scholarly journals Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored

Children ◽  
2021 ◽  
Vol 8 (12) ◽  
pp. 1083
Author(s):  
Hye-ji Han ◽  
Kyung Taek Hong ◽  
Hyun Jin Park ◽  
Bo Kyung Kim ◽  
Hong Yul An ◽  
...  
Keyword(s):  
T Cell ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Large Cell ◽  
Hematologic Malignancies ◽  
Initial Presentation ◽  
Rapid Screening ◽  
Response To Treatment ◽  
Response Monitoring ◽  
Complete Control

It is complicated to establish a consensus on the management and diagnosis of malignancy-triggered hemophagocytic lymphohistiocytosis (M-HLH) in children, as an initial presentation of malignancy is complicated. In this paper, we analyze the clinical characteristics and outcomes of eight pediatric patients in which M-HLH was the initial presentation of malignancy. All patients had hematologic malignancies: three subcutaneous panniculitis-like T-cell lymphomas, two acute lymphoblastic leukemias, two anaplastic large cell lymphomas, and a systemic EBV + T-cell lymphoma of childhood. The incidence rate of M-HLH among leukemia and malignant lymphoma patients in our institution was 1.9%. From the initial diagnosis of HLH, the median time taken to be diagnosed as a malignancy was about 1.3 months. The majority of patients received HLH-targeted immunosuppression and/or etoposide at first. The patients’ clinical response to treatment for HLH and malignancies were varied. Five out of the eight patients died, one of whom died due to HLH-related cerebral edema after the initiation of chemotherapy. The median overall survival was 1.6 years. In order to improve the survival rate, the early detection of M-HLH, rapid screening for malignancy, and complete control of M-HLH with HLH-directed therapy followed by a thorough response monitoring are required.

scholarly journals Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis in Rheumatologic Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Yael Kusne ◽  
Michael Christiansen ◽  
Christopher Conley ◽  
Juan Gea-Banacloche ◽  
Ayan Sen
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Histoplasma Capsulatum ◽  
Hematologic Malignancies ◽  
Rapid Diagnosis ◽  
Rheumatologic Disease ◽  
Disseminated Histoplasmosis ◽  
Serious Infections ◽  
Time To Diagnosis ◽  
Prompt Diagnosis

Background. Hemophagocytic lymphohistiocytosis (HLH) was originally described in pediatric patients presenting with fever, hepatosplenomegaly, and blood cell abnormalities. Later, HLH was recognized to occur in adults, often associated with hematologic malignancies or serious infections. Conclusion. Patients presenting with HLH are critically ill, and rapid diagnosis is key. In adults, the search for the trigger must begin promptly as time to diagnosis effects survival. The underlying trigger in our patients was Histoplasma capsulatum infection, which is rare in the southwestern United States. Prompt diagnosis led to recovery in one patient, while the other did not survive.

scholarly journals Hemophagocytic Lymphohistiocytosis in Association with Primary Cutaneous Anaplastic Large Cell Lymphoma

2014 ◽  
Vol 2014 ◽  
pp. 1-7
Author(s):  
Aneesh Basheer ◽  
Somanath Padhi ◽  
Ramesh Nagarajan ◽  
Vinoth Boopathy ◽  
Sudhagar Mookkappan ◽  
...  
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Lymphoproliferative Disorders ◽  
Elderly Male ◽  
Anaplastic Lymphoma ◽  
T Cell Lymphomas ◽  
Large Cell Lymphoma

Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)−) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors.

Hemophagocytic Lymphohistiocytosis in Adults: A Case Series of 30 Patients At a U.S. Tertiary Care Center

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4681-4681
Author(s):  
Sarah A. Buckley ◽  
Daniel Egan ◽  
Roland B. Walter
Keyword(s):  
T Cell ◽  
Hospital Mortality ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Hematologic Malignancies ◽  
T Cell Lymphoma ◽  
Stevens Johnson Syndrome ◽  
Nk T Cell

Abstract Abstract 4681 Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by overwhelming immune activation that results in excessive inflammation, phagocytosis of blood cells by macrophages, and end-organ damage. Both familial forms (“primary HLH”), often presenting in childhood, and sporadic forms (“secondary HLH”), often presenting in adults, have been recognized. While pediatric HLH is well characterized, only limited data is available on the diagnosis and treatment of HLH in adults. This study, the largest single center case series of adult HLH to date, characterizes cases treated at the University of Washington since 2000 to further our understanding of the clinical characteristics and treatment outcomes of HLH in the adult population. Patients and Methods: Potential cases of HLH were identified by searching hospital billing records for the appropriate ICD-9 code (288.4). Information available from the electronic medical records was used for disease confirmation according to the HLH-2004 criteria, and to obtain relevant information on demographics as well as patient- and disease-related characteristics (including laboratory data, treatment, and outcome. Results: Among the 30 cases who met HLH-2004 diagnostic criteria, the median age was 42 (range: 19 – 76) years. The most common signs and symptoms were fever (100%), splenomegaly (80%), hepatomegaly (40%), lymphadenopathy (40%), and rash (37%). The most common laboratory abnormalities during hospitalization were hyperferritinemia (100%), hepatitis (100%), pancytopenia (93%), elevated creatinine (73%), elevated triglycerides (60%), and low fibrinogen (57%). The presumed causes of HLH were: 30% viral (EBV [n=9], HSV [n=1]), 23% malignancies (B-cell lymphoma [n=4], NK/T-cell lymphoma [n=2], acute lymphoblastic leukemia [n=1]), 20% rheumatologic (Still's disease [n=2], lupus-like syndrome [n=2], Stevens-Johnson syndrome [n=1], dermatomyositis [n=1]), 17% EBV-driven lymphoma (Hodgkin's disease [n=2], NK/T-cell lymphoma [n=1], post-transplant lymphoproliferative disorder-like syndrome [n=1]), 3% bacterial sepsis, and 6% idiopathic. Genetic testing was performed in only 4 patients, without identification of mutations. For treatment, corticosteroids were frequently used; fewer than half the patients received cyclosporine, etoposide, intravenous immunoglobulins, rituximab, interleukin-1 receptor antagonist, or intrathecal therapy. Two patients were retreated after relapse, and one received an allogeneic hematopoietic cell transplant. Two patients received no treatment: one died before therapy was initiated; in the other, treatment was withheld due to pregnancy, and symptoms resolved post-partum. The in-hospital mortality rate was 50%, with most patients dying of multi-organ failure. In univariate analysis, factors associated with in-hospital mortality were older age (49 vs 35 years; p=0.025), lower hemoglobin nadir (6.4 vs 7.3 g/dL; p=0.020), lower triglycerides (284 vs 447 mg/dL; p=0.027), and higher total bilirubin (15.0 vs 6.4 mg/dL; p=0.019); gender, ethnicity, number of co-morbid conditions, symptoms, latency to treatment, and treatment used were not significantly associated. The best outcomes were seen in patients with EBV-driven lymphoma (80% survival, n=5), while the worst outcomes were seen in patients with non-EBV-driven hematologic malignancies (29% survival, n= 7). Patients with EBV-driven disease were more likely to be treated with etoposide (75 vs 11%); those with malignancy were less likely to be treated with cyclosporine (25 vs 65%). Conclusion: A high index of suspicion for HLH should be maintained for adults who present with fever, splenomegaly, cytopenias, and hepatitis. HLH is associated with high mortality, particularly when occurring in older patients, those with anemia and hyperbilirubinemia, and those with non-EBV-driven hematologic malignancies. Further studies from this and other cohorts are needed to develop and refine prognostic criteria for outcomes in adult HLH. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Nelarabine in the Treatment of Refractory T-Cell Malignancies

2010 ◽  
Vol 4 ◽  
pp. CMO.S4364 ◽  
Author(s):  
Andrew M. Roecker ◽  
Amy Stockert ◽  
David F. Kisor
Keyword(s):  
T Cell ◽  
Pediatric Patients ◽  
Lymphoblastic Leukemia ◽  
Hematologic Malignancies ◽  
Lymphoblastic Lymphoma ◽  
Appropriate Use ◽  
Fda Approval ◽  
Cell Acute Lymphoblastic Leukemia ◽  
T Cell Malignancies ◽  
Dose Dependent

Nelarabine is a nucleoside analog indicated for the treatment of adult and pediatric patients with T-cell acute lymphoblastic leukemia (T-ALL) or T-cell lymphoblastic lymphoma (T-LBL) that is refractory or has relapsed after treatment with at least two chemotherapy regimens. After being first synthesized in the late 1970s and receiving FDA approval in 2005, the appropriate use of nelarabine for refractory hematologic malignancies is still being elucidated. Nelarabine is the prodrug of 9-β-D-arabinofuranosylguanine (ara-G) which when phosphorylated intracellularly to ara-G triphosphate (ara-GTP), preferentially accumulates in cancerous T-cells. Dose-dependent toxicities, including neurotoxicity and myelosuppression, have been documented and may, in turn, limit the ability to appropriately treat the diagnosed malignancy. This article will summarize the pharmacologic properties of nelarabine and will address the current place in therapy nelarabine holds based upon the results of the available clinical trials to date.

scholarly journals Perils and Pitfalls Regarding Differential Diagnosis and Treatment of Primary Cutaneous Anaplastic Large-Cell Lymphoma

2011 ◽  
Vol 11 ◽  
pp. 1048-1055 ◽  
Author(s):  
Michael D. Diamantidis ◽  
Athena D. Myrou
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Correct Diagnosis ◽  
Large Cell ◽  
Skin Lesions ◽  
Good Prognosis ◽  
Response To Treatment ◽  
Large Cell Lymphoma

Primary cutaneous anaplastic large-cell lymphoma (PC-ALCL), belonging to the CD30+ T-cell lymphoproliferative disorders (PCLPDs), is a rare T-cell lymphoma, presenting on the skin and characterized by very good prognosis and response to treatment in the majority of cases. Nevertheless, PC-ALCL must be distinguished from secondary skin lesions in systemic ALCL, which confer a poor prognosis, and other CD30+ PCLPDs, reactive conditions, or borderline cases. Given their rarity and heterogeneity, these entities represent diagnostic and therapeutic challenges, thus requiring a multidisciplinary approach and expertise to ensure appropriate diagnosis and management. There are several perils and pitfalls that exist regarding the differential diagnosis, the possible progression, and the treatment of PC-ALCL. Careful staging, correlation of clinical findings with histopathology and immunopathology, and thorough follow-up are essential in order to achieve a correct diagnosis and proper treatment of the disease.

scholarly journals a Phase I Trial of Bendamustine in Combination with Clofarabine and Etoposide in Pediatric Patients with Relapsed or Refractory Hematologic Malignancies

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1628-1628 ◽  
Author(s):  
Sima Jeha ◽  
Monika L. Metzger ◽  
Kristine R Crews ◽  
Patrick Campbell ◽  
Raul C. Ribeiro ◽  
...  
Keyword(s):  
T Cell ◽  
Inflammatory Response ◽  
Hodgkin Lymphoma ◽  
Dose Level ◽  
Pediatric Patients ◽  
Hematologic Malignancies ◽  
Systemic Inflammatory Response ◽  
Cross Resistance ◽  
Combination Regimen ◽  
Platelet Recovery

Abstract Introduction: Bendamustine is an alkylator with anti-metabolite properties that shows incomplete cross resistance with other alkylators such as cyclophosphamide. The combination of cyclophosphamide, clofarabine and etoposide is often used in the treatment of children with relapsed leukemia, most of whom have significant prior exposure to cyclophosphamide. We evaluated the maximum tolerated dose (MTD) and safety profile of bendamustine when used in combination with clofarabine and etoposide in pediatric patients with relapsed or refractory hematologic malignancies. Methods: Patients are eligible if they are younger than 22 years-old, have relapsed or refractory hematologic malignancies following 2 or more prior regimens, and have adequate organ function. Using the rolling 6 design, participants received bendamustine at one of 3 dose levels (escalating doses of 30, 40, or 60mg/m2/day) on Days 1-5 in combination with clofarabine (40 mg/m2/day), etoposide (100 mg/m2/day), and dexamethasone (8 mg/m2/day) daily on Days 1-5. We obtained pharmacokinetic (PK) studies to assess for potential time-dependent changes in bendamustine clearance over the 5 day course in this combination regimen since most PK studies of bendamustine have been conducted in adult patients with dose schedules of 90-120 mg/m2/day for 2 days. Results: Sixteen patients (12 males and 4 females) with median age 11 years (range 4 to 17 years) were enrolled: 10 B-cell acute lymphoblastic leukemia (B-ALL), 1 early T-cell precursor (ETP) leukemia, 1 gamma delta T-cell ALL, 2 Hodgkin lymphoma, and 2 T-cell non-Hodgkin lymphoma. Six patients were treated on dose level 1, six on dose level 2, and four on dose level 3. One patient with hyperleukocytosis died from severe systemic inflammatory response syndrome (SIRS). Dose limiting toxicity was failure to recover peripheral blood counts on Day 42. The recommended dose of bendamustine in this combination is 30mg/m2 daily over 5 days. Ten responses were observed: 6 complete remissions (CR), 1 durable minimal residual disease (MRD)-negative CR without platelet recovery in the patient with ETP-ALL, and 3 partial remissions. Eight patients proceeded to transplant. Nine patients died (5 from progressive disease, 2 from transplant complications, 1 from SIRS and 1 from complications of subsequent salvage chemotherapy). Six patients are alive at a median follow up of 12 months (range 2 to 29 months). Conclusions: Bendamustine is well tolerated in combination with clofarabine and etoposide and shows efficacy in multiple relapsed and refractory hematologic malignancies. Dose reductions on the first day of therapy are warranted in patients at risk of tumor lysis syndrome to avoid severe systemic inflammatory response. Disclosures Bhojwani: Amgen: Other: Blinatumumab global pediatric advisory board 2015.

scholarly journals "Real-World" Peripheral T-Cell Lymphomas Experience in Portugal: Is There Reason for Optimism?

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5287-5287
Author(s):  
Ivan Dlouhy ◽  
Rita Gavancha ◽  
Inês Coelho ◽  
Catalina Gomez ◽  
Inês Barbosa ◽  
...  
Keyword(s):  
T Cell ◽  
Single Agent ◽  
Treatment Strategies ◽  
Large Cell ◽  
New Drugs ◽  
Response To Treatment ◽  
Hematopoietic Stem ◽  
Histologic Subtype ◽  
T Cell Lymphomas ◽  
Peripheral T Cell Lymphomas

Introduction: Peripheral T-cell lymphomas (PTCL) are a heterogeneous group of mature T-cell neoplasms with aggressive behavior and dismal outcomes. Anthracycline-based chemotherapy is commonly used upfront; hematopoietic stem-cell transplantation (HSCT) is employed as consolidation by some groups. Refractory/relapsed cases (R/R) have a median survival of less than 6 months. Our objective was to analyze the outcomes of all consecutive PTCLs diagnosed and treated in a single center during 16 years according to histological subtype, disease phase (first line and R/R) and treatment strategies. Patients and methods: All adult PTCL patients referred to our center between 2003 and 2019 were included. All cases were locally diagnosed based on the current WHO classification by an expert hemato-pathologist. Initial clinical features, treatment and outcomes were analyzed, as well as salvage strategies. Results: A total of 188 patients were included (118 male, 70 female; median age 62 years). Median OS was 22.7 months, with a median follow up of 54 months. Histological subtypes, initial features, response to treatment and survival are detailed in table 1. Briefly, most cases were diagnosed at advanced stages, with extranodal involvement in 69% and elevated beta-2 microglobulin (B2m) in 77%; half had B symptoms. OS according to different subtypes is shown in figure 1. Of note, anaplastic large cell lymphoma (ALCL)-ALK+ patients had a 5-year OS of 88%. Interestingly, fifteen patients had circulating lymphoma cells (12 PTCL not otherwise specified [NOS], 2 hepatosplenic lymphoma and 1 angioimmunoblastic lymphoma [AITL]), with no impact on outcome. PTCL-NOS was the most prevalent subtype (40%) followed by AITL; unexpectedly, the proportion of PTCL-NOS cases increased while AITL cases decreased after 2008. Seven cases belonging to the recently recognized nodal PTCL with T follicular helper (TFH) phenotype were observed, with baseline characteristics and outcome similar to other PTCLs. IPI score index stratified patients into 4 groups with 24-month OS of 71%, 55%, 42% and 16% for low, int-low, int-high and high risk patients, respectively (P=.049). Only B2m and IPI score maintained independent significance for OS (HR= 3.2 and 1.8, respectively, P<.01) in a multivariate analysis that also included histologic subtype and frontline treatment. Most patients were treated with CHOP (75%), although other regimens were increasingly used in recent years, including CHOEP (9%). Young (<65 years) PTCL-NOS patients had a better outcome when treated with CHOEP compared to CHOP (24-month OS of 100% vs. 37%, P=.04); this difference was not noted in ALCL-ALK- and in AITL cases. Twenty-three patients (21% of transplant-eligible cases) underwent HSCT (18 autologous, 5 allogeneic), mostly (16/23) in first remission. As expected, patients not responding to frontline therapy or relapsing after CR showed a dismal outcome (median OS of 4.1 months from R/R date). Intensive platinum-based salvage treatments (26 cases) led to a median OS from relapse of only 6 months. In contrast, single-drug Gemcitabine at first relapse showed a median OS 17.4 months in 5 elderly patients. Of 76 R/R transplant eligible patients, only 7 underwent HSCT(4 allogeneic, 3 autologous), with a 5-year OS of 86%. Eight patients (4 AITL, 4 ALCL) received Brentuximab-Vedotin (BV) at first or later relapse, with improved outcomes compared to other regimes (24-month OS of 63% vs. 22%, P=.03). Conclusion: Initial features, treatments and outcomes for PTCL have not significantly changed in a 16-year period. Only a minority of cases underwent HSCT or received new agents. Ideal salvage regimens are not defined and, in our experience, single agent Gemcitabine or BV performed better than more aggressive combinations. Although promising new drugs have been recently approved for PTCL, their impact on outcome is still not clear. Recent progresses in molecular characterization of the disease may translate into better outcomes through prospective collaborative efforts in the near future. Disclosures Silva: Gilead Sciences: Consultancy, Other: Travel support, Research Funding; Janssen Cilag: Consultancy, Other: Travel support; Abbvie: Consultancy, Other: Travel support; Celgene: Consultancy; Roche: Consultancy, Other: Travel support.

scholarly journals CRISPR/Cas9 Generation of Npm1-Alk in Transplantable Murine Hematopoietic Stem Cells Accurately Models ALK-Positive Lymphoma in Recipients

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 779-779
Author(s):  
Soumya Sundara Rajan ◽  
Lingxiao Li ◽  
Kranthi Kunkalla ◽  
Amit Dipak Amin ◽  
Nitin Agarwal ◽  
...  
Keyword(s):  
Stem Cells ◽  
T Cell ◽  
Hematopoietic Stem Cells ◽  
Cell Lymphoma ◽  
Chromosomal Rearrangements ◽  
Large Cell ◽  
Hematologic Malignancies ◽  
Wild Type ◽  
Hematopoietic Stem ◽  
Large Cell Lymphoma

Abstract Chromosomal rearrangements resulting in generation of novel fusion oncogenes are common in hematologic malignancies. These disease drivers are key therapeutic targets and form the basis of animal model development for preclinical studies. For example, retroviral introduction of the chronic myeloid leukemia (CML) fusion BCR-ABL to hematopoietic stem cells (HSCs) results in a myeloproliferative disease similar to accelerated-phase human CML when transplanted to recipient mice. This model and many others are established traditionally through retroviral or germline introduction of human fusion oncogenes to the murine genome. Recent advances in CRISPR/Cas9 technology now permit direct editing of the murine genome to create endogenous genotypes that more accurately reflect configurations found in human diseases. To date, these techniques have not been successfully applied to the modeling of fusion oncogene-driven hematologic malignancies. Anaplastic Large Cell lymphoma (ALCL) is a T-cell non-Hodgkin lymphoma common in adolescents and young adults and driven in ~70% of cases by chromosomal rearrangements involving Anaplastic Lymphoma Kinase (ALK). Most commonly, t(2:5; p23:q35) fuses the 3' ALK kinase domain to the 5' oligomerization domain of the constitutively expressed Nucleophosmin1 (NPM1) gene. An existing immunocompetent model of ALK+ lymphoma employs a CD4 promoter-driven NPM1-ALK transgene but results in immature T-lymphomas in about two-thirds and B-lineage plasma-cell lymphomas in the rest of animals. We employed CRISPR/Cas9 vectors containing guide strands designed to generate double-stranded DNA cleavage in mouse chromosomes 11 and 17 at breakpoints predicted to generate an in-frame Npm1-Alk fusion oncogene. Wild-type HSCs derived from fetal livers were divided and subjected to either transient CRISPR or mock transfection during a brief ex vivo passage followed by immediate transplantation to sub-lethally irradiated wild-type recipients. Mice initially transplanted with CRISPR-modified HSCs developed an ALK+ large cell lymphoma with a latency of ~9 months, while mock transfected controls sacrificed in parallel were phenotypically normal. qPCR analysis of lymphoid organs from mice that developed disease showed extremely high expression of Alk and Tnfrsf8, which encodes CD30. Pathologically, tumors contained large malignancy T cells with anaplastic morphology. Immunohistochemistry confirmed ALK protein expression, including nuclear localization classic for NPM1-ALK, and intense CD30 cell-surface staining. One recipient instead developed a CD30-negative ALK+ diffuse large B-cell lymphoma. Transplantation of primary T-lymphoma cells to secondary recipients resulted also in ALK+ T-cell lymphomas in recipients with more rapid onset infiltrating lymph nodes, spleen, liver, and other organs. T-cell receptor clonality analyses through β-chain deep sequencing show an oligoclonal T-cell disease in both primary and secondary recipients. We therefore demonstrate successful genomic editing of transplantable murine hematopoietic stem cells to generate a novel model of a fusion oncogene-driven hematologic malignancy. These methods are widely applicable to additional lymphomas and leukemias and could fuel development of improved in vivo preclinical model systems. Disclosures No relevant conflicts of interest to declare.

scholarly journals Primary Lymphoma of the Liver: A Case Report and Review

2000 ◽  
Vol 14 (11) ◽  
pp. 955-957 ◽  
Author(s):  
Franzjosef Schweiger ◽  
Rowen Shinder ◽  
Sheldon Rubin
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
T Cell ◽  
Carcinoembryonic Antigen ◽  
Bone Marrow Involvement ◽  
Large Cell ◽  
Response To Treatment ◽  
Primary Lymphoma ◽  
Cell Type ◽  
Rare Tumours

The case of a previously healthy man who developed primary non-Hodgkin’s lymphoma of the liver is presented. Biopsy confirmed that the tumour was of the diffuse large cell type and was of apparent T-cell origin. The diagnosis of these rare tumours is suggested by the presence of a hepatic mass without lymphadenopathy, splenomegaly or bone marrow involvement, as well as normal carcinoembryonic antigen and alpha-fetoprotein levels. However, histological examination of tissue is essential to confirm the diagnosis. The response to treatment varies, but surgical resection and/or chemotherapy can result in prolonged remissions. The literature on this topic is briefly reviewed.

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