-cell lymphoma presenting as unilateral periorbital swelling: a case report.

Peripheral T-Cell Lymphoma Presenting as a Pancreatic Mass: Case Report and Review of Literature

Peripheral T-Cell Lymphoma Presenting as a Pancreatic Mass: Case Report and Review of Literature

Isolated hypoglossal nerve palsy as a presenting symptom of metastatic peripheral T-cell lymphoma -not otherwise specified (PTCL-NOS): a unique case & a review of the literature.

Extensive and significant technological advancements have enhanced the sensitivity and accuracy of the pathologic classification, diagnosis, and therapeutics of lymphoma. These advances have prompted a more comprehensive understanding of neoplastic behavior and have led to improvements in both treatment and prognosis. This paper presents a comprehensive review of lymphoma and features a case report of a unique presentation of peripheral T-cell lymphoma – not otherwise specified that presented with isolated hypoglossal nerve dysfunction.

Fatal peripheral T-cell lymphoma presenting as hypereosinophilia and spontaneous tumor lysis syndrome: A rare case report

Peripheral T-cell lymphomas (TCLs) are a heterogeneous group of aggressive, uncommon forms of T-cell or natural killer cell-derived non-Hodgkin's lymphomas. Acute tumor lysis syndrome (TLS) is a multiple metabolic derangement that occurs due to the treatment of malignancies or spontaneous tumor cell death. We report the case of a 56-year-old male patient who presented with marked eosinophilic leukocytosis, rash, and lymphadenopathy at the initial visit but finally manifested with TCL and spontaneous TLS. On the first visit, hemogram showed a marked eosinophilic leukocytosis and raised lactate dehydrogenase. The patient refused for admission; however, lymph node biopsy was done on day-care basis. After 3 days, the patient presented in the emergency room with shortness of breath, altered renal and liver parameters, and dyselectrolytemia. Based on histopathological with immunohistochemistry findings on lymph node biopsy and clinical and laboratory finding, the patient was diagnosed as TCL-not otherwise specified with TLS.

Peripheral T-Cell Lymphoma Presenting as an Auricle Mass

Peripheral T-Cell Lymphoma Presenting as an Auricle Mass

Peripheral T-cell Lymphoma Presenting with Chylothorax

말초형 T세포 ë¦¼í”„ì¢ ì€, ë¦¼í”„ì ˆì™¸ 증상이 ë¹„íŠ¹ì´ì ìœ¼ë¡œ ë‚˜íƒ€ë‚ ê²½ìš° 질병이 진행하기 ì „ê¹Œì§€ 진단이 ì–´ë ¤ìš¸ 수 있다. 본 증례는 T세포 ë¦¼í”„ì¢ ì— 의한 ë¦¼í”„ì ˆ ì¢ ëŒ€ì˜ ë¹ ë¥¸ 진행과 함께 드문 합병증인 ìœ ë¯¸í‰ìœ¼ë¡œ 발현한 환자이며 ë¹„íŠ¹ì • 말초 T세포 ë¦¼í”„ì¢ ìœ¼ë¡œ 확진된 1예로 호흡곤란을 ìœ ë°œí•˜ëŠ” ìœ ë¯¸í‰ì„ ì ì ˆížˆ ì¡°ì ˆí•˜ì—¬, 항암화학요법을 속히 ì‹œí–‰í•˜ê³ ë¶€ë¶„ë°˜ì‘ì„ 얻을 수 있었던 증례이다. 중심 단어: ìœ ë¯¸í‰; ë¦¼í”„ì¢ ; 흉수

An Unusual Suspect Causing Hypoxemic Respiratory Failure.

Introduction: Antisynthetase syndrome (ASS) is characterized by the presence of anti-Jo-1 antibodies in conjunction with clinical findings of fever, polymyositis-dermatomyositis, and interstitial lung disease (ILD). Inflammatory myopathies carry a high risk of malignancy, but this association is less well outlined in ASS. We present the case of a patient with ASS who developed non-Hodgkin’s lymphoma with acute hypoxemic respiratory failure. Case Presentation: A 44-year-old female with ASS presented with acute hypoxemic respiratory failure. She was empirically treated with broad-spectrum antibiotics for a health care–associated pneumonia; however, she failed to improve. Chest computed tomography revealed extensive bilateral ground glass opacities as well as extensive mediastinal and axillary lymphadenopathy. Infectious workup was negative. A surgical lung biopsy revealed peripheral T-cell lymphoma (PTCL). The patient was started on chemotherapy with complete resolution of hypoxemic respiratory failure. Conclusions: Malignancy is very rare in the setting of ASS; and our case illustrates the unique presentation of PTCL in ASS. In addition, lung involvement in PTCL is variable (incidence ranging from 8% to 20%); and in this case, bilateral multifocal consolidation was biopsied and proven to be PTCL involving the lungs. This case highlights the rare noninfectious conditions that can present as acute hypoxemic respiratory failure in the setting of ASS.

Anaplastic large cell lymphoma arises in thymocytes and requires transient TCR expression for thymic egress.

Anaplastic large cell lymphoma (ALCL) is a peripheral T-cell lymphoma presenting mostly in children and young adults. The natural progression of this disease is largely unknown as is the identity of its true cell of origin. Here we present a model of peripheral ALCL pathogenesis where the malignancy is initiated in early thymocytes, before T-cell receptor (TCR) β-rearrangement, which is bypassed in CD4/NPM–ALK transgenic mice following Notch1 expression. However, we find that a TCR is required for thymic egress and development of peripheral murine tumours, yet this TCR must be downregulated for T-cell lymphomagenesis. In keeping with this, clonal TCR rearrangements in human ALCL are predominantly in-frame, but often aberrant, with clonal TCRα but no comparable clonal TCRβ rearrangement, yielding events that would not normally be permissive for survival during thymic development. Children affected by ALCL may thus harbour thymic lymphoma-initiating cells capable of seeding relapse after chemotherapy.

Constrictive pericarditis as the first presentation of untreated Angioimmunoblastic Peripheral T-cell Lymphoma in a young patient

Primary and secondary cardiac lymphoma mainly involves pericardium as pericardial effusion or cardiac tamponade, and when it does so, already the disease is in an advanced stage with many other viscera already affected. 1 Lymphoma leading to cardiac constriction is very rare, and the manifestations of constriction are predominantly subclinical or insidious or autopsy diagnosed. Hence a symptomatic clinical presentation of this rare sequel, like in our case, needs high index of suspicion for an underlying malignant disease. 2 Our case is further distinct with regards to the subtype of lymphoma leading to the constriction, the age of presentation of the patient, the clinical course of the disease, and the distribution of the organs involved. Read more....

VI. FDG-PET as a biomarker in lymphoma: from qualitative to quantitative analysis.

VI. FDG-PET as a biomarker in lymphoma: from qualitative to quantitative analysis.

Peripheral T-cell lymphoma presenting as testicular mass; a diagnostic challenge

BackgroundT-cell lymphomas involve the testis infrequently, which deserve special attention because of the poor prognosis and the need to make an appropriate diagnosis, which could lead to a better therapeutic strategy.Case presentationA 40-year-old man presented with right testicular swelling for past three months. The swelling was painless, hard and rubbery. Testicular ultrasound showed diffuse increase in size of the testicle, with alteration in its echogenicity. The patient underwent orchidectomy, and based on histopathological and immunohistochemical tests, a peripheral T-cell lymphoma, not otherwise specified was diagnosed.ConclusionTesticular peripheral T-cell lymphomas are rare and aggressive cancers, with clinical differentials of seminoma and non-neoplastic conditions.

Peripheral T-cell lymphoma presenting as Pott′s spine

Peripheral T-cell lymphoma presenting as Pott′s spine

A case of peripheral T-cell lymphoma presenting with acute liver failure.

A 68-year-old woman was evaluated by her primary physician for swelling and pain in the right neck. Treatment with antibiotics failed to achieve any improvement. Two weeks later, she was hospitalized to the gastroenterology department because of liver dysfunction and pneumonia. Disseminated intravascular coagulation (DIC) was diagnosed, and protease inhibitor and steroid pulse therapy were started. She was transferred to our department for further evaluation the following day. Bone marrow examination revealed hemophagocytosis and infiltration of CD3-positive cells. Multiple masses were identified in the liver. Her prothrombin time was 35.7% of the standard value 17days from disease onset, despite improvement of DIC. She was diagnosed with acute liver failure based on the Japanese diagnostic criteria. Her general condition worsened quickly, which prevented use of chemotherapy, and she died after a total course of 19days. Autopsy revealed atypical lymphocytes in the liver. The diagnosis was peripheral T-cell lymphoma.

Peripheral T-cell lymphoma presenting as myelofibrosis with the expression of basic fibroblast growth factor

Myelofibrosis is often observed in chronic myeloproliferative disorders (CMPD), but non-Hodgkin's lymphoma with diffuse myelofibrosis is rare. We describe an elderly case with peripheral T-cell lymphoma-unspecified (PTCL) presenting as diffuse myelofibrosis. Bone marrow biopsy revealed infiltration of atypical lymphocytes and diffuse myelofibrosis without any increase in megakaryocytes. To discuss the pathogenesis of fibrosis, we examined cytokines relative to fibrosis using immunostaining. The expression of basic fibroblast growth factor (bFGF) was diffusely detected in the area of extracellular matrix of bone marrow. In addition, in situ hybridization revealed that infiltrating lymphoma cells expressed bFGF mRNA. Basic FGF, originally identified based on its mitogenicity for fibroblasts, has multiple potential, influencing neoangiogenesis, bone marrow fibrosis and the proliferation of tumor cells. Basic FGF might play an important role in the pathogenesis of myelofibrosis in the present case.

Peripheral T-cell lymphoma presenting as an ischemic stroke in a 23-year-old woman: a case report and review of the literature

IntroductionPeripheral T-cell lymphoma of the unspecified variant is a highly aggressive subtype of T-cell non-Hodgkin's lymphoma. This is the first reported case of this type of lymphoma presenting as an ischemic stroke in a woman.Case presentationA previously healthy 23-year-old woman presented with fever and hemiplegia. She was subsequently intubated after scoring 7 out of 15 at the Glasgow Coma Scale. Brain computed tomography scans of the patient depicted a massive sylvian infarction while an abdominal computed tomography scan revealed multiple enlarged abdominal lymph nodes and a retroperitoneal mass adjacent to the left psoas muscle. A diagnostic work up for inherited thrombophilia yielded negative results. Blood and cerebrospinal fluid cultures for infectious agents also gave negative results. A biopsy of the retroperitoneal mass guided by computed tomography was inconclusive. A biopsy of an enlarged inguinal lymph node of the patient, combined with an immunophenotypic analysis, revealed an unspecified variant of peripheral T-cell lymphoma. The patient underwent chemotherapy but developed multiple organ failure. She died 26 days after she was admitted to our intensive care unit.ConclusionPeripheral T-cell lymphoma of the unspecified variant is a highly aggressive subtype of peripheral T-cell lymphomas. The latter exhibit no consistent immunophenotypic, genetic, or clinical features. Clinicians should be aware of atypical clinical presentations of the above lymphomas such as ischemic stroke.