Hemophagocytic Lymphohistiocytosis in the Elderly

Altook, Reyna; Ruzieh, Mohammed; Singh, Avneet; Alamoudi, Wael; Moussa, Zeinab; Alim, Hussam; Safi, Fadi; Duggan, Joan

doi:10.1016/j.amjms.2018.07.004

Cited by 11 publications

(6 citation statements)

References 59 publications

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“…It commonly presents as a febrile illness with multiple organ dysfunction mimicking infections, malignancies, and autoimmune disorders. In a study of 71 elderly patients with HLH, fever was the presenting symptom in all 100% of patients [6]. The HLH-2004 study included 369 cases, and fever was a presenting symptom in 95% of patients, while bicytopenia and splenomegaly were seen in 92% and 89% of cases, respectively [7].…”

Section: Discussionmentioning

confidence: 99%

A Diagnostic Dilemma of Secondary Hemophagocytosis Lymphohistiocytosis in an Elderly Patient

Mulkareddy¹,

Bhalla²,

Garg³

2020

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a rapidly progressive fatal condition. Although well described in the pediatric population, cases of secondary HLH are seen in adolescents and young adults. In the elderly, HLH has been shown to have a poor prognosis. Owing to its varied presentation and multisystemic involvement, diagnosis is often delayed. Due to its high mortality, prompt diagnosis and treatment are crucial. Here we present a case of secondary HLH in a 69-year-old male, who presented with fever for one week. Initial laboratory workup revealed a bicytopenia and elevated creatinine. He was initially treated with broad-spectrum antibiotics; however, a comprehensive infectious workup was negative. CT scan of the abdomen revealed splenomegaly. Further investigations revealed an elevated ferritin and triglycerides. Due to the constellation of findings, he was started on corticosteroids for concerns of HLH. Bone marrow biopsy was obtained, which revealed dysplastic changes and hemophagocytosis, consistent with HLH. This case highlights the diagnostic challenge and prognosis of HLH in the elderly population, suggesting that diagnosis and treatment should not be delayed for histological confirmation.

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Section: Discussionmentioning

confidence: 99%

A Diagnostic Dilemma of Secondary Hemophagocytosis Lymphohistiocytosis in an Elderly Patient

Mulkareddy¹,

Bhalla²,

Garg³

2020

Cureus

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“…HLH should be suspected when any of the characteristic signs and symptoms of HLH are present which include fever, splenomegaly, blood cytopenia, hepatitis and/or hepatomegaly, coagulopathy, and central nervous system disturbances [ 4 ]. Patients with hepatic involvement can progress to acute liver failure, which necessitates a liver transplant emergently in some cases [ 5 ]. As HLH can mimic many conditions, including liver failure and septic shock, the diagnosis may easily be missed.…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis (HLH) in an Elderly Male With Epstein-Barr Virus (EBV) Viremia

Ghias,

Carducci,

Romero

et al. 2024

Cureus

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This is a case of a 75-year-old male with a complicated past medical history who presented initially with weakness, fevers, exertional dyspnea, cough, and confusion. His initial workup revealed elevated aspartate transaminase (AST), alanine transaminase (ALT), bilirubin, and D-dimer. Right upper quadrant (RUQ) ultrasound revealed a partially contracted gallbladder with gallstones, so he underwent laparoscopic cholecystectomy. Due to worsening hyperbilirubinemia and anemia, he later underwent a liver biopsy which showed Epstein-Barr virus (EBV)-positive lymphoid infiltration. He developed anemia, thrombocytopenia, and low fibrinogen. He met the criteria for hemophagocytic lymphohistiocytosis (HLH) with 6/8 HLH-2004 criteria and an H score of 230 with a 96-98% probability of HLH. The patient was promptly treated with steroids, rituximab, and etoposide; however, the patient’s health continued to deteriorate, and he expired. This case highlights the challenges of early diagnosis of HLH in the elderly patient population due to large differentials, confounding comorbidities, and the rarity of the diagnosis in this age range.

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“… [3] Altook et al . [4] described HLH as ‘a rare disease of massive, dysregulated cytokine release and secondary multi-organ failure […] associated with high mortality’. [4] …”

Section: Introductionmentioning

confidence: 99%

“…However, it is increasingly recognised that some patients with secondary HLH may have an underlying genetic predisposition. [4 , 7 , 11]…”

Section: Introductionmentioning

confidence: 99%

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Haemophagocytic lymphohistiocytosis: Five years’ experience at tertiary hospitals in Free State Province, South Africa

2020

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The first case of haemophagocytic lymphohistiocytosis (HLH) was described in 1939 by Scott and Robb-Smith. [1] After their patients had initially been diagnosed with 'atypical Hodgkin disease' , they named the condition histiocytic medullary reticulosis. [1,2] It was later classified as a type of malignant histiocytosis. [3] Altook et al. [4] described HLH as 'a rare disease of massive, dysregulated cytokine release and secondary multiorgan failure […] associated with high mortality'. [4] HLH is classified according to aetiology, namely primary (genetic) or secondary (acquired). [5] Patients with primary HLH have an underlying genetic cause or a history of familial predisposition. These underlying causes involve genetic mutations that eradicate proteins crucially required for normal function of cytotoxic T-cells and natural killer (NK) cells. [6] In 70-80% of cases, primary HLH occurs in infants and young children, [7] with symptoms presenting within the first year of life, and 10% of cases diagnosed in the neonatal period. [8-10] However, cases have been described in older children and adults. [11] Secondary HLH occurs in the absence of a known genetic lesion and is triggered by an underlying condition resulting from a malignant, infectious, or autoimmune stimulus. [6] The secondary causes of HLH are summarised in Table 1. However, it is increasingly recognised that

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Hemophagocytic Lymphohistiocytosis in the Elderly

Cited by 11 publications

References 59 publications

A Diagnostic Dilemma of Secondary Hemophagocytosis Lymphohistiocytosis in an Elderly Patient

A Diagnostic Dilemma of Secondary Hemophagocytosis Lymphohistiocytosis in an Elderly Patient

Hemophagocytic Lymphohistiocytosis (HLH) in an Elderly Male With Epstein-Barr Virus (EBV) Viremia

Haemophagocytic lymphohistiocytosis: Five years’ experience at tertiary hospitals in Free State Province, South Africa

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