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Intravascular lymphoma can affect almost any organ. Because of the heterogeneity of its manifestations, clinical diagnosis is usually difficult.
A woman of 83 reported three weeks of generalized tiredness, bilateral leg swelling and the development of a lesion on her abdominal wall. Her medical history was unremarkable apart from an umbilical hernia prosthetic mesh repair 4 years previously. On examination, she had pitting oedema of the legs and an irregular superficially spreading violaceous indurated plaque affecting the anterior abdominal wall and both breasts (Figure 1). White cell count was 13.9 × 109/L, haemoglobin 12.4 g/dL, platelet count 220 × 109/L and erythrocyte sedimentation rate 13 mm/h. Serum urea and electrolytes, amylase, liver biochemistry, immunoglobulins, autoimmune antibodies, Ca 125 and carcinoembryonic antigen were within normal limits whereas the serum albumin (25 g/L), arterial oxygen (8.9 kPi) and lactate dehydrogenase (270 iu/L) were deranged. An abdominal ultrasound examination ruled out occlusion of the vena cava or portal vein; in the area of swelling the abdominal subcutaneous tissue appeared oedematous and inflamed. The possibility of hernial mesh infection was considered and the patient was started on antibiotics. However, fine needle aspiration of the periumbilical mass yielded scanty lymphocytes with no bacteria on culture, and the antibiotic treatment was stopped. No malignant cells were seen. A CT scan of the abdomen and pelvis showed considerable oedema of the abdominal wall but was otherwise normal. During her stay in hospital, her serum albumin fell progressively and the oedema spread to affect the rest of her body.
Core biopsies from each breast showed vascular spaces containing malignant cells with associated necrosis and thrombi (Figure 2a). Immunostaining for cytokeratin gave negative results, but staining for common leucocyte antigen was strongly positive in intravascular cells, indicating an intravascular lymphoma (Figure 2b). The malignant cells were negative for CD3 (T-cell marker) and positive for CD20 (B-cell marker)—i.e. the lymphoma was of B-cell type. The patient died six weeks after the onset of her symptoms.
Intravascular lymphoma (IVL) is characterized by uncontrolled proliferation of lymphoid cells within the lumen of capillaries, small veins and arteries. Pfleger and Tappeiner first described it as angioendotheliomatosis proliferans systemisata in 19591. It is a rare disease that affects men and women equally and occurs in the fifth decade of life or later. IVL has protean manifestations because it can affect any organ, but there is a predilection to involve the brain and skin. Cerebral involvement is associated with changes in mental status ranging from confusion to dementia, and focal neurological deficits. The most frequently reported subcutaneous skin changes are nodules or plaques. The nature of the nodules and plaques is highly variable: they are often irregular in shape and can be raised, soft, firm, mobile or indurated. They usually appear as violaceous, haemorrhagic, ecchymotic or hyperpigmented lesions2. Other cutaneous manifestations include a diffuse black discoloration covering 50% of the body surface, plaques with patchy coagulative necrosis and pitting oedema3.
On histological examination, widespread occlusion of small vessels is seen, with malignant mononuclear cells limited to the intravascular spaces. Analysis of surface antigen expression and immunoglobulin gene rearrangement has indicated conclusively that this malignant disorder is lymphoid in origin4. Most tumours are of B-cell lineage although rare cases of T-cell-related IVL have been described.3,5. A noteworthy feature of IVL compared to leukaemia and other forms of malignant lymphomas is that, despite the large number of intravascular tumour cells, these can seldom be identified in peripheral blood smears. Lactate deyhdrogenase and the erythrocyte sedimentation rate tend to be above normal. Cerebrospinal fluid protein is slightly raised.
Because of a low rate of diagnosis in life, data on therapeutic interventions are difficult to analyse. Corticosteroids, chemotheraphy, radiation therapy and plasmapheresis have been tried. Anecdotal data indicate that combination chemotherapy is better than steriod alone or than steriod plus radiotherapy6. Several reports indicate that long disease-free survival is possible with aggressive combination therapy3, but usual survival is only 4-13 months after onset of symptoms5.