Limits...
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

Flow cytometric analysis of bone-marrow cells and morphology of antigen-presenting cells (APC) from long-term-cultures. ( A) NKT (CD3+/CD56+ cells) were no longer present in the bone marrow of the patient. (B- C) Images show Giemsa-stained cytospin preparations of 28-day-cultured haemophagocytes with characteristic autophagy vacuoles and inclusion bodies containing undigested phagocytozed material (arrows). ( D- I) Further FACS analysis revealed a high expression of CD63 on all BMCs and also an increased expression of CD11b on granulocytes.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3473307&req=5

Figure 3: Flow cytometric analysis of bone-marrow cells and morphology of antigen-presenting cells (APC) from long-term-cultures. ( A) NKT (CD3+/CD56+ cells) were no longer present in the bone marrow of the patient. (B- C) Images show Giemsa-stained cytospin preparations of 28-day-cultured haemophagocytes with characteristic autophagy vacuoles and inclusion bodies containing undigested phagocytozed material (arrows). ( D- I) Further FACS analysis revealed a high expression of CD63 on all BMCs and also an increased expression of CD11b on granulocytes.

Mentions: Flow cytometric analysis revealed the absence of NKT cells and an increased expression of CD163 on all bone marrow cells. CD163, a receptor for haemoglobin-haptoglobin complexes, is involved in clearance and endocytosis of haemoglobin/haptoglobin complexes by macrophages and may thereby protect tissues from free haemoglobin-mediated oxidative damage. The plasma levels of soluble CD163 in HLH are considerably higher than those found in infections or autoimmune diseases [11]. In the peripheral blood, we also found a slight increase of the relative amount of B-lymphocytes as well as an increased expression of CD63 (degranulation marker) and CD11b (migration marker on neutrophils) on bone marrow cells (Figure3, A-I).


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)

Flow cytometric analysis of bone-marrow cells and morphology of antigen-presenting cells (APC) from long-term-cultures. ( A) NKT (CD3+/CD56+ cells) were no longer present in the bone marrow of the patient. (B- C) Images show Giemsa-stained cytospin preparations of 28-day-cultured haemophagocytes with characteristic autophagy vacuoles and inclusion bodies containing undigested phagocytozed material (arrows). ( D- I) Further FACS analysis revealed a high expression of CD63 on all BMCs and also an increased expression of CD11b on granulocytes.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Flow cytometric analysis of bone-marrow cells and morphology of antigen-presenting cells (APC) from long-term-cultures. ( A) NKT (CD3+/CD56+ cells) were no longer present in the bone marrow of the patient. (B- C) Images show Giemsa-stained cytospin preparations of 28-day-cultured haemophagocytes with characteristic autophagy vacuoles and inclusion bodies containing undigested phagocytozed material (arrows). ( D- I) Further FACS analysis revealed a high expression of CD63 on all BMCs and also an increased expression of CD11b on granulocytes.
Mentions: Flow cytometric analysis revealed the absence of NKT cells and an increased expression of CD163 on all bone marrow cells. CD163, a receptor for haemoglobin-haptoglobin complexes, is involved in clearance and endocytosis of haemoglobin/haptoglobin complexes by macrophages and may thereby protect tissues from free haemoglobin-mediated oxidative damage. The plasma levels of soluble CD163 in HLH are considerably higher than those found in infections or autoimmune diseases [11]. In the peripheral blood, we also found a slight increase of the relative amount of B-lymphocytes as well as an increased expression of CD63 (degranulation marker) and CD11b (migration marker on neutrophils) on bone marrow cells (Figure3, A-I).

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