Intravenous Immunoglobulin Treatment for Macrophage Activation Syndrome Complicating Chronic Granulomatous Disease

Álvarez-Cardona, Aristóteles; Rodríguez-Lozano, Ana Luisa; Blancas-Galicia, Lizbeth; Rivas-Larrauri, Francisco; Yamazaki‐Nakashimada, Marco Antonio

doi:10.1007/s10875-011-9616-5

Cited by 46 publications

(27 citation statements)

References 24 publications

(44 reference statements)

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“…Although dysregulated inflammation can manifest as sterile granulomas and discoid lupus in patients with CGD, all reported cases of HLHwithoverwhelming pathologic immune activation occur in the setting of persistent infection [4][5][6][7][8][9] suggesting that impaired pathogen killing underlies the pathologic hyperactive inflammation. Interestingly, all patients who received intravenous immunoglobulin and pathogen-specific therapies survived, 4,5,7,8 while the reported deaths occurred in patients who received either inadequate pathogen-specific therapy or immunosuppressive therapies for HLH.…”

Section: Discussionmentioning

confidence: 99%

“…Interestingly, all patients who received intravenous immunoglobulin and pathogen-specific therapies survived, 4,5,7,8 while the reported deaths occurred in patients who received either inadequate pathogen-specific therapy or immunosuppressive therapies for HLH. 6,9 Therefore, perhaps in patients with CGD who also have infection-triggered HLH, greater focus on eliminating the infection by immune-enhancing strategies may paradoxically decrease the hyperactive inflammatory response and improve the clinical outcome.…”

Section: Discussionmentioning

confidence: 99%

“…Accordingly, infection-triggered hemophagocytic lymphohistiocytosis (HLH) has been reported in patients with CGD with concurrent infections. [4][5][6][7][8][9] However, there have been no reports of HLH as the presenting clinical finding leading to the diagnosis of CGD.…”

Section: Introductionmentioning

confidence: 99%

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Chronic Granulomatous Disease Presenting as Hemophagocytic Lymphohistiocytosis: A Case Report

et al. 2014

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Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent infections and a dysregulated inflammatory response. Infection-triggered hemophagocytic lymphohistiocytosis (HLH), which manifests itself as pathologic hyperactive inflammation, has been observed in subjects with CGD. However, there have been no reports of HLH as the initial presentation with subsequent diagnosis of CGD. Furthermore, the primary therapeutic strategy for HLH focuses on immunosuppressive therapies, which limits immune-mediated tissue damage. With immunodeficiency, this therapeutic strategy may worsen the outcome. This article discusses an 8-week-old Hispanic male who presented with fever of unknown origin. The initial diagnostic evaluation demonstrated pathologic hyperactive inflammation, meeting the HLH-2004 diagnostic criteria without an identified infectious etiology. Immunosuppressive therapy was initiated, with subsequent disseminated candida septic shock and sepsis-induced multisystem organ failure. Additional evaluations ultimately established the diagnosis of CGD. We transitioned to an immune-enhancing strategy with granulocyte and immunoglobulin infusions, and intensified antifungal therapies. These interventions ultimately led to the clearance of the fungal infection and the resolution of the hyperactive inflammatory state. This case represents the first reported case of HLH as the presenting finding leading to the subsequent diagnosis of CGD. It serves as a reminder that both immunodeficiency and inflammatory disorders may share features of pathologic hyperactive inflammation and highlights the conundrum that clinicians face when treating HLH in the setting of an unresolved infection. In this case report, we demonstrate that immune-enhancing therapies may aid in the control and the clearance of the infection, thus paradoxically decreasing the pathologic hyperactive inflammatory response.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Chronic Granulomatous Disease Presenting as Hemophagocytic Lymphohistiocytosis: A Case Report

et al. 2014

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“…Although the association of CGD with Bcc infection is well known, only few case reports have described the presence of HLH [11–14] (Table 3) during the course of Bcc infection in CGD patients. Furthermore, although other medical conditions could lead to infection with Bcc [7], we are not aware of any report of HLH during Bcc infection in absence of CGD.…”

Section: Discussionmentioning

confidence: 99%

Undiagnosed Chronic Granulomatous Disease,Burkholderia cepacia complexPneumonia, and Acquired Hemophagocytic Lymphohistiocytosis: A Deadly Association

Maignan

Verdant

Bouvet

et al. 2013

Case Reports in Pulmonology

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Background. Chronic granulomatous disease is a rare inherited disorder of the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. The clinical course of the disease is marked by recurrent infections, including Burkholderia cepacia complex infection. Case Report. Here we report the case of a 21-year-old male hospitalized for a Burkholderia cepacia complex pneumonia. Despite the broad spectrum antibiotic treatment, fever continued and patient's condition worsened. Anemia and thrombocytopenia developed together with hypofibrinogenemia. The patient died of multiple organ dysfunction 17 days after his admission. Autopsy revealed hemophagocytosis, suggesting the diagnosis of acquired hemophagocytic lymphohistiocytosis. DNA analysis showed a deletion in the p47phox gene, confirming the diagnosis of autosomal recessive chronic granulomatous disease. Discussion. In addition to chronic granulomatous disease, recent findings have demonstrated that Burkholderia cepacia complex can decrease activity of the NADPH oxidase. Interestingly, hemophagocytic lymphohistiocytosis is characterized by an impaired function of the T-cell mediated inflammation which is partly regulated by the NADPH oxidase. Physicians should therefore pay particular attention to this deadly association.

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“…Indeed, all of the published cases of MAS developing in CGD patients have been associated with microbial infections such as Burkholderia cepacia, Staphyloccocus epidemidis, Leishmania, and fungi at the onset of MAS. [9][10][11] In contrast, there have been no reports of MAS developing in patients with clinically significant CGDassociated bowel inflammation, although the patients also have persistent hyperinflammation and are at risk for MAS during the clinical course of the disease, similar to patients with sJIA.…”

mentioning

confidence: 99%

A Case of Macrophage Activation Syndrome Developing in a Patient With Chronic Granulomatous Disease-associated Colitis

Akagi

Kawai

Watanabe

et al. 2014

Journal of Pediatric Hematology/Oncology

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Although macrophage activation syndrome (MAS) develops in some patients with chronic granulomatous disease (CGD), all of the reported cases have been associated with pathogenic microbial infections. We report a 2-year-old boy with CGD-associated colitis who suffered from MAS without any clinical signs of a microbial infection. He was treated with 1 course of methylprednisolone pulse therapy and the clinical symptoms improved; however, the colitis was difficult to control even with immunosuppressive drugs, and he eventually required hematopoietic stem cell transplantation 1 year after the onset of MAS. It is likely that MAS develops in patients with CGD colitis independent of microbial infections.

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Intravenous Immunoglobulin Treatment for Macrophage Activation Syndrome Complicating Chronic Granulomatous Disease

Abstract: The exaggerated inflammatory response characteristic of CGD patients could play a role in the development of this complication. IVIG appears to be a safe and effective first line treatment in these patients.

Cited by 46 publications

References 24 publications

Chronic Granulomatous Disease Presenting as Hemophagocytic Lymphohistiocytosis: A Case Report

Chronic Granulomatous Disease Presenting as Hemophagocytic Lymphohistiocytosis: A Case Report

Undiagnosed Chronic Granulomatous Disease,Burkholderia cepacia complexPneumonia, and Acquired Hemophagocytic Lymphohistiocytosis: A Deadly Association

A Case of Macrophage Activation Syndrome Developing in a Patient With Chronic Granulomatous Disease-associated Colitis

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