Platelet-specific hemophagocytosis in a patient with juvenile dermatomyositis

Kobayashi, I.

doi:10.1080/080352500750027989

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…There has been no report about the cytokine profile in JDM-MAS with IP. To our knowledge, only 5 patients with JDM-MAS were reported [ 4 , 7 – 10 ]. There were 4 boys and 1 girl, the median age of whom was 12 years ranging from 7 to 14.…”

Section: Discussionmentioning

confidence: 99%

Successful control of juvenile dermatomyositis-associated macrophage activation syndrome and interstitial pneumonia: distinct kinetics of interleukin-6 and -18 levels

et al. 2015

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BackgroundMacrophage activation syndrome (MAS) is the secondary hemophagocytic lymphohistiocytosis associated with rheumatic diseases. Recently, the different cytokine profiles between systemic juvenile idiopathic arthritis (sJIA)-associated MAS (sJIA-MAS) and juvenile systemic lupus erythematosus (JSLE)-associated MAS (JSLE-MAS) were reported. However, there is little information about juvenile dermatomyositis (JDM)-associated MAS (JDM-MAS).Case presentationA 4-year-old girl with JDM was hospitalized because of fever, erythema, hepatosplenomegaly, cytopenia, liver dysfunction and coagulopathy. Bone marrow aspiration revealed appreciable numbers of activated and hemophagocytosing macrophages. She was diagnosed as having JDM–MAS complicated with interstitial pneumonia (IP) based on the findings of the elevation of serum Krebs von den Lungen-6 (KL-6) levels and chest computed tomography findings. We analyzed circulating levels of interleukin (IL)-2,4,6,10,18, tumor necrosis factor-α and interferon-γ in the patient. Hypercytokinemia occurred at the diagnosis of MAS and IP, showing with the prominent elevations of IL-6 and IL-18 levels. The cytokine profiles were distinct from those reported in patients with sJIA-MAS or JSLE-MAS. High-dose corticosteroid and cyclosporine therapy led to a drastic improvement of MAS with decreased IL-6 levels. Subsequent cyclophosphamide therapy successfully controlled IP, paralleled with the declining pattern of IL-18 and KL-6 levels.ConclusionThis is the first report to describe a successful treatment and the cytokine profile of JDM-MAS and IP. Serum IL-6 and IL-18 levels may be useful for predicting the disease activity of JDM-MAS and IP, respectively.

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Section: Discussionmentioning

confidence: 99%

Successful control of juvenile dermatomyositis-associated macrophage activation syndrome and interstitial pneumonia: distinct kinetics of interleukin-6 and -18 levels

et al. 2015

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“…However, the platelet-specific hemophagocytosis was more prominent than seen in patients with juvenile idiopathic arthritis- or Kawasaki disease-associated HPS. Kobayashi et al 19 reported a boy with juvenile dermatomyositis who showed marked thrombophagocytosis, and suggested that platelet-specific antibody facilitated selective phagocytosis by activated macrophages in the bone marrow. In our patient, high levels of circulating IFN-γ and platelet-associated immunoglobulin G could accelerate thrombophagocytosis by the enhanced expression of FcγR on macrophages and the eat-me signal on platelets, respectively.…”

Section: Discussionmentioning

confidence: 99%

Recurrent atrial fibrillation after high-dose methylprednisolone therapy in a girl with lupus-associated hemophagocytic syndrome

Yamamura

Ohga

Nishiyama

et al. 2011

Lupus

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Hemophagocytic syndrome (HPS) is a serious complication of systemic lupus erythematosus (SLE). A 15-year-old female with lupus-nephritis developed HPS. Bone marrow study showed florid thrombophagocytosis. There was no associated infection. High-dose methylprednisolone therapy ameliorated HPS. However, atrial fibrillation (Af) repeated after the infusion and required direct-current cardioversion. No underlying diseases were found in the heart and endocrine system. Chest roentgenogram and echocardiography were normal. Electrocardiogram showed slightly prolonged PR interval in sinus rhythm. Af occurred at high circulating levels of interferon-γ and interleukin (IL)-10, but not IL-6, IL-2, tumor necrosis factor-α, C-reactive protein or catecholamines. This is the first observation that high-dose corticosteroid induced Af in a case of lupus-HPS. Af is unusual in SLE children without cardiac disease, while conduction defect occurs associated with lupus-myocarditis. Lupus-HPS may be an aggressive SLE subset with cardiac involvement. High-dose corticosteroid infusion controls lupus activity, but could disclose the cardiac stress in lupus-HPS patients.

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“…Thrombocytopenia in HLH patients is most commonly attributed to disseminated intravascular coagulation or is multifactorial. However, prominent marrow thrombophagocytosis (reported rarely in the literature 2,3 ) leads us to hypothesize that this may have also contributed to this patient's persistent thrombocytopenia and PLT refractoriness. The patient was unable to tolerate treatment as his condition rapidly deteriorated, leading to his expiration.…”

mentioning

confidence: 93%

Platelet transfusion refractoriness and thrombophagocytosis in an HIV/AIDS patient with hemophagocytic lymphohistiocytosis

Hudgins

Vergara‐Lluri

et al. 2020

Transfusion

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Platelet-specific hemophagocytosis in a patient with juvenile dermatomyositis

Cited by 5 publications

References 10 publications

Successful control of juvenile dermatomyositis-associated macrophage activation syndrome and interstitial pneumonia: distinct kinetics of interleukin-6 and -18 levels

Successful control of juvenile dermatomyositis-associated macrophage activation syndrome and interstitial pneumonia: distinct kinetics of interleukin-6 and -18 levels

Recurrent atrial fibrillation after high-dose methylprednisolone therapy in a girl with lupus-associated hemophagocytic syndrome

Platelet transfusion refractoriness and thrombophagocytosis in an HIV/AIDS patient with hemophagocytic lymphohistiocytosis

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