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Selected Publication:
Popper, HH; Zenz, W; Mache, C; Ohlinger, W.
Familial haemophagocytic lymphohistiocytosis. A report of three cases with unusual lung involvement.
Histopathology. 1994; 25(5):439-445
Doi: 10.1111/j.1365-2559.1994.tb00005.x
(- Case Report)
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- Leading authors Med Uni Graz
- Popper Helmuth
- Co-authors Med Uni Graz
- Mache Christoph
- Zenz Werner
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- Abstract:
- Three cases of familial haemophagocytic lymphohistiocytosis are presented with lung infiltration by haemophagocytic histiocytes. In all patients the diagnosis was based on hepatosplenomegaly, thrombocytopenia and anaemia, abnormal increase in triglycerides, ferritin and LDH, hypofibrinogenaemia and lymphohistiocytosis with haemophagocytosis in bone marrow. Two patients died of respiratory failure due to interstitial pneumonia. In these two patients the pneumonia was obscured and misinterpreted by the pathologists. A careful re-examination revealed lymphohistiocytosis and haemophagocytic cells within the lung parenchyma. The third patient showed alveolar wall infiltration by haemophagocytic histiocytes and lymphocytes. In early childhood acute or recurrent interstitial pneumonia should prompt a search for haemophagocytic histiocytes, and familial haemophagocytic lymphohistiocytosis should be included in the differential diagnosis.
- Find related publications in this database (using NLM MeSH Indexing)
- Child, Preschool -
- Female -
- Histiocytosis, Non-Langerhans-Cell - genetics
- Humans - genetics
- Immunohistochemistry - genetics
- Infant - genetics
- Lung - pathology
- Lung Diseases, Interstitial - pathology
- Male - pathology
- Find related publications in this database (Keywords)
- Familial Hemophagocytic Lymphohistiocytosis
- Unusual Interstitial Pneumonia
- Lung Infiltrates