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Table 8-1. Clinical Stigmata of Hairy Cell Leukemia

Clinical Evaluation Common Splenomegaly (90 percent) Circulating hairy cells (90 percent) Pancytopenia (70 percent) Infection (70 percent) Bacterial, fungal and viral infections Unique susceptibility to atypical mycobacteria Less Common Hepatomegaly (35 percent) Lymphadenopathy (25 percent)

Osteolytic lesions, autoimmune disease, vasculitis—very rare Bone Marrow Examination Positive staining for tartrate-resistent acid phosphatase (TRAP) Dry tap; reticular fibrosis

Diffusely infiltrating mononuclear cells separated by clear cytoplasms giving bone marrow a "fried egg" appearance abnormalities in 19 percent, azotemia in 27 percent, and hypergammaglobulinemia, which may rarely be monoclonal, in 18 percent. Hypogammaglobulinemia, unlike in chronic lympho-cytic leukemia, is rare.

Patients with HCL are susceptible to both gram-positive and gram-negative bacterial infections as well as to atypical mycobacterial disease, particularly Mycobacterium kansasii. The frequency of infection appears to correlate not with prior splenectomy or previous systemic therapy but with the degree of neutropenia and monocytopenia. Nonpyogenic infections with Aspergillus, Histoplasma, Cryptococcus, and Pneumocystis carinii occur with greater frequency among HCL patients. During the follow-up of 127 patients with HCL, 47 had culture-proven infections, 40 had clinically significant infectious episodes without positive culture documentation, and 40 had no infectious complications.45

Rarely, HCL may occur in association with immunologic disorders such as polyarthritis nodosa and leukocytoclastic vasculitis. Skin infiltration has been described.46'47 Bone involvement may present with diffuse osteoporosis or, more commonly, as lytic lesions involving the axial skeleton, particularly the femoral heads.23'48 Patients with skeletal complications tend to have higher tumor burdens and marrows more diffusely infiltrated by HCL. Patients with chylous and serous ascites, and even pleural effusions, have also been reported.49,50

In a series of 116 patients with HCL followed for over two decades, several unusual presentations and complications were encountered, including spontaneous rupture of the spleen, massive splenomegaly from hairy cell infiltration with normal blood counts and bone marrow evaluations, spinal cord compression with paralysis, protein-losing enteropathy from hairy cell infiltration of the bowel, and esophageal perforation with a fistulous tract.51

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