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Macrophage activation syndrome in a patient with pulmonary inflammatory myofibroblastic tumour.

Kuppe C, Westphal S, Bücher E, Moeller MJ, Heintz B, Schneider ME, Floege J - Allergy Asthma Clin Immunol (2012)

Bottom Line: The patient was admitted to the intensive care unit with a history of prolonged remitting fever, hepatosplenomegaly, bilaterally enlarged thoracic lymph nodes and an acute severe inflammatory response syndrome (SIRS).Up-regulated cytokine production (e.g. IL-1ß and IL-6), increased levels of ferritin and circulating soluble interleukin-2 receptor (sIL-2R, sCD25) led to the differential diagnosis of MAS.Immunosuppressive therapy with corticosteroids and cyclosporine was an effective treatment in this patient.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Nephrology and Clinical Immunology, University Hospital of the Aachen University of Technology (RWTH), Aachen, Germany. ckuppe@ukaachen.de.

ABSTRACT
We describe for the first time a case of macrophage activation syndrome (MAS) in a patient with a history of inflammatory myofibroblastic tumour (inflammatory pseudotumour, IPT) of the lung and thoracic spine. The patient was admitted to the intensive care unit with a history of prolonged remitting fever, hepatosplenomegaly, bilaterally enlarged thoracic lymph nodes and an acute severe inflammatory response syndrome (SIRS). Up-regulated cytokine production (e.g. IL-1ß and IL-6), increased levels of ferritin and circulating soluble interleukin-2 receptor (sIL-2R, sCD25) led to the differential diagnosis of MAS. Bone marrow aspiration, the main tool for a definite diagnosis, revealed macrophages phagocytosing haematopoietic cells. Immunosuppressive therapy with corticosteroids and cyclosporine was an effective treatment in this patient.

No MeSH data available.


Related in: MedlinePlus

Aspects of the pseudoinflammatory tumour of the lung in the patient. ( A) A chest radiography revealed a mass in projection of the upper left lung lobe. ( B) A computed tomography of the lung revealed a mass in the left lobe of the lung in contact with the thoracic spine. ( C- D) Histological analysis revealed the diagnosis of a pseudoinflammatory tumour, with the characteristic spindle-like cell structure of fibroblasts.
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Figure 1: Aspects of the pseudoinflammatory tumour of the lung in the patient. ( A) A chest radiography revealed a mass in projection of the upper left lung lobe. ( B) A computed tomography of the lung revealed a mass in the left lobe of the lung in contact with the thoracic spine. ( C- D) Histological analysis revealed the diagnosis of a pseudoinflammatory tumour, with the characteristic spindle-like cell structure of fibroblasts.

Mentions: The patient, a 27 year-old male, presented initially to his general practitioner because of increasing back pain. As an electrical mechanic he traveled extensively from Germany to China, Russia and South Korea and was mainly involved in the installation of electronic devices in newly built tunnels. Due to back pain and an evolving neurological deficit of the lower extremities, the patient was admitted to an external orthopaedic department. A magnetic resonance imaging (MRI) of the spine revealed an atypical tumour in the left upper lung lobe encasing the vertebrae Th3-Th4. A computerized tomography (CT) of the thorax revealed a mass of 3.9 cm x 2.9 cm in the left upper lung lobe (Figure1A, B, arrows). Histological examination of a biopsy of the mass showed an IPT with large amounts of histiocytes, lymphocytes and plasma cells (Figure1C, D). After neurosurgical therapy, the neurological deficit in the lower extremities improved and the patient was discharged. In a second operation, the lung tumour was removed. Histological examination of this material also revealed an IPT. Given the extensive travel history of the patient, various infectious agents were excluded (mycobacteria, legionella, Borrelia burgdorferi, pneumocystis, Epstein-Barr virus (EBV), herpes simplex virus, human immunodeficiency virus-1/2, parvovirus, respiratory viruses, cytomegalovirus (CMV) and other bacteria, stool pathogens and urinary histoplasma antigen). During the following two years, the patient was intermittently treated with corticosteroids, and low-dose radiation therapy was performed in order to control tumour growth locally, as recommended previously [10].


Macrophage activation syndrome in a patient with pulmonary inflammatory myofibroblastic tumour.

Kuppe C, Westphal S, Bücher E, Moeller MJ, Heintz B, Schneider ME, Floege J - Allergy Asthma Clin Immunol (2012)

Aspects of the pseudoinflammatory tumour of the lung in the patient. ( A) A chest radiography revealed a mass in projection of the upper left lung lobe. ( B) A computed tomography of the lung revealed a mass in the left lobe of the lung in contact with the thoracic spine. ( C- D) Histological analysis revealed the diagnosis of a pseudoinflammatory tumour, with the characteristic spindle-like cell structure of fibroblasts.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3473307&req=5

Figure 1: Aspects of the pseudoinflammatory tumour of the lung in the patient. ( A) A chest radiography revealed a mass in projection of the upper left lung lobe. ( B) A computed tomography of the lung revealed a mass in the left lobe of the lung in contact with the thoracic spine. ( C- D) Histological analysis revealed the diagnosis of a pseudoinflammatory tumour, with the characteristic spindle-like cell structure of fibroblasts.
Mentions: The patient, a 27 year-old male, presented initially to his general practitioner because of increasing back pain. As an electrical mechanic he traveled extensively from Germany to China, Russia and South Korea and was mainly involved in the installation of electronic devices in newly built tunnels. Due to back pain and an evolving neurological deficit of the lower extremities, the patient was admitted to an external orthopaedic department. A magnetic resonance imaging (MRI) of the spine revealed an atypical tumour in the left upper lung lobe encasing the vertebrae Th3-Th4. A computerized tomography (CT) of the thorax revealed a mass of 3.9 cm x 2.9 cm in the left upper lung lobe (Figure1A, B, arrows). Histological examination of a biopsy of the mass showed an IPT with large amounts of histiocytes, lymphocytes and plasma cells (Figure1C, D). After neurosurgical therapy, the neurological deficit in the lower extremities improved and the patient was discharged. In a second operation, the lung tumour was removed. Histological examination of this material also revealed an IPT. Given the extensive travel history of the patient, various infectious agents were excluded (mycobacteria, legionella, Borrelia burgdorferi, pneumocystis, Epstein-Barr virus (EBV), herpes simplex virus, human immunodeficiency virus-1/2, parvovirus, respiratory viruses, cytomegalovirus (CMV) and other bacteria, stool pathogens and urinary histoplasma antigen). During the following two years, the patient was intermittently treated with corticosteroids, and low-dose radiation therapy was performed in order to control tumour growth locally, as recommended previously [10].

Bottom Line: The patient was admitted to the intensive care unit with a history of prolonged remitting fever, hepatosplenomegaly, bilaterally enlarged thoracic lymph nodes and an acute severe inflammatory response syndrome (SIRS).Up-regulated cytokine production (e.g. IL-1ß and IL-6), increased levels of ferritin and circulating soluble interleukin-2 receptor (sIL-2R, sCD25) led to the differential diagnosis of MAS.Immunosuppressive therapy with corticosteroids and cyclosporine was an effective treatment in this patient.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Nephrology and Clinical Immunology, University Hospital of the Aachen University of Technology (RWTH), Aachen, Germany. ckuppe@ukaachen.de.

ABSTRACT
We describe for the first time a case of macrophage activation syndrome (MAS) in a patient with a history of inflammatory myofibroblastic tumour (inflammatory pseudotumour, IPT) of the lung and thoracic spine. The patient was admitted to the intensive care unit with a history of prolonged remitting fever, hepatosplenomegaly, bilaterally enlarged thoracic lymph nodes and an acute severe inflammatory response syndrome (SIRS). Up-regulated cytokine production (e.g. IL-1ß and IL-6), increased levels of ferritin and circulating soluble interleukin-2 receptor (sIL-2R, sCD25) led to the differential diagnosis of MAS. Bone marrow aspiration, the main tool for a definite diagnosis, revealed macrophages phagocytosing haematopoietic cells. Immunosuppressive therapy with corticosteroids and cyclosporine was an effective treatment in this patient.

No MeSH data available.


Related in: MedlinePlus