Due to the aggressive nature of the disease, patients may initially present at a more advanced stage, with coagulopathies, hemophagocytic syndrome, and multi-organ failure. [en.wikipedia.org]

All patients presented acutely, with fever (6/7), cytopenias (6/7), and splenomegaly (4/7). Four patients had lymphadenopathy, 4 had extranodal disease. Bone marrow involvement was present in 5, with hemophagocytosis in 3. [ncbi.nlm.nih.gov]

[…] granular lymphocyte leukemia Prevalence: Unknown Inheritance: Not applicable or Multigenic/multifactorial Age of onset: Adult ICD-10: C94.7 OMIM: - UMLS: C1292777 C1522378 MeSH: - GARD: 10493 MedDRA: 10028811 The documents contained in this web site are presented [orpha.net]

• Abdominal Lymphadenopathy

  A PEC-CT for staging of renal cell carcinoma revealed marked splenomegaly with hypointense nodules, mild abdominal lymphadenopathy, and new lung nodules, concerning for possible metastatic carcinoma or lymphoma. [nature.com]

• Fatigue

  Patients usually present with constitutional symptoms (malaise, weight loss, fatigue), and hepatosplenomegaly is commonly found on physical exam. Lymphadenopathy is also found to a lesser extent. [en.wikipedia.org]

  Epstein-Barr virus (EBV) A type of herpes virus that causes mononucleosis (a highly infectious disease that causes fever, fatigue, malaise and sore throat). [cancer.ca]

  It is most commonly used in combination with chemotherapy The radiation may be administered by a machine placed outside the body, or by placing a radioactive material inside the body The side effects of radiation therapy include nausea, vomiting, fatigue [dovemed.com]

  Constant antigenic stimulus such as a cytomegalovirus reactivation may be the underlying etiology of clonal T-LGL expansion and may contribute to cytopenias and fatigue seen in transplant patients [ 38 ]. 5. [intechopen.com]

  The patient was admitted with a fever of 102F for 10 days, fatigue, and night sweats. Prior to the presentation, he had 3 months of intermitted low-grade fever, leukopenia and thrombocytopenia. [nature.com]

• Fever of Unknown Origin

  Neta Goldschmidt, Alexander Gural, Abraham Kornberg, Galia Spectre, Andrei Shopen and Ora Paltiel, Prolonged fever of unknown origin and hemophagocytosis evolving into acute lymphoblastic leukemia, American Journal of Hematology, 76, 4, (364-367), (2004 [doi.org]

  Patient 2 was a 48-year-old African-American male with a history of type 2 diabetes, who initially presented with fever of unknown origin, night sweats, myalgia, and headache. [nature.com]

  He presented with a fever of unknown origin, subsequently developed erythematous skin nodules, generalized lymphadenopathy, and hepatosplenomegaly, and then died of multiple organ failure. [thedoctorsdoctor.com]

• High Fever

  fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. [orpha.net]

  Clinics Patients usually present extremely ill with high fever, significant weight loss, cytopenia, jaundice and abnormal liver function. Hepatosplenomegaly is often massive accompanied by lymphadenopathy but less commonly skin lesions. [atlasgeneticsoncology.org]

  T-cells or NK-cells [129,142] Severe mosquito bite allergy (SMBA) Hypersensitivity to mosquito bites, characterized by high fever after mosquito bites, ulcers, necrosis, and scarring. [omicsonline.org]

• Sore Throat

  Epstein-Barr virus (EBV) A type of herpes virus that causes mononucleosis (a highly infectious disease that causes fever, fatigue, malaise and sore throat). [cancer.ca]

• Diplopia

  Yamashita Shun-ichiro Kageyama Ikuyo Endo Hiromasa Tsuda Hideki Akiyama Images in Hematology First Online: 25 May 2011 A 22-year-old woman was referred to our hospital with a 1-month history of recurrent fever, subacute onset of bilateral orbital pain, and diplopia [link.springer.com]

• Hypertriglyceridemia

  The diagnosis of HLH can be established by: A. a molecular diagnosis consistent with HLH or B. five of 8 criteria listed below are fulfilled; 1) Fever; 2) Splenomegaly; 3) Bicytopenias (hemoglobin 9 /L, platelets 9 /L); 4)Hypertriglyceridemia ( 265 mg [sciforschenonline.org]

  Patients usually have fever, splenomegaly and cytopenias, hypertriglyceridemia and hypofibrinogenemia, and hemophagocytosis in the BM, spleen, and/or LN [ 142 ]. [omicsonline.org]

• Bicytopenia

  The diagnosis of HLH can be established by: A. a molecular diagnosis consistent with HLH or B. five of 8 criteria listed below are fulfilled; 1) Fever; 2) Splenomegaly; 3) Bicytopenias (hemoglobin 9 /L, platelets 9 /L); 4)Hypertriglyceridemia ( 265 mg [sciforschenonline.org]

• Erythroblast

  […] x1012/L 4.35-5.55 hemoglobin 14.1 g/dL 13.7-16.8 hematocrit 42.6 % 40.7-50.1 platelet 18 x109/L 158-348 white blood cell 3.7 x109/L 3.3-8.6 Hemogram myelocyte 5 % 0 neutrophil 58 % 40-69 lymphocyte 33 % 21-35 monocyte 1 % 4-8 atypical lymphocyte 3 % 0 erythroblast [sciforschenonline.org]

• Hypercellular Bone Marrow

  When a bone marrow biopsy is performed, the abnormal NK-cell infiltrate could be overlooked due to interstitial infiltration in a background of a hypercellular bone marrow. [nature.com]

Chemotherapy is the main treatment for stage III/IV nasal NK cell lymphomas, as well as the non-nasal and aggressive subtypes. [ncbi.nlm.nih.gov]

Pralatrexate is one compound currently under investigations for the treatment of PTCL. [en.wikipedia.org]

Treatments ANKL is treated with chemotherapy. Some people may be offered a stem cell transplant. Chemotherapy Chemotherapy is the main treatment for ANKL. [cancer.ca]

The prognosis of ENKL was better than that of ANKL (median survival 10 versus 1.9 months, P 0.0001) but was comparable when restricted to stage IV cases (4.0 months, P 0.16). [ncbi.nlm.nih.gov]

Extranodal NK/T-Cell Lymphoma, Nasal Type (ENKL) (see Extranodal NK, T-Cell Lymphoma, Nasal Type ), which is believed to be derived from stage 4 NK cells in secondary lymphoid tissue These two diseases share a poor responsiveness to chemotherapy and poor prognosis [mdnxs.com]

ANKL usually progresses quickly and often has a poor prognosis. There is a very rare slow-growing (indolent) type of NK-cell leukemia that has a more favourable prognosis. [cancer.ca]

AU - Olteanu, Horatiu PY - 2017/5/1 Y1 - 2017/5/1 N2 - Aggressive NK cell leukemia (ANKL) is an exceedingly rare form of leukemia and carries a poor prognosis, with a median survival of only 2 months. [jhu.pure.elsevier.com]

We could not perform biopsy of the lesions due to the poor general condition of the patient, and the exact etiology of her ocular manifestation remains to be elucidated. [link.springer.com]

[…] predominance Sites Most commonly peripheral blood, bone marrow, liver and spleen, but any organ can be affected Overlap with extranodal NK/T cell lymphoma with multiorgan involvement may exist; unclear if they represent different facets of the same disease Etiology [pathologyoutlines.com]

There is no association with EBV virus and the etiology is unclear. Figure 2. Flow cytometry, NK cell, CD3- and CD16 # 2. [blog.cellavision.com]

Etiology ANKL is almost always associated with Epstein-Barr virus (EBV) infection. EBV plays a role in the pathobiology of ANKL and is considered responsible for its aggressive clinical features. [atlasgeneticsoncology.org]

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.com]

Epidemiology Incidence Rare: only 150 cases have been reported in the literature Accounts for Sex : approximately M F Median Age of Onset : 40’s Race : occur mostly in non-Caucasians Association with Epstein-Barr Virus (see Epstein-Barr Virus ) Physiology [mdnxs.com]

Epidemiology and Mortality Age: median age 42 Incidence: rare Sex: slight male dominance Sources Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds): WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised [seer.cancer.gov]

Epidemiology Asian young to middle age adults with fever, hepatosplenomegaly, lymphadenopathy Rare, especially in non-Asian populations Median age 42 year; either no sex predilection or slight male predominance Sites Most commonly peripheral blood, bone [pathologyoutlines.com]

No Article Media No Metrics Keywords: Epstein-Barr virus ; JAK/STAT pathway ; NK cells ; aggressive NK leukemia/lymphoma ; epidemiology Document Type: Research Article Affiliations: 1: Laboratory of Pathology, Center for Cancer Research, National Cancer [ingentaconnect.com]

Jump to: navigation, search Aggressive NK-cell leukemia Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Aggressive NK-cell leukemia from other Diseases Epidemiology and Demographics [wikidoc.org]

[…] at Clinical Trials.gov US National Guidelines Clearinghouse NICE Guidance FDA on Aggressive NK-cell leukemia pathophysiology CDC on Aggressive NK-cell leukemia pathophysiology Aggressive NK-cell leukemia pathophysiology in the news Blogs on Aggressive [wikidoc.org]

These results indicated that the chemokine system might play an important role in the pathophysiology of ANKL and that chemokine receptor profiling might be a novel tool for discriminating ANKL cells from benign NK cells. [ncbi.nlm.nih.gov]

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.com]

Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 Aggressive NK-cell leukemia pathophysiology On the Web Most recent articles Most cited articles Review articles CME Programs Powerpoint slides [wikidoc.org]

CNS prophylaxis is used to try to prevent lymphoma cells from entering the tissue covering the brain and spinal cord. CNS prophylaxis or treatment may be given as intrathecal chemotherapy. [cancer.ca]

Read now World Cancer Day 2019 This year we are joining the mission by collating some of the high impact research published in Oxford University Press journals which highlight prevention and risk factors of different cancers. [annonc.oxfordjournals.org]

How can Aggressive Natural Killer Cell Leukemia be Prevented? Currently, the cause of Aggressive Natural Killer Cell Leukemia is unknown. Hence, it is not possible to prevent the condition. [dovemed.com]

EBV-infected NK cells constitutively secrete high levels of interferon-gamma (IFN-G) which helps maintain cell survival, and preventing apoptosis via autocrine fashion [15]. [sciforschenonline.org]