Pediatric acute lymphoblastic leukemia complicated by secondary hemophagocytic lymphohistiocytosis

Devecioğlu, Ömer; Anak, Sema; Atay, Didem; Aktan, Pinar; Devecioğlu, Esra; Ozalp, Beril; Sarıbeyoğlu, Ebru

doi:10.1002/pbc.22064

Cited by 19 publications

(9 citation statements)

References 9 publications

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“…Consistent with this, homozygous Yeti mice show marked increases in monocytes in both the bone marrow and spleen. The excessive hematopoiesis observed in Yeti mice is interesting given that some patients with certain leukemic malignancies can develop concurrent HLH or MAS (46–50). Although it is thought that HLH and MAS are byproducts associated with the leukemia, our results suggest that the relationship between HLH-like disorders and myeloproliferative syndromes may be more complicated.…”

Section: Discussionmentioning

confidence: 99%

A Novel Model for IFN-γ–Mediated Autoinflammatory Syndromes

Reinhardt

Liang

Bao

et al. 2015

The Journal of Immunology

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Autoinflammatory disease and hyperinflammatory syndromes represent a growing number of diseases associated with inappropriately controlled inflammation in multiple organs. Systemic inflammation commonly results from dysregulated activation of innate immune cells, and therapeutic targeting of the Interleukin-1 beta (IL-1β)–pathway has been used to ameliorate some of these diseases. Some hyperinflammatory syndromes, however, such as hemophagocytic lymphohistiocytosis and the newly classified proteasome disability syndromes, are refractory to such treatments, suggesting that other factors or environmental stressors may be contributing. In comparing two cytokine reporter mouse strains, we identify interferon gamma (IFN-γ) as a mediator of systemic autoinflammatory disease. Chronically elevated levels of IFN-γ resulted in progressive multi-organ inflammation and two copies of the mutant allele resulted in increased mortality accompanied by myeloproliferative disease. Disease was alleviated by genetic deletion of T-bet. These studies raise the possibility that therapeutics targeting the IFN-γ pathway might be effective in hyperinflammatory conditions refractory to IL-1β-targeted therapies.

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Section: Discussionmentioning

confidence: 99%

A Novel Model for IFN-γ–Mediated Autoinflammatory Syndromes

Reinhardt

Liang

Bao

et al. 2015

The Journal of Immunology

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“…CMV infection has been associated with HPS (CMV-AHS) in different settings. It has been observed in healthy patients [76,77], premature infants [78], patients with inflammatory bowel disease [79,80], rheumatological diseases [81,82], cancer [83] and in transplant recipients [84,85]. In a prospective study of 171 patients undergoing HSCT, 7 (4%) developed HPS with CMV being the most common trigger in 3 cases [86].…”

Section: Other Herpesvirusesmentioning

confidence: 99%

Viral infections associated with haemophagocytic syndrome

Maakaroun

Moanna

Jacob

et al. 2010

Reviews in Medical Virology

163

101

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Haemophagocytic syndrome (HPS) or haemophagocytic lymphohistiocytosis (HLH) is a rare disease caused by a dysfunction of cytotoxic T cells and NK cells. This T cell/NK cell dysregulation causes an aberrant cytokine release, resulting in proliferation/activation of histiocytes with subsequent haemophagocytosis. Histiocytic infiltration of the reticuloendothelial system results in hepatomegaly, splenomegaly, lymphadenopathy and pancytopenia ultimately leading to multiple organ dysfunctions. Common clinical features include high fevers despite broad spectrum antimicrobials, maculopapular rash, neurological symptoms, coagulopathy and abnormal liver function tests. Haemophagocytic syndrome can be either primary, i.e. due to an underlying genetic defect or secondary, associated with malignancies, autoimmune diseases (also called macrophage activation syndrome) or infections. Infectious triggers are most commonly due to viral infections mainly of the herpes group, with EBV being the most common cause. HPS can be fatal if untreated. Early recognition of the clinical presentation and laboratory abnormalities associated with HPS and prompt initiation of treatment can be life saving. HPS triggered by viral infections generally does not respond to specific antiviral therapy but may be treated with immunosuppressive/immunomodulatory agents and, in refractory cases, with bone marrow transplantation.

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“…Other than EBV, the most common herpesviruses associated with HPS are CMV and human herpesvirus-8 3. CMV-associated HPS (CMV-HPS) has been reported both in non-immunocompromised4 and immunocompromised patients, such as patients with inflammatory bowel disease,5 rheumatological diseases,6 cancer7 and transplant patients 8. To the best of our knowledge, only nine cases of CMV-HPS in Japan have been described, and those cases consisted of patients with rheumatological diseases (n=5),9 10 haematological malignancies (n=2),11 diabetes mellitus (n=3, 2 of 3 had rheumatological diseases) and chronic kidney disease (n=1), suggesting a low morbidity rate.…”

Section: Discussionmentioning

confidence: 99%

Evidence for cytomegalovirus-induced haemophagocytic syndrome in a young patient with AIDS

et al. 2013

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A 29-year-old man with HIV infection was referred to our department because of a 1-month history of low-grade fever and fatigue. Bone marrow aspiration and biopsy showed findings consistent with haemophagocytic syndrome (HPS), and immunohistochemical assessment showed cytomegalovirus (CMV) infection. HIV-associated HPS can occur at any stages of HIV disease and requires diverse differential diagnosis. CMV-associated HPS (CMV-HPS) in patients with HIV infection is relatively rare, but the present case showed that the clinicians should consider the possibility of CMV-HPS as a clinical feature of CMV infection.

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Pediatric acute lymphoblastic leukemia complicated by secondary hemophagocytic lymphohistiocytosis

Cited by 19 publications

References 9 publications

A Novel Model for IFN-γ–Mediated Autoinflammatory Syndromes

A Novel Model for IFN-γ–Mediated Autoinflammatory Syndromes

Viral infections associated with haemophagocytic syndrome

Evidence for cytomegalovirus-induced haemophagocytic syndrome in a young patient with AIDS

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