Hemophagocytic lymphohistiocytosis (HLH) secondary to Ehrlichia chaffeensis with bone marrow involvement

Naqash, Abdul Rafeh; Yogarajah, Meera; Vallangeon, Bethany D.; Hafiz, Maida; Patel, Deval; Kolychev, Evelina; Lebron, Dora; Liles, Darla

doi:10.1007/s00277-017-3085-8

Cited by 14 publications

(7 citation statements)

References 9 publications

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“…HLH is a rare, but potentially fatal syndrome characterized by uncontrolled and dysfunctional activation of the mononuclear phagocytic system resulting in a hyper inflammatory response 1,2 . Patients can often present with a sepsis‐like syndrome causing multiorgan dysfunction, culture negative sepsis, or encephalopathy of unknown etiology 1,3 . This patient came to the hospital and was approached as fever with neutropenia and received broad spectrum antibiotics as per institutional guidelines.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Patients can often present with a sepsis-like syndrome causing multiorgan dysfunction, culture negative sepsis, or encephalopathy of unknown etiology. 1,3 This patient came to the hospital and was approached as fever with neutropenia and received broad spectrum antibiotics as per institutional guidelines. During his fulminant clinical course, he met the published criteria for a diagnosis of HLH with fever, cytopenia (Hb < 9 and platelets < 100 000), hypertriglyceridemia, elevated ferritin, and sCD25.…”

Section: Discussionmentioning

confidence: 99%

“…There are very few cases reported of HLH secondary to Ehrlichiosis (Table 1). [2][3][4][5][6][7][8][9] This patient was admitted to the hospital in late spring in Arkansas when Ehrlichia infections are more common. Furthermore, Arkansas has one of the highest incidence rates (more than 6.4 cases per million) in the United States according to a Disease Control and Prevention (CDC) report.…”

Section: Discussionmentioning

confidence: 99%

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Ehrlichia‐induced hemophagocytic lymphohistiocytosis after autologous stem cell transplant

Mitma

Burgess²,

Rhee

2021

Transplant Infectious Dis

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Ehrlichia‐induced hemophagocytic lymphohistiocytosis after autologous stem cell transplant

Mitma

Burgess²,

Rhee

2021

Transplant Infectious Dis

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“…The following were excluded from the analysis: articles published in languages other than English; case reports not providing an appropriate amount of information, or where the diagnosis was not certain; and those reporting non-human subjects. The final number of articles included was 69, which resulted in a total number of 98 patients ( Figure 1 ) [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , ...…”

Section: Methodsmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases

Jevtic,

da Silva,

Haylock

et al. 2024

Infectious Disease Reports

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Hemophagocytic lymphohistiocytosis (HLH) secondary to tick-borne infections is a rare but potentially life-threatening syndrome. We performed a scoping review according to PRISMA guidelines to systematically analyze the existing literature on the topic. A total of 98 patients were included, with a mean age of 43.7 years, of which 64% were men. Most cases, 31%, were reported from the USA. Immunosuppression was present in 21.4%, with the most common cause being previous solid organ transplantation. Constitutional symptoms were the most common, observed in 83.7% of the patients, while fever was reported in 70.4% of cases. Sepsis was present in 27.6%. The most common laboratory abnormalities in this cohort were thrombocytopenia in 81.6% of patients, while anemia, leukopenia, and leukocytosis were observed in 75.5%, 55.1%, and 10.2%, respectively. Liver enzyme elevation was noted in 63.3% of cases. The H-score was analyzed in 64 patients, with the mean value being 209, and bone marrow analysis was performed in 61.2% of patients. Ehrlichia spp. was the main isolated agent associated with HLH in 45.9%, followed by Rickettsia spp. in 14.3% and Anaplasma phagocytophilum in 12.2%. Notably, no patient with Powassan virus infection or Lyme borreliosis developed HLH. The most common complications were acute kidney injury (AKI) in 35.7% of patients, shock with multiple organ dysfunction in 22.5%, encephalopathy/seizure in 20.4%, respiratory failure in 16.3%, and cardiac complications in 7.1% of patients. Treatment included antibiotic therapy alone in 43.9%, while 5.1% of patients were treated with immunosuppressants alone. Treatment with both antibiotics and immunosuppressants was used in 51% of patients. Appropriate empiric antibiotics were used in 62.2%. In 43.9% of cases of HLH due to tick-borne disease, patients received only antimicrobial therapy, and 88.4% of those recovered completely without the need for immunosuppressive therapy. The mortality rate in our review was 16.3%, and patients who received inappropriate or delayed empiric therapy had a worse outcome. Hence, we suggest empiric antibiotic treatment in patients who are suspected of having HLH due to tick-borne disease or in whom diagnostic uncertainty persists due to diagnostic delay in order to minimize mortality.

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“…The positive E. Chaffeensis IgG likely represents prior infection, as the patient and family reported multiple tick exposures, or a false positive in setting of his systemic inflammatory state, rather than active infection. This was important to elucidate, as there have been case reports of secondary HLH due to E. Chaffeensis 9–15…”

Section: Differential Diagnosismentioning

confidence: 99%

Secondary hemophagocytic lymphohistiocytosis due to Heartland virus

Ahlers

Matthews²,

Perez³

et al. 2022

BMJ Case Rep

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An older man from the mid-Southeastern USA presented with acute onset of fever, fatigue, and non-bloody diarrhoea. There was high suspicion for tick-borne illness given exposure history, clinical presentation and laboratory abnormalities. Despite prompt treatment with doxycycline, the patient clinically worsened. He was diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH) due to Heartland virus (HRTV). This is the second documented case of secondary HLH due to HRTV, and the first in a relatively immunocompetent patient. Furthermore, though HRTV has been primarily concentrated in the Central USA, our case provides evidence of further geographic expansion of HRTV, mirroring the increased range of the Lone Star tick. Clinicians should consider HRTV when a patient with a clinical presentation consistent with tick-borne illness fails to respond to doxycycline. Furthermore, healthcare providers should be aware of the geographic expansion of HRTV and the potential of HRTV to lead to secondary HLH.

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Hemophagocytic lymphohistiocytosis (HLH) secondary to Ehrlichia chaffeensis with bone marrow involvement

Cited by 14 publications

References 9 publications

Ehrlichia‐induced hemophagocytic lymphohistiocytosis after autologous stem cell transplant

Ehrlichia‐induced hemophagocytic lymphohistiocytosis after autologous stem cell transplant

Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases

Secondary hemophagocytic lymphohistiocytosis due to Heartland virus

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