scholarly journals Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens

2013 ◽  
Vol 162 (3) ◽  
pp. 376-382 ◽  
Author(s):  
DeepakBabu Chellapandian ◽  
Rupali Das ◽  
Kristin Zelley ◽  
Susan J. Wiener ◽  
Huaqing Zhao ◽  
...  
Keyword(s):  
Epstein Barr Virus ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Barr Virus ◽  
Epstein Barr

Haemophagocytic lymphohistiocytosis, interferon-gamma-naemia and Epstein-Barr virus involvement

1994 ◽  
Vol 88 (3) ◽  
pp. 656-658 ◽  
Author(s):  
Shinsaku Imashuku ◽  
Shigeyoshi Hibi ◽  
Fumihiro Fujiwara ◽  
Satoshi Ikushima ◽  
Shinjiro Todo
Keyword(s):  
Interferon Gamma ◽  
Epstein Barr Virus ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Barr Virus ◽  
Epstein Barr

EBV Associated Post-Transplant Lymphoproliferative Disorder Complicated with Haemophagocytic Lymphohistiocytosis After Allogeneic Stem Cell Transplant: A Rare Complication with Fatal Outcome

2020 ◽  
Vol 4 (02) ◽  
pp. 49-51
Author(s):  
Tangia Muquith
Keyword(s):  
Stem Cell ◽  
Lymphoproliferative Disorder ◽  
Epstein Barr Virus ◽  
Stem Cell Transplant ◽  
Cell Transplant ◽  
Allogeneic Stem Cell Transplant ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Post Transplant ◽  
Barr Virus ◽  
Epstein Barr

Epstein –Barr virus related post-transplant lymphoproliferative disorder is a fatal and life-threatening complication because of immunocompromised state.1 Haemophagocytic lymphohistiocytosis is a sign of poor outcome in EBV associated PTLD after allogeneic stem cell transplantation. It is particularly common when in vivo T cell depletion strategies have been applied.2 In both situations, post-transplant lymphoproliferative disorder and Haemophagocytic lymphohistiocytosis, infection with EBV is the key mechanism. Here I present a case of 29 years old female with acute myeloid leukaemia after second allogeneic stem cell transplant, who developed PTLD complicated with Haemophagocytic lymphohistiocytosis secondary to Epstein – Barr virus (EBV) infection. Patient was treated with chemo immunotherapy and responded but ultimately died after 100 days of transplant. The association of HLH and EBV related PTLD is rare and data on outcome of these patients are limited with very high mortality.

Case of haemophagocytic lymphohistiocytosis following Epstein-Barr virus infection

2021 ◽  
Vol 14 (3) ◽  
pp. e241222
Author(s):  
Valeri Kraskovsky ◽  
Jason Harhay ◽  
Martin Jeffery Mador
Keyword(s):  
Blood Cell ◽  
Cell Count ◽  
White Blood Cell Count ◽  
Fdg Pet ◽  
Epstein Barr Virus ◽  
Density Lipoprotein ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Blood Cell Count ◽  
Barr Virus ◽  
Epstein Barr

Haemophagocytic lymphohistiocytosis (HLH) is a rare diagnosis that carries a high degree of mortality. We present this case of a previously healthy 22-year-old woman, who was admitted acutely ill to the hospital. One week prior, she had been seen by her primary care physician for fatigue and malaise. At that time, she was noted to have anterior and posterior cervical lymphadenopathy. She was referred to the emergency room and was diagnosed with acute Epstein-Barr virus (EBV) mononucleosis based on her clinical symptoms and positive heterophile antibody test. She was discharged after an uneventful 48-hour stay on the wards. She represented 7 days after discharge with cough, fatigue, nausea, vomiting, epigastric abdominal pain, diarrhoea, weight loss and subjective fevers. She had also reported haematemesis, epistaxis and melaena. Vital signs included temperature 36.9°C, blood pressure 90/50 mm Hg, heart rate 130 beats per minute and respiratory rate 32 breaths per minute. Physical examination was notable for an acutely ill appearing woman with scleral icterus, hepatosplenomegaly and palpable cervical and axillary lymphadenopathy. Complete blood count showed pancytopaenia with haemoglobin 59 g/L (normal 120–160 g/L), white blood cell count 2.7×109/L (normal 4–10.5×109/L) and platelet count 50×109/L (normal 150–450×109/L). The white blood cell count differential included 58% neutrophils (normal 38%–77%) with immature neutrophils in band form elevated at 45% (normal <14%), 16% lymphocytes (normal 20%–48%), 7% monocytes (normal <12%) and no eosinophils (normal <6%). Blood smear revealed anisocytosis, poikilocytosis and hypochromia. Coagulation panel showed elevated levels of d-dimer level at 1.39 µg/mL (normal <0.45 µg/mL), prolonged prothrombin time at 34.4 s (normal 11–15 s), prolonged activated partial thromboplastin time of 55.6 s (normal 25–34 s), prolonged international normalised ratio at 3.31 (normal <1.1) and low fibrinogen 60 mg/dL (normal >200 mg/dL). Lipid panel showed cholesterol at 114 mg/dL (normal 125–200 mg/dL), triglycerides 207 mg/dL (normal 30–150 mg/dL), high-density lipoprotein cholesterol 10 mg/dL (normal 40–60 mg/dL) and low-density lipoprotein cholesterol 63 mg/dL (normal <100 mg/dL). Other lab abnormalities included elevated ferritin of 6513 ng/mL (normal 10–150 ng/mL) and elevated lactate dehydrogenase of 1071 unit/L (normal 95–240 unit/L). Soluble interleukin-2 receptor alpha level was elevated at 60 727 units/mL (normal 223–710 units/mL). Fluorodeoxyglucose–positron emission tomography (FDG-PET) scan showed abnormal tracer localisation within the paratracheal, hilar, pelvic, abdominal and subcarinal lymph nodes, along with FDG-PET positive hepatosplenomegaly. A bone marrow biopsy showed hypercellular marrow (95% cellularity) with trilineage haematopoiesis, haemophagocytic cells, polytypic plasmacytosis and T-cell lymphocytosis, along with positive latent membrane protein-1 immunohistochemical staining for EBV. EBV quantitative DNA PCR showed >1 million copies. These findings were consistent with a diagnosis of HLH secondary to EBV infection. Despite intense therapy with the HLH-94 protocol, the patient expired from her illness after a prolonged hospital course.

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