High frequency of fatal haemophagocytic lymphohistiocytosis syndrome in enteropathy-associated T cell lymphoma

2012 ◽  
Vol 44 (4) ◽  
pp. 343-349 ◽  
Author(s):  
Aurelien Amiot ◽  
Matthieu Allez ◽  
Xavier Treton ◽  
Claire Fieschi ◽  
Lionel Galicier ◽  
...  
Keyword(s):  
T Cell ◽  
High Frequency ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Haemophagocytic Lymphohistiocytosis

scholarly journals Natural killer/T-cell lymphoma and secondary haemophagocytic lymphohistiocytosis in pregnancy

2018 ◽  
pp. bcr-2018-224832
Author(s):  
Barbara Neistadt ◽  
Aakriti Carrubba ◽  
Michael V Zaretksy
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Multiorgan Failure ◽  
Male Infant ◽  
Fetal Distress ◽  
Chemotherapeutic Agents ◽  
T Cell Lymphoma ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Aged Woman ◽  
In Pregnancy

Haemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disorder. It is challenging to diagnose due to its rarity and variation in clinical presentation, laboratory abnormalities and underlying aetiologies. A reproductive-aged woman, gravida 2 para 1001 at 27 weeks gestation presented with fever, hypotension and subacute upper respiratory infection. She delivered a male infant by caesarean section secondary to fetal distress. Subsequently, she was diagnosed with T-cell lymphoma and secondary HLH. Despite management with supportive care and multiple chemotherapeutic agents, she ultimately died of multiorgan failure. Patients with HLH secondary to malignancy have a particularly poor prognosis. This case highlights the importance of considering secondary HLH in the differential diagnosis of a patient with fever, pancytopenia and systemic symptoms of unclear aetiology in pregnancy.

High frequency of Epstein?Barr virus in Chinese peripheral T-cell lymphoma

1994 ◽  
Vol 24 (2) ◽  
pp. 115-122 ◽  
Author(s):  
X.G. ZHOU ◽  
S.J. HAMILTON-DUTOIT ◽  
Q.H. YAN ◽  
G. PALLESEN
Keyword(s):  
T Cell ◽  
High Frequency ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Barr Virus ◽  
Epstein Barr

scholarly journals High Frequency of Genetic Aberrations in Enteropathy-Type T-Cell Lymphoma

2003 ◽  
Vol 83 (10) ◽  
pp. 1509-1516 ◽  
Author(s):  
Anne K Baumgärtner ◽  
Andreas Zettl ◽  
Andreas Chott ◽  
German Ott ◽  
Hans Konrad Müller-Hermelink ◽  
...  
Keyword(s):  
T Cell ◽  
High Frequency ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Genetic Aberrations

Low Frequency of Epstein Barr Virus Association and High Frequency of p53 Overexpression in an Argentinean Pediatric T-Cell Lymphoma Series

2009 ◽  
Vol 12 (1) ◽  
pp. 28-34 ◽  
Author(s):  
Paola Chabay ◽  
Elena De Matteo ◽  
Mario Lorenzetti ◽  
Anahí Vijnovich Barón ◽  
Pamela Valva ◽  
...  
Keyword(s):  
T Cell ◽  
High Frequency ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
Low Frequency ◽  
T Cell Lymphoma ◽  
P53 Overexpression ◽  
Barr Virus ◽  
Epstein Barr

scholarly journals Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics

Blood ◽  
1994 ◽  
Vol 84 (8) ◽  
pp. 2640-2648 ◽  
Author(s):  
B Schlegelberger ◽  
Y Zhang ◽  
K Weber-Matthiesen ◽  
W Grote
Keyword(s):  
T Cell ◽  
High Frequency ◽  
Cell Lymphoma ◽  
Single Cells ◽  
T Cell Lymphoma ◽  
Neoplastic Disease ◽  
Cell Of Origin ◽  
Interphase Cytogenetics ◽  
Angioimmunoblastic Lymphadenopathy ◽  
T Cell Lymphomas

Abstract Trisomy 3, trisomy 5, and an X additional chromosome are the most frequent chromosome aberrations in angioimmunoblastic lymphadenopathy with proteinemia (AILD)-type T-cell lymphomas. To evaluate the frequency of +3 and +X clones, fluorescence in situ hybridization studies with centromere-specific probes for chromosome 3 and X were done in 41 patients with peripheral T-cell lymphomas (PTL). With this interphase cytogenetic approach, 32 of 41 patients (78%) showed +3 clones, and 14 patients (34%) +X clones. These frequencies far exceeded those observed with metaphase cytogenetics (+3, 41%; +X, 20%). Summing up the results of metaphase and interphase cytogenetics, aberrant clones were found in 37 of 41 patients with PTL (90%) and 32 of 36 patients with AILD-type T-cell lymphoma (89%). Although AILD-type T- cell lymphoma is considered a neoplastic disease, it is an exception in that it shows a high frequency of cytogenetically unrelated clones and single cells that cannot be derived from a common cell of origin because of their completely different karyotypes. In five patients, double hybridization with centromere-specific probes for chromosomes 3 and X showed that these aberrations occurred in different cells. When the results of metaphase and interphase cytogenetics were combined, 17 of 36 patients with AILD-type T-cell lymphoma (47%) had unrelated clones. This high frequency of oligoclonal proliferations may be caused by increased genetic instability and an immune defect resulting in impaired elimination of aberrant cells.

scholarly journals Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics

Blood ◽  
1994 ◽  
Vol 84 (8) ◽  
pp. 2640-2648 ◽  
Author(s):  
B Schlegelberger ◽  
Y Zhang ◽  
K Weber-Matthiesen ◽  
W Grote
Keyword(s):  
T Cell ◽  
High Frequency ◽  
Cell Lymphoma ◽  
Single Cells ◽  
T Cell Lymphoma ◽  
Neoplastic Disease ◽  
Cell Of Origin ◽  
Interphase Cytogenetics ◽  
Angioimmunoblastic Lymphadenopathy ◽  
T Cell Lymphomas

Trisomy 3, trisomy 5, and an X additional chromosome are the most frequent chromosome aberrations in angioimmunoblastic lymphadenopathy with proteinemia (AILD)-type T-cell lymphomas. To evaluate the frequency of +3 and +X clones, fluorescence in situ hybridization studies with centromere-specific probes for chromosome 3 and X were done in 41 patients with peripheral T-cell lymphomas (PTL). With this interphase cytogenetic approach, 32 of 41 patients (78%) showed +3 clones, and 14 patients (34%) +X clones. These frequencies far exceeded those observed with metaphase cytogenetics (+3, 41%; +X, 20%). Summing up the results of metaphase and interphase cytogenetics, aberrant clones were found in 37 of 41 patients with PTL (90%) and 32 of 36 patients with AILD-type T-cell lymphoma (89%). Although AILD-type T- cell lymphoma is considered a neoplastic disease, it is an exception in that it shows a high frequency of cytogenetically unrelated clones and single cells that cannot be derived from a common cell of origin because of their completely different karyotypes. In five patients, double hybridization with centromere-specific probes for chromosomes 3 and X showed that these aberrations occurred in different cells. When the results of metaphase and interphase cytogenetics were combined, 17 of 36 patients with AILD-type T-cell lymphoma (47%) had unrelated clones. This high frequency of oligoclonal proliferations may be caused by increased genetic instability and an immune defect resulting in impaired elimination of aberrant cells.

scholarly journals EP18 Use of anakinra for haemophagocytic lymphohistiocytosis in adults

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Luke Sammut ◽  
Saerrah Murryam ◽  
Natalie Vincent ◽  
Brian Davidson ◽  
Christopher J Edwards
Keyword(s):  
T Cell ◽  
Clinical Remission ◽  
Cell Lymphoma ◽  
Ferritin Level ◽  
Treatment Protocol ◽  
T Cell Lymphoma ◽  
Recent Experience ◽  
Atypical Pneumonia ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Life Threatening

Abstract Background Haemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening syndrome characterised by severe immune activation. The primary form occurs in early childhood and is fatal without treatment. Secondary HLH can occur at any age, usually in the setting of autoimmune disease, malignancy and infections. Macrophage activation syndrome is a form of HLH occurring in patients with juvenile idiopathic arthritis or other rheumatological conditions. The 2004 paediatric HLH treatment protocol is an established regime of etoposide, dexamethasone and ciclosporin. In recent years, studies have suggested a role for Anakinra in paediatric patients with HLH. We describe our recent experience with Anakinra in adult patients with secondary HLH. Methods We performed a retrospective case review study of four adults diagnosed with HLH and treated with anakinra between 2014 and 2018. Results Three males and one female with mean age of 35 (18-53) fulfilled the 2004 HLH diagnostic criteria. Three patients had confirmed secondary HLH with the primary trigger being CMV infection, a connective tissue disease (CTD) & T-cell lymphoma and adult-onset Still’s disease. The aetiology of the fourth patient was uncertain although viral infection was likely. Common presenting features were fever, hyperferritinaemia and anaemia. Three patients were neutropaenic and two pancytopaenic. The peak mean ferritin level was 50358µg/L, (10052 - 126808). All patients were treated with anakinra 100mg subcutaneously per day. Ferritin fell rapidly, and at day 7 following treatment, the mean was 3688µg/L (645 - 8957). Three patients achieved initial clinical remission. The first stopped anakinra after 14 days of treatment and has done well, the second has remained on treatment. The third achieved initial clinical remission, stopped anakinra after two months but relapsed and died from atypical pneumonia. The fourth with CTD and T-cell lymphoma had a large drop in ferritin but did not achieve remission and died (Table 1). Conclusion HLH is a rare life threatening disorder. Similar to paediatric studies, anakinra appears to reduce ferritin rapidly in adults with secondary HLH. Anakinra may be effective in improving the clinical outcome in adults with non-malignancy associated secondary HLH. However, consistent with previous reports, patients with malignancy associated HLH had a worse survival. Disclosures L. Sammut None. S. Murryam None. N. Vincent None. B. Davidson None. C.J. Edwards Honoraria; C.J.E has received support to attend meetings and received honoraria from Amgen.

Sign in / Sign up

Export Citation Format

Share Document