Dommann-Scherrer C, Zimmermann D, Hassam S, Odermatt B F, Risti B, Maurer R
Department für Pathologie, Universität Zürich, Stadtspital Triemli.
Verh Dtsch Ges Pathol. 1992;76:122-5.
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.
一名43岁男性患富含T细胞的B细胞淋巴瘤（TCRBCL）并伴有噬血细胞综合征（HS）的病例报告。就诊时，噬血细胞累及与淋巴瘤相同的器官，即脾脏、肝脏、腹部淋巴结和骨髓。作为缓解化疗引起的粒细胞减少的支持措施，给予了细胞因子粒细胞-巨噬细胞集落刺激因子（GM-CSF）。血液粒细胞计数最初改善后，全血细胞减少症再次出现，导致致命的败血症。尸检显示骨髓中巨噬细胞大量增殖，噬血细胞现象与全血细胞减少症存在形态学关联。外源性GM-CSF增强了原本主要对TCRBCL产生反应的HS，这一观察结果引发了内源性GM-CSF是否可能在触发HS中起作用的问题。TCRBCL与HS之间的这种关联迄今尚未见报道。
