Granulozytisches Tonsillensarkom bei myelodysplastischem Syndrom

HISTORY AND ADMISSION FINDINGS: A 60-year-old man with anaemia was found to have a myelodysplastic syndrome (MDS) which was classified as refractory anemia with ringsideroblasts (RAEB). Because of severe fever the patient was hospitalized. Waldeyer's throat ring revealed signs of acute inflammation. LABORATORY FINDINGS: Indicated severe anemia and thrombocytopenia. Leucocytes were 8000/ml with a pathological shift to the left (1920/ml blasts). CRP (4.7 g/dl) and BSG (59/91mm) were abnormal. An axial CT of the neck showed a left sided paratonsillar abscess. DIAGNOSIS AND THERAPY: The tonsillitis was treated with antibiotics. Persistent fever up to 39.8 degrees C suggested resistance to therapy. After three weeks the patient developed septicemia and died. Autopsy revealed MDS with excess of blasts in transformation (RAEBT-t). Granulocytic sarcoma was found in the throating where there was no paratonsillar abscess. Generalized miliary tuberculosis was proved at autopsy, explaining the continuous fever resistant to antibiotic treatment. CONCLUSION: Granulocytic sarcoma is rarely associated with myelodysplastic syndrome. Deficiency in the immune system may facilitate the occurrence of miliary tuberculosis.