A 37 year old man was admitted in September 1999 with a two month history of weight loss, symptoms of anaemia, and rectal bleeding. On examination, he appeared pale, underweight, and tachycardic, with petechiae and ecchymosis on the lower limbs. Fundoscopic examination showed bilateral papilloedema and several retinal flame haemorrhages. A seventh left cranial nerve lesion was found. The liver edge was 5 cm below the right costal border. Laboratory studies showed a haemoglobin concentration of 45 g/litre, white blood cell count of 4.1 × 109/litre, platelet count of 50 × 109/litre, and 35% blast cells; some blast cells contained Auer rods. Lactate dehydrogenase was 12 931 IU/litre (normal range, 91–180). Chest radiography showed mediastinal expansion. Computed tomography of the brain showed a solitary hyperdense subdural nodule, 2.9 cm in diameter in the occipital region (fig 1). Computed tomography of the abdomen and pelvis showed a solid mass, 10 cm in diameter, with regular edges and central hypodensity, compatible with necrosis of the right lobe of the liver. The liver–kidney space was occupied by tumour. The left kidney showed a 4 cm tumour mass. The rectum and sigmoid were infiltrated by an irregular 8 cm diameter tumour (fig 2A, B).
Figure 1 Brain computed tomography scan showing a solitary mass at the right occipital region, indicated by a closed arrow.
Figure 2 (A) Abdominal computed tomography scan showing a solid tumour with hypodensities suggestive of necrosis in the hepatorenal space and retroperitoneal cavity, causing a compression effect against the right kidney. (B) (more ...)
Bone marrow aspiration was compatible with AML M2 (fig 3A, B). Flow cytometry showed that the peripheral blood was positive for CD4, CD11, CD13, CD14, CD33, CD45, and HLA-DR. A bone marrow karyotypic study revealed a 8;21 translocation. A percutaneous ultrasound guided biopsy of the mass anterior to the right kidney was done. A touch preparation showed abundant Sudan black positive myeloblasts (some with Auer rods) (fig 3C, D). The cerebral spinal fluid contained Sudan black and myeloperoxidase positive myeloblasts (fig 3E, F).
Figure 3 (A) Low power view of a bone marrow smear (Wright stain) showing a clump of myeloblasts; original magnification, ×40. (B) High power view of a bone marrow smear (Wright stain) (more ...)
The patient received combination chemotherapy and craniospinal radiotherapy, and achieved complete haematological remission, which was maintained for 18 months. Despite an allogeneic bone marrow transplant, he subsequently relapsed and at relapse had myeloid sarcomas in the inferior pole of the right kidney, at the right ureterovesical junction, and the rectum.