Hemophagocytic lymphohistiocytosis in adults

Pandey, Yadav; Atwal, Dinesh; Konda, Manojna; Bimali, Milan; Middleton, D.; Yarlagadda, Naveen; Firwana, Belal; Sasapu, Appalanaidu

doi:10.1080/08998280.2020.1740052

Cited by 4 publications

(3 citation statements)

References 24 publications

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“…Among the 41 patients, 35 (85.4%) received HLH-directed therapy, and 19 (46.3%) achieved remission. The most commonly used regimen for HLH treatment was a combination of dexamethasone and etoposide (53.7%) (10).…”

Section: Discussionmentioning

confidence: 99%

Successful management of adult hemophagocytic lymphohistiocytosis with an etoposide-containing regimen: Case report and literature review

Yıldız

Akdeniz²,

Çebi

et al. 2023

Med Sci Discov

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Objective: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by a hyperimmune response. Although HLH is well described in the pediatric population, less is known about the appropriate management in adults. Despite the inclusion of etoposide in HLH society protocols, some clinicians refrain from using it due to its cytotoxicity and potential adverse effects. Case: We report here the case of a 43-year-old male presenting with fever and pancytopenia, who was diagnosed with HLH following further evaluation. The patient immediately started the HLH-2004 initial protocol, which consisted of dexamethasone, etoposide, cyclosporine, and IVIG. Symptoms improved after 2 weeks of treatment. Subsequent bone marrow biopsy showed normal results, and the patient achieved complete remission, leading to the termination of treatment in the 12th week of the protocol. At 8 months post-treatment, the patient remained recurrence-free with normal hematological and biochemical test results. Conclusion: This case underscores the importance of considering etoposide-containing regimens, particularly for medically fit patients.

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Section: Discussionmentioning

confidence: 99%

Successful management of adult hemophagocytic lymphohistiocytosis with an etoposide-containing regimen: Case report and literature review

Yıldız

Akdeniz²,

Çebi

et al. 2023

Med Sci Discov

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“…In the healthy individual, cytotoxic lymphocytes (CTLs) and Natural Killer (NK) cells help regulate the immune system by lysing excess or damaged macrophages. 1 In HLH, dysfunction of immune-regulating cells causes overactivation of macrophages which engulf blood cells (causing cytopenia), leading to a cytokine storm and quickly progressing to multiorgan failure. 1 HLH is most common in the pediatric population, where it can be linked to an underlying genetic disorder.…”

Section: Introductionmentioning

confidence: 99%

“… 1 In HLH, dysfunction of immune-regulating cells causes overactivation of macrophages which engulf blood cells (causing cytopenia), leading to a cytokine storm and quickly progressing to multiorgan failure. 1 HLH is most common in the pediatric population, where it can be linked to an underlying genetic disorder. 2 However, it is becoming increasingly recognized in adults, who may account for up to 40% of cases.…”

Section: Introductionmentioning

confidence: 99%

A Fatal Etiology of Splenic Infarction

Masterman¹,

Singh²,

Singh³

et al. 2022

Journal of Community Hospital Internal Medicine Perspectives

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Hemophagocytic lymphohistiocytosis (HLH) is a rare systemic inflammatory disorder that is rapidly progressive and carries a poor prognosis. We present a case of HLH caused by undiagnosed B cell lymphoma, presenting initially with splenic infarction. Both HLH and splenic infarction are secondary conditions with a wide range of underlying etiologies. When either condition is identified, a prompt search for the underlying trigger is needed to prevent devastating consequences. We demonstrate the difficulties and barriers that can delay the diagnosis of HLH, and emphasize the importance of early treatment in improving survival rates.

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Case of haemophagocytic lymphohistiocytosis following Epstein-Barr virus infection

2021

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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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Hemophagocytic lymphohistiocytosis in adults

Cited by 4 publications

References 24 publications

Successful management of adult hemophagocytic lymphohistiocytosis with an etoposide-containing regimen: Case report and literature review

Successful management of adult hemophagocytic lymphohistiocytosis with an etoposide-containing regimen: Case report and literature review

A Fatal Etiology of Splenic Infarction

Case of haemophagocytic lymphohistiocytosis following Epstein-Barr virus infection

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