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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

Routine and immunohistochemical stainings of biopsies from cubital veins and bone marrow. ( A- B) Biopsies of cubital veins showed complete loss of normal vein architecture with massive infiltration of lymphocytes (arrows in A), histiocytes (arrows in B), macrophages and fibroblasts. ( C- D) Further immunohistochemical analysis revealed massively increased numbers of proliferating Ki-67+/CD68+ macrophages (arrows in C and D). ( E- G) Bone marrow biopsies of the patient revealed haemophagocytosis, the defining criterion of MAS. Characteristically, the histiocytes showed degenerated and nucleated proerythoblasts within their cytoplasm (arrows E- G).
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Figure 2: Routine and immunohistochemical stainings of biopsies from cubital veins and bone marrow. ( A- B) Biopsies of cubital veins showed complete loss of normal vein architecture with massive infiltration of lymphocytes (arrows in A), histiocytes (arrows in B), macrophages and fibroblasts. ( C- D) Further immunohistochemical analysis revealed massively increased numbers of proliferating Ki-67+/CD68+ macrophages (arrows in C and D). ( E- G) Bone marrow biopsies of the patient revealed haemophagocytosis, the defining criterion of MAS. Characteristically, the histiocytes showed degenerated and nucleated proerythoblasts within their cytoplasm (arrows E- G).

Mentions: After two-and-a-half years, the internal fixator was removed without complications. A few days later, the patient presented to a general practitioner because of dizziness and mild fever. The operation wound appeared normal and laboratory tests revealed no evidence of infection. During the following two weeks, the symptoms increased and the patient was admitted to the hospital. Postoperative spondylodiscitis was ruled out by CT and MRI of the spine. The CT scan revealed enlarged mediastinal lymph nodes and hepatomegaly. Subsequently, he developed SIRS and was admitted to the intensive care unit. Blood cultures, initial bone marrow aspiration as well as a biopsy and transesophageal echocardiography all came back negative or normal. Histologic examination of material obtained during mediastinoscopy as well as a bronchial lavage showed no malignant cells; the CD4/CD8 ratio was not elevated. No granulomas were observed and sarcoidosis thus appeared unlikely. Subsequently, both elbows started to appear swollen and hot. A biopsy ruled out fasciitis but immunohistopathological evaluation showed histiocytic inflammation with CD68-positive foamy histiocytes and absence of CD1a immunoreactivity (excluding a Langerhans cell histiocytosis) (Figure2 A-C). A liver biopsy was performed; the tissue showed no signs of sarcoidosis and only a mild non-specific inflammatory response. Additionally, there was no evidence for a malignant B- or T-cell lymphoma in subclonal tests using semi-nested PCRs of the biopsy material from the lungs. A positron emission tomography (PET) CT scan showed no hypermetabolic region. Finally, histological evaluation of a second bone marrow biopsy showed haemophagocytic histiocytes (Figure2, E-G).


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)

Routine and immunohistochemical stainings of biopsies from cubital veins and bone marrow. ( A- B) Biopsies of cubital veins showed complete loss of normal vein architecture with massive infiltration of lymphocytes (arrows in A), histiocytes (arrows in B), macrophages and fibroblasts. ( C- D) Further immunohistochemical analysis revealed massively increased numbers of proliferating Ki-67+/CD68+ macrophages (arrows in C and D). ( E- G) Bone marrow biopsies of the patient revealed haemophagocytosis, the defining criterion of MAS. Characteristically, the histiocytes showed degenerated and nucleated proerythoblasts within their cytoplasm (arrows E- G).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Routine and immunohistochemical stainings of biopsies from cubital veins and bone marrow. ( A- B) Biopsies of cubital veins showed complete loss of normal vein architecture with massive infiltration of lymphocytes (arrows in A), histiocytes (arrows in B), macrophages and fibroblasts. ( C- D) Further immunohistochemical analysis revealed massively increased numbers of proliferating Ki-67+/CD68+ macrophages (arrows in C and D). ( E- G) Bone marrow biopsies of the patient revealed haemophagocytosis, the defining criterion of MAS. Characteristically, the histiocytes showed degenerated and nucleated proerythoblasts within their cytoplasm (arrows E- G).
Mentions: After two-and-a-half years, the internal fixator was removed without complications. A few days later, the patient presented to a general practitioner because of dizziness and mild fever. The operation wound appeared normal and laboratory tests revealed no evidence of infection. During the following two weeks, the symptoms increased and the patient was admitted to the hospital. Postoperative spondylodiscitis was ruled out by CT and MRI of the spine. The CT scan revealed enlarged mediastinal lymph nodes and hepatomegaly. Subsequently, he developed SIRS and was admitted to the intensive care unit. Blood cultures, initial bone marrow aspiration as well as a biopsy and transesophageal echocardiography all came back negative or normal. Histologic examination of material obtained during mediastinoscopy as well as a bronchial lavage showed no malignant cells; the CD4/CD8 ratio was not elevated. No granulomas were observed and sarcoidosis thus appeared unlikely. Subsequently, both elbows started to appear swollen and hot. A biopsy ruled out fasciitis but immunohistopathological evaluation showed histiocytic inflammation with CD68-positive foamy histiocytes and absence of CD1a immunoreactivity (excluding a Langerhans cell histiocytosis) (Figure2 A-C). A liver biopsy was performed; the tissue showed no signs of sarcoidosis and only a mild non-specific inflammatory response. Additionally, there was no evidence for a malignant B- or T-cell lymphoma in subclonal tests using semi-nested PCRs of the biopsy material from the lungs. A positron emission tomography (PET) CT scan showed no hypermetabolic region. Finally, histological evaluation of a second bone marrow biopsy showed haemophagocytic histiocytes (Figure2, E-G).

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