Presentation
He received radiotherapy and a year later presented tumor activity. He also presented infiltration to the bone marrow and underwent chemotherapy. [ncbi.nlm.nih.gov]
Only one case of nasal T cell lymphoma presenting as nasal septal perforation, oronasal fistula and a concomitant palatal ulcer has been described. We report this case of a perforating palatal ulcer as a rare presentation of nasal lymphoma. [ijdvl.com]
In this study, we present a rare case of ENKTL presenting as a breast mass in a 30-year-old Caucasian female in the United States. [cureus.com]
A few cases of NKTCL presenting primarily in the lymph nodes have also been described. [orpha.net]
Entire Body System
-
Difficulty Walking
Afterward, he presented paresthesia and paresis of the lower extremities, difficulty walking, loss of sphincter control, and seizures. [ncbi.nlm.nih.gov]
-
Painter
He works as a painter, with constant exposure to inhaled chemicals. The month prior to consultation, he was treated for acute sinusitis with amoxicillin for 10 days due to bilateral nasal obstruction and occasional epistaxis. [revistas.unal.edu.co]
-
Recurrent Sinusitis
This paper reports the case of a 31-year-old male patient, with one month of nasal obstruction, recurrent sinusitis, palatal ulceration and a necrotizing lesion. [revistas.unal.edu.co]
Respiratoric
-
Anosmia
Patient’s previous medical records disclosed h/o of nasal obstruction on and off and anosmia and histopathological diagnosis of previous biopsy was given as round cell tumor by a private lab and as tissue insufficient for reporting by TATA memorial hospital [ncbi.nlm.nih.gov]
Gastrointestinal
-
Rectal Bleeding
There was a history of weight loss without rectal bleeding. [npplweb.com]
-
Hematochezia
A 52-year-old female patient presented with hematochezia and fever. The endoscopic finding showed a gastric ulcer with a recent bleeding stigma. [e-ce.org]
Liver, Gall & Pancreas
-
Hepatic Mass
[…] adnexa, with variable rimming, tumor necrosis and angiodestruction Positive stains: CD3epison, TIA1; variable CD2, CD3, CD8, CD45RO, CD56 Negative stains: CD4, CD7, CD57, betaF1, TdT HIV associated Case report of 42 year old man with AIDS, parotid and hepatic [pathologyoutlines.com]
Jaw & Teeth
-
Periodontitis
[…] extranodal nasal NK/T-cell lymphoma case, involving the maxillary sinus, floor of the orbit, and interestingly extending to the oral cavity through the alveolar bone and buccal mucosa, preserving the palate, leading to a primary misdiagnosis of aggressive periodontal [ncbi.nlm.nih.gov]
Musculoskeletal
-
Osteophyte
X-ray nasopharynx showed anterior osteophytes in the cervical vertebrae suggestive of cervical spondylosis. Negative microbiological findings ruled out infectious nature of the lesion. Syphilis was excluded by serology. [ncbi.nlm.nih.gov]
Urogenital
-
Testicular Disease
The mean age at presentation with primary testicular disease (stage IE) is 44 years (range: 30-66 years) [2]. Most of the patients are Asian. [latunisiemedicale.com]
-
Testicular Mass
We report the case of a 28-year-old man presenting with a painful testicular mass noted for six weeks. The patient received surgical testicular and epididymal biopsies. [latunisiemedicale.com]
Neurologic
-
Paresthesia
Afterward, he presented paresthesia and paresis of the lower extremities, difficulty walking, loss of sphincter control, and seizures. [ncbi.nlm.nih.gov]
Workup
A complete staging workup was initiated. [cureus.com]
[…] anaplastic TCL, five cases of NK-like TCLs with similar histologic features but lacked EBER expression and displayed clonal T-cell receptor- γ gene rearrangement, and 10 cases with no additional tissue available for immunohistochemical or molecular workup [nature.com]
Serum
-
Anisocytosis
In peripheral blood smear- RBCs showed mild anisocytosis with hypochromia, microcytes (positive), few macrocytes were seen, WBC series showed neutrophilia, toxic granules were seen in neutrophils, platelets were adequate and parasites were absent. [ncbi.nlm.nih.gov]
Treatment
In disseminated ENKL, systemic chemotherapy remains the mainstay of treatment 2. However, in recent years, novel agents have been identified for the treatment of ENKL. Chemotherapeutic agents not affected by P-glycoprotein, such as methotrexate 4. [bloodjournal.org]
After completion of treatment, patients should consider consolidative treatment with bone marrow transplantation. [tcllfoundation.org]
Treatment [ edit ] Overview [ edit ] RT is recommended as the primary treatment. Consolidative CT should be considered for pts with adverse risk factors or Stage IIE disease. [en.wikibooks.org]
SMILE, VIPD) The type of salvage treatment will not be a criterion for exclusion. Thus, patients who had received various kinds of salvage treatment including anthracycline-containing regimens can be included. [clinicaltrials.gov]
Surgery is not an appropriate treatment without coadjutant therapy. [webmedcentral.com]
Prognosis
Secondary Lymphoid Tissue This is distinct from Aggressive NK Cell Leukemia (ANKL) (see NK Cell Leukemia ), which is believed to be derived from stage 5 NK cells in peripheral blood These two diseases share poor responsiveness to chemotherapy and a poor prognosis [mdnxs.com]
Infection with EBV and high levels of LDH can be found and constitute findings of poor prognosis (8). [revistas.unal.edu.co]
Prognosis Clinically aggressive; dismal prognosis. [medical-dictionary.thefreedictionary.com]
Based on this recent progress, nowadays this subtype is categorized as a lymphoma with intermediate prognosis, but the overall treatment results are not satisfactory 4. [bloodjournal.org]
Extra-nodal NK/T-cell lymphoma should be distinguished from peripheral T-cell lymphoma since it is associated with poorer prognosis compared with peripheral T-cell. [latunisiemedicale.com]
Etiology
Causes : The mechanism of disease etiology and pathogenesis remains unclear for Extranodal NK T cell Lymphoma, and studies are needed to explore underlying molecular pathology and etiological factors. [xpertdox.com]
EBER in-situ hybridization ( Am J Clin Pathol 2001;115:266 ) In US, paranasal sinus lymphomas are often diffuse large B cell type, EBV- in one study ( Am J Surg Pathol 1999;23:1356 ) Gynecologic tumors are rare ( Arch Pathol Lab Med 2000;124:1510 ) Etiology [pathologyoutlines.com]
(Etiology) The exact cause of Extranodal NK/T Cell Lymphoma, Nasal type is not completely understood However, it is observed that there is a universal correlation with being infected with the Epstein Barr virus; the virus that causes mononucleosis infection [dovemed.com]
In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology [books.google.it]
Etiology Risk factors for NKTCL are as follows: Inherited immunodeficiencies – Klinefelter syndrome, Chédiak-Higashi syndrome, ataxia telangiectasia syndrome, Wiskott-Aldrich syndrome, and common variable immunodeficiency disease Acquired immunodeficiency [emedicine.medscape.com]
Epidemiology
Epidemiology Sex : M F Median Age of Onset : 50’s Race : occur mostly in non-Caucasians Presentation : 70-80% of cases present with limited stage (stage I or II) disease Physiology Probable Derivation from Stage 4 NK Cells in Secondary Lymphoid Tissue [mdnxs.com]
[…] destructive lesion s Pain in the chest, abdomen, or bone s (for no known reason) Ulcerated nodular skin lesion s Weight loss (for no known reason) Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Progression and Transformation None Epidemiology [seer.cancer.gov]
Summary To define the epidemiological and clinical features and complementary investigation findings of extranodal NK/T-cell lymphoma, nasal type and to discuss the diagnostic difficulties and the various treatment options. [em-consulte.com]
Epidemiology and pathogenesis of nasal NK/T-cell lymphoma: a mini-review. ScientificWorldJournal. 2011 Feb 14. 11:422-8. [Medline]. Swerdlow E, Campo E, Harris NE, et al. IARC, Lyon. [emedicine.medscape.com]
Mortality patterns of rock and slag mineral wool production workers: an epidemiological and environmental study. British journal of industrial medicine. 1982 Feb;39(1):45-53. 45. Cancer IAfRo, Humans IWGotEoCRt. [ijorl.mums.ac.ir]
Pathophysiology
This characterization led to the identification of deregulated genes and signaling pathways that might be relevant to the pathophysiology and clinicopathologic features of the disease and bring rationale for the development of new therapies. [bloodjournal.org]
T-cell lymphoma Angioimmunoblastic T-cell lymphoma Anaplastic large-cell lymphoma T-cell proliferations of uncertain malignant potential include the following: Lymphomatoid papulosis Hodgkin lymphoma Histiocytic and dendritic-cell neoplasms Mastocytosis Pathophysiology [emedicine.medscape.com]
However, EBV nodal NKTCL is manifested primarily by its involvement in lymph nodes ; it also has clinical, pathological, pathophysiological, and genetic features that differ significantly from those of ENKTCL-NT. [en.wikipedia.org]
Prevention
Screening and prevention of colorectal cancer Source: Finnish Medical Society Duodecim This article is freely available only to users in the UK. [evidence.nhs.uk]
CNS prophylaxis aimed at preventing the lymphoma cells from entering the brain or spinal cord. [xpertdox.com]
CNS prophylaxis is used to try to prevent the lymphoma cells from entering the brain or spinal cord. CNS prophylaxis is usually given as intrathecal chemotherapy. [cancer.ca]
These affect the activity of the immune system in several different ways; they can help the immune system to attack the lymphoma cells and prevent the lymphoma from growing. proteasome inhibitors like bortezomib (Bortezomib Accord or Velcade ) and carfilzomib [lymphomas.org.uk]