Forme Fruste of HLH (haemophagocytic lymphohistiocytosis): diagnostic and therapeutic challenges

Inoue, Shinichi; Mangat, Chetna; Rafee, Yaseen; Sharman, Mahesh

doi:10.1136/bcr-2014-206190

Cited by 9 publications

(7 citation statements)

References 23 publications

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“… 24) Both KD and HLH have an inappropriate hypercytokinemia triggered by a variety of factors and greatly modified by genetic heterogeneity in response to infection or inflammation. 25) To date, only primary HLH has been thought to involve genetic factors; however, recently, there is evidence of some degree of genetic predisposition even in secondary HLH. 4) …”

Section: Discussionmentioning

confidence: 99%

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

et al. 2018

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PurposeHemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone.MethodsWe performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups.ResultsThe total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5–1792.0) pg/mL vs. 233.0 (IQR, 107.0–544.0) pg/mL.ConclusionThe NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

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Section: Discussionmentioning

confidence: 99%

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

et al. 2018

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“…This suggests that the pathophysiology of both KD and HLH may include inappropriate upregulation of cytokines perhaps triggered by an unidentified infectious agent. 19 Interestingly, the first case described by Dr. Kawasaki in 1961 was a 4-year-old boy presented with coombs positive hemolytic anemia. 20 The boy had all the common clinical features of KD except rash as well as abnormal hematologic features.…”

Section: Discussionmentioning

confidence: 99%

A Child With Kawasaki Disease and Coronary Artery Aneurysm During Acute Epstein-Barr Virus Infection

Rafee

Abdel‐Haq

2020

Infect Dis Clin Pract

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Kawasaki disease (KD) is the most common childhood cause of acquired coronary artery disease in the developed world. The diagnosis is often challenging, and atypical presentations may be associated with increased morbidity and mortality. We describe an 18-month-old toddler boy who developed clinical manifestations of KD with coronary artery aneurysmal dilatation and autoimmune hemolytic anemia during Epstein-Barr virus infection. The child had evidence of polyclonal activation of B cells that caused serological tests to be positive for different infections. Our case suggests an unusual immunological response to primary Epstein-Barr virus infection may lead to KD manifestation and coronary artery damage.

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“…Therefore, clinical lab ndings such as a low level of NK cells (natural killer), neutropenia, thrombocytopenia, and an extreme elevation of ferritin, particularly in children rather than adults, are important in establishing the disease (10,11). The presence of diseases that mimic the same clinical picture and presentation, such as Kawasaki disease or other autoimmune diseases, makes the search for the true diagnosis so exhaustive and di cult (12) .…”

Section: Introductionmentioning

confidence: 99%

Haemophagocytic lymphocytosis (HLH) vicious cycle in a Cannabinoids addict patient case report and disease review.

Amer

Hamdar

Amer

et al. 2023

Preprint

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Haemophagocytic lymphocytosis (HLH) is a rare life-threatening syndrome caused by the uncontrolled activity of cytokines, natural killers, and macrophages, which can alter the activity of the organism, resulting in multiple organ dysfunction and mortality. Fever, splenomegaly, coagulopathies, dyspnea, changes in mental status, or irritability may be associated with HLH diseases. Depending on the underlying causes, such as bacterial or viral infections, HLH may be primary, hereditary, or secondary. The early diagnosis and treatment of patients are directly related to their prognosis and clinical outcome. On the other hand, HLH can present a number of obstacles, particularly for children and newborns, as well as hematological defects which might cause other autoimmune disorders. Case presentation: We present a case of a 28-year-old male patient admitted to the hospital with a history of persistent high grade fever for two weeks, right lower limb swelling three weeks prior to admission, and right side weakness for one month. Aside from being addicted to cannabis for three years, the patient has no prior medical history. On admission, the patient was awake, conscious, oriented, and hemodynamically stable. A complete blood count, ultrasound examinations, and a whole-body CT scan revealed that the results were favorable for multiple enlarged lymph nodes and hepatosplenomegaly. The patient was given acyclovir, Vfend, Colistin, Targocid, Tinam, Septrin, and anticoagulant during the follow-up. The patient's clinical condition was rapidly deteriorating; a bone marrow biopsy was performed, which revealed haemophagocytosis; and dexamethasone was started. The patient's clinical condition deteriorated during his hospital stay until he went into cardiac arrest and had to be resuscitated for 30 minutes with pulseless electrical activity. Background and Aim: This case suggests that we should be vigilant to the patient who is admitted to the hospital with symptoms for unknown reasons, in order to diagnose HLH as soon as possible and clarify its cause, and it also puts several theories regarding the pathogenicity of this disease in our hands, which will be described in this case, making this case a subject for discussion and research in the medical field. Conclusion: Haemophagocytic lymphocytosis (HLH) is a severe inflammatory disease that improperly controls the body's immune response. Viruses and bacterial infections are just two examples of the various etiological causes that can cause it. Lab results and symptom presentations that are particular to the primary infection can indicate it. Diagnosis and treatment must be provided as soon as possible in order to reduce morbidity and mortality.

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Forme Fruste of HLH (haemophagocytic lymphohistiocytosis): diagnostic and therapeutic challenges

Cited by 9 publications

References 23 publications

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

A Child With Kawasaki Disease and Coronary Artery Aneurysm During Acute Epstein-Barr Virus Infection

Haemophagocytic lymphocytosis (HLH) vicious cycle in a Cannabinoids addict patient case report and disease review.

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