scholarly journals Progressive Heart Failure and Death as the Initial Manifestation of NK/T-Cell Lymphoma: A Case Report and Literature Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Ziyu Zhang ◽  
Shuai Wang ◽  
Qingchun Liang ◽  
Daoquan Peng
Keyword(s):  
Heart Failure ◽  
T Cell ◽  
Cell Lymphoma ◽  
Cytotoxic T Cells ◽  
Late Enhancement ◽  
T Cell Lymphoma ◽  
Rare Type ◽  
Initial Manifestation ◽  
Non Hodgkin Lymphoma ◽  
Nk T Cell

Natural killer/T-cell (NK/T-cell) lymphoma is a rare-type non-Hodgkin lymphoma derived from NK cells or cytotoxic T cells. Here, we present a case of a 40-year-old woman who experienced quick-developed global heart failure and then was diagnosed with NK/T-cell lymphoma through lymphoid biopsy. Neither transthoracic echocardiography nor any radiological images detected a mass in her heart or pericardium. Elevated plasma troponin level and diffused patchy areas of gadolinium late enhancement on cardiac magnetic resonance were compatible with myocarditis. Considering the persistently elevated cytokine level, systemic inflammation symptoms, acute respiratory distress syndrome, and cardiac dysfunction, a cytokine storm secondary to NK/T-cell lymphoma was considered. Due to the refractory malignant arrhythmia, the patient died soon after being admitted to our hospital.

scholarly journals The Pathologic and Genetic Characteristics of Extranodal NK/T-Cell Lymphoma

Life ◽  
2022 ◽  
Vol 12 (1) ◽  
pp. 73
Author(s):  
Hyunsung Kim ◽  
Young Hyeh Ko
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Cytotoxic T Cells ◽  
Treatment Strategies ◽  
T Cell Lymphoma ◽  
Cell Of Origin ◽  
Genetic Characteristics ◽  
Cytotoxic Molecules ◽  
Nk T Cell

Extranodal NK/T-cell lymphoma is a neoplasm of NK cells or cytotoxic T cells presenting in extranodal sites, most often in the nasal cavity. The typical immunophenotypes are cCD3+, sCD3−, CD4−, CD5−, CD8−, CD16−, and CD56+ with the expression of cytotoxic molecules. Tumor subsets express NK cell receptors, CD95/CD95L, CD30, MYC, and PDL1. Virtually all the tumor cells harbor the EBV genome, which plays a key role in lymphomagenesis as an epigenetic driver. EBV-encoded oncoproteins modulate the host-cell epigenetic machinery, reprogramming the viral and host epigenomes using host epigenetic modifiers. NGS analysis revealed the mutational landscape of ENKTL, predominantly involving the JAK–STAT pathway, epigenetic modifications, the RNA helicase family, the RAS/MAP kinase pathway, and tumor suppressors, which indicate an important role of these pathways and this group of genes in the lymphomagenesis of ENKTL. Recently, three molecular subtypes were proposed, the tumor-suppressor/immune-modulator (TSIM), MGA-BRDT (MB), and HDAC9-EP300-ARID1A (HEA) subtypes, and they are well-correlated with the cell of origin, EBV pattern, genomic alterations, and clinical outcomes. A future investigation into the function and interaction of discovered genes would be very helpful for better understanding the molecular pathogenesis of ENKTL and establishing better treatment strategies.

Primary Extranodal NK/T-Cell Lymphoma Presenting as Neurolymphomatosis Involving Multiple Cranial Nerves: A Case Report

2021 ◽  
pp. 1-8
Author(s):  
Sharmila Ghosh ◽  
Jacques Azzi ◽  
Amy M. Chan ◽  
Kambiz Nael ◽  
Anne S. Renteria ◽  
...  
Keyword(s):  
T Cell ◽  
English Language ◽  
Cell Lymphoma ◽  
Hematologic Malignancy ◽  
Cranial Nerves ◽  
Cauda Equina ◽  
Rare Condition ◽  
T Cell Lymphoma ◽  
Initial Manifestation ◽  
Nk T Cell

Neurolymphomatosis (NL) is a rare condition caused by the lymphomatous or leukemic infiltration of nerves and manifests as neuropathy. Most often, NL is associated with B-lineage non-Hodgkin lymphoma (NHL) and only infrequently occurs in conjunction with T- or NK-lineage NHL. Extranodal NK/T-cell lymphoma (ENKTL)-associated NL is exceedingly unusual, with only 9 cases described in the English language literature, in addition to our case. Diagnosis of NL is challenging, as the entity can mimic neuropathies of more common etiologies, and an adequate biopsy may be difficult to obtain. Timely diagnosis demands a high index of suspicion, especially for patients without a history of hematologic malignancy. We expand upon a unique case of NL exclusively involving cranial nerves and cauda equina nerve roots, as the initial manifestation of ENKTL, and contextualize our findings within the framework of previously reported NK/T-lineage NL cases.

scholarly journals Orbital Inflammatory Lesion as an Initial Manifestation of Systemic Nasal Type NK/T-Cell Lymphoma

2012 ◽  
Vol 52 (2) ◽  
pp. 137-139 ◽  
Author(s):  
Keiki Kawakami ◽  
Ryugo Ito ◽  
Yasutaka Tono ◽  
Akira Yazaki ◽  
Tetsuya Murata ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Inflammatory Lesion ◽  
T Cell Lymphoma ◽  
Initial Manifestation ◽  
Nasal Type ◽  
Nk T Cell

scholarly journals Inert CD30-positive extranodal NK/T cell lymphoma with large cell transformation: a case report

2020 ◽  
Author(s):  
Hui Wang ◽  
PengYi Yu ◽  
Qing Li
Keyword(s):  
T Cell ◽  
Cell Transformation ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Lymphoid Cells ◽  
T Cell Lymphoma ◽  
Epstein Barr Virus Infection ◽  
Rare Type ◽  
Large Cell Transformation ◽  
Nk T Cell

Abstract Background Extranodal natural killer (NK)/T-Cell Lymphoma is a rare type of cytotoxic lymphoma associated with Epstein-Barr virus infection, highly invasive and relatively resistant to chemotherapy. Inert CD30-positive extranodal NK/T cell lymphoma with large cell transformation is very rare. Case presentation A married 55-year-old male was admitted to hospital in February 2017, because of “diagnosis of malignant lymphoma for about 12 years with high fever and a left neck mass present for about 3 weeks". The patient was diagnosed to NK/T Cell Lymphoma as early as 2006, repeated relapses in June 2010 and April 2013. The patient was biopsied again in February 2017, lymph node, about 2.5×2×1.3cm.The section was off-white and tender. Microscopic examinations showed a diffuse proliferation of small and atypical lymphoid cells, admixed with large lymphoid cells. Immunohistochemically, the tumor cells exhibited positivity for CD30, CD3, CD43, CD2, CD56, TIA, negativity for CD4, CD8, AE1/AE3, CD20, ALK, EMA staining. In situ hybridization for EBER was strongly positive in the specimens.Conclusions Herein, we report a case of CD30-positive extranodal NK/T Cell Lymphoma with large cell transformation, nasal type, in indolent course over a period of 12 years. The accurate diagnosis of NK/T-cell lymphoma with CD30 expression and large cell transformation is very important. The disease usually has a poor prognosis related to several factors, but the indolent behavior of the present case is more unusual. A long-term follow-up is suggested to be performed to inspect the progression for this tumor.

Outcome Of Hematopoietic Cell Transplantation (HCT) In Pediatric Patients With Non-Hodgkin Lymphoma (NHL): Single Institution Results From Saudi Arabia

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5522-5522
Author(s):  
Asim F Belgaumi ◽  
Hassan A Sumaili ◽  
Amani A Al-Kofide ◽  
Mouhab Ayas ◽  
Hassan El-Solh ◽  
...  
Keyword(s):  
T Cell ◽  
Hodgkin Lymphoma ◽  
Burkitt Lymphoma ◽  
Pediatric Patients ◽  
Cell Lymphoma ◽  
Lymphoblastic Lymphoma ◽  
T Cell Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Line Therapy ◽  
Nk T Cell

Abstract Although HCT is an accepted component of the treatment strategy for relapsed/refractory pediatric NHL, only few studies have reported on the outcome for these patients. Most have reported on small numbers of patients, with survivals ranging from 27% to 75%. Clinical data were retrospectively retrieved for patients with NHL who had undergone HCT. Pre-HCT information, including pathologic diagnosis, response to first- and second-line therapy and pre-HCT disease status were collected, in addition to details of the transplant process and patient and disease outcome. Between 1996 and 2012, 28 pediatric patients with NHL underwent HCT. Primary diagnosis for these patients included Burkitt lymphoma (n=13), Large B-cell lymphoma (n=4), T-Lymphoblastic lymphoma (n=4), NK/T cell lymphoma/leukemia (n=3), Peripheral T-cell lymphoma (n=2), B-lymphoblastic lymphoma ((n=1) and anaplastic large cell lymphoma (n=1). The median age at HCT was 7.65 years (mean 8.2; range 1-14.3). Twenty had suffered a relapse of their disease, while five had primary progression; three patients with NK/T lymphomas underwent HCT as part of their first-line therapy. Fourteen patients had autologous (autoHCT) and 14 had allogeneic HCT (alloHCT). Among alloHCT, 11 had matched-related grafts while 3 had unrelated umbilical cord blood (UCB) grafts. At the time of HCT, 23 patients were in CR (CR1=7, CR2=15, CR3=1), and 5 had partial responses. HCT conditioning was myeloablative for all patients; in 18 patients, it was TBI-based. Fourteen patients suffered recurrence of their lymphoma post HCT at a median of 1.17 months from HCT (mean 6.2; range 0.63-42); 4 died in CR due to transplant-related toxicity, of these 3 were post alloHCT and one post autoHCT. Three patients have developed secondary malignancies (SMN; 2 post alloHCT and 1 post autoHCT). 10 patients were alive at last follow-up, all of whom were in CR. The 5-year estimated OS from SCT is 38.7%, with and EFS of 26%. There was no difference in 5-year OS or EFS among patients who received alloHCT v. autoHCT (OS 28.6% v. 49%; p=0.53, EFS 14.3% v. 37.5%; p=0.25) and among patients who did or did not receive TBI (OS 33.3% v. 48%; p=0.37, EFS 27.8% v. 18.8%; p=0.66). OS/EFS for patients with Burkitt lymphoma was 23.1%. Of the three patients with NK/T cell lymphoma two remain alive in CR 13.7 and 5.1 years after HCT. The outcome of relapsed/refractory non-Hodgkin lymphoma of childhood remains suboptimal. In addition to a high post-HCT relapse rate of 50%, HCT-related toxic mortality and SMN contribute to the poor outcome for this cohort of patients. Disclosures: No relevant conflicts of interest to declare.

Long-term survival of the nasal NK/T cell lymphoma

2008 ◽  
Vol 149 (17) ◽  
pp. 801-805
Author(s):  
Péter Rajnics ◽  
László Krenács ◽  
András Kenéz ◽  
Zoltán Járay ◽  
Enikő Bagdi ◽  
...  
Keyword(s):  
T Cell ◽  
Treatment Guidelines ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Diagnostic Procedures ◽  
T Cell Lymphoma ◽  
Term Survival ◽  
Barr Virus ◽  
Significant Difference ◽  
Nk T Cell

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein–Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.

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