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Osteolytic bone lesions

3%

Autoimmune diseases

rare

least 5 cm below the left costal margin in approximately 60% of patients.8 Early satiety or abdominal fullness caused by splenomegaly is present is 25% of patients at diagnosis.5

Hepatomegaly, rarely a significant finding, is present in 20% of patients. Unlike many other lympho-proliferative disorders, peripheral adenopathy is uncommon at diagnosis, with fewer than 10% of patients having lymph nodes larger than 2 cm. However, with the advent of computed tomography and other diagnostic imaging modalities, significant internal adenopathy can be demonstrated in about 30% of patients. Although not common at diagnosis, internal lymphadenopathy may develop after a prolonged disease course9 and is present in 75% of patients at autopsy.10 Such characteristic localization of HCL is likely due to the expression of the integrin receptor, a4pi, by the hairy cells and its interaction with the vascular adhesion molecule-1 (VCAM-1) found on splenic and hepatic endothelia, bone marrow, and splenic stroma.11

Spontaneous splenic rupture, spinal cord compression with paralysis due to neuronal infiltration by leukemic cells, protein-losing enteropathy from bowel infiltration by leukemic cells, esophageal perforation, uveitis, serous and chylous ascites, and pleural and pericardial effusion have been described in patients with HCL.12-14

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