[T-cell rich B-cell lymphoma associated with hemophagocytic syndrome].
Report of a T-cell rich B-cell lymphoma (TCRBCL) in a 43 years old man with an associated haemophagocytic syndrome (HS). At presentation the haemophagocytic cells involved the same organs as the lymphoma, i.e. spleen, liver, abdominal lymph nodes and bone marrow. As supportive measure to alleviate chemotherapy-induced granulocytopenia the cytokine granulocyte-macrophage colony-stimulating factor (GM-CSF) was given. After an initial improvement of the blood granulocyte count pancytopenia developed again, resulting in fatal sepsis. Autopsy demonstrated massive proliferation of macrophages in the bone marrow with haemophagocytosis as morphological correlation to the pancytopenia. The observation that exogenous GM-CSF enhanced the preexistent HS primarily reactive to the TCRBCL raises the question if endogenous GM-CSF may play a role in triggering a HS. The observed association of TCRBCL and HS has not been reported so far.
D000328:Adult; D000380:Agranulocytosis / Q000139:chemically induced / Q000628:therapy; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D001344:Autopsy; D001761:Bleomycin / Q000008:administration & dosage / Q000009:adverse effects; D001853:Bone Marrow / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects; D004317:Doxorubicin / Q000008:administration & dosage / Q000009:adverse effects; D005047:Etoposide / Q000008:administration & dosage / Q000009:adverse effects; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use; D006684:HLA-DR Antigens / Q000032:analysis; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000473:pathology*; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008264:Macrophages / Q000473:pathology; D008297:Male; D011241:Prednisone / Q000008:administration & dosage / Q000009:adverse effects; D013160:Splenic Neoplasms / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D014750:Vincristine / Q000008:administration & dosage / Q000009:adverse effects
D015703:Antigens, CD; D006684:HLA-DR Antigens; D001761:Bleomycin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D016178:Granulocyte-Macrophage Colony-Stimulating Factor; D003520:Cyclophosphamide; D011241:Prednisone
T-Zell reiches B-Zell Lymphom assoziiert mit hämophagozytischem Syndrom.