A Case of Hemophagocytic Lymphohistiocytosis Associated With Mediterranean Spotted Fever in a Healthy 29-Year-Old Female

Karra, Nour; Dolinski, Rina; Akria, Luiza; Yampoulski, Yevgeni; Awad, Jamal

doi:10.1093/ofid/ofaa355

Cited by 7 publications

(5 citation statements)

References 44 publications

(51 reference statements)

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“…MSF can trigger the onset of HLH. 14 15 HLH was an initial concern in our patient given the high ferritin, however, he did not progress to develop a clinical or biochemical syndrome consistent with HLH. Complications in the convalescent phase of MSF are thought to be mostly secondary to immune-mediated mechanisms and are primarily neurological, including sensorineural hearing loss, 9 aseptic meningitis, meningoencephalitis, myelitis, polyradiculoneuritis and facial paralysis.…”

Section: Discussionmentioning

confidence: 63%

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Mediterranean spotted fever with multiorgan involvement

Davis

Ahmado

Warrell³

et al. 2022

BMJ Case Rep

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A previously well man in his 50s returned to the UK after a trip to the Mediterranean. The day after returning he developed malaise, fevers, rigors and severe headache. He was hospitalised with sepsis, multiorgan involvement, a maculopapular rash and an eschar on each hip. Serology was positive forRickettsiaspp (spotted fever group) with a rise in titre from 1:64 to 1:1024 eight days later. Blood and tissue PCR were also positive forRickettsiaspp. He had cardiac, pulmonary, renal, ocular and neurological involvement. He completed a 14-day course of doxycycline and recovered well. This is a case of likely Mediterranean spotted fever (MSF) caused byRickettsia conorii,which is endemic to the Mediterranean basin. We highlight the need for awareness and early treatment to prevent severe complications. This case is also the first to describe Purtscher-like retinopathy in the context of likely MSF.

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Section: Discussionmentioning

confidence: 63%

“…Gastrointestinal bleeding and rhabdomyolysis13 can also occur. MSF can trigger the onset of HLH 14 15. HLH was an initial concern in our patient given the high ferritin, however, he did not progress to develop a clinical or biochemical syndrome consistent with HLH.…”

Section: Discussionmentioning

confidence: 65%

Mediterranean spotted fever with multiorgan involvement

Davis

Ahmado

Warrell³

et al. 2022

BMJ Case Rep

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“…Diarrhoea and bowel symptoms are not part of the formal HLH 2004 or HLH 2008 diagnostic criteria, and as a result, the presence of diarrhoea in HLH/MAS is not well-described in the literature 11. A handful of case reports have noted an association between HLH and diarrhoea secondary to various abdominal pathologies such as appendicitis, IBD, infectious colitis and T-cell lymphoma 38–42. Pagel et al identified a subtype of inherited familial HLH (type 5 familial HLH) presenting with copious, severe, chronic watery diarrhoea, in which defects in the lysosomal-dependent exocytosis pathway (STXBP2) were inherited in an autosomal recessive manner 43.…”

Section: Discussionmentioning

confidence: 99%

Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis

et al. 2022

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Haemophagocytic lymphocytic histiocytosis (HLH) is a rare, life-threatening condition caused by abnormal activation of cytotoxic T lymphocytes, natural killer cells and macrophages resulting in hypercytokinaemia and immune-mediated injury of multiple organ systems. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others. Commonly observed and documented symptoms include fever, organomegaly and lymphadenopathy. Given the potential for multiorgan failure in HLH/MAS, early identification, diagnosis and initiation of treatment is essential. We present a case of secondary HLH/MAS with acute inflammatory gastroenteritis in a middle-aged woman with a history of systemic lupus erythematosus.

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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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A Case of Hemophagocytic Lymphohistiocytosis Associated With Mediterranean Spotted Fever in a Healthy 29-Year-Old Female

Cited by 7 publications

References 44 publications

Mediterranean spotted fever with multiorgan involvement

Mediterranean spotted fever with multiorgan involvement

Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis

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

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