Part III • White Cell Disorders with fibrotic material and liquid marrow is unable to be aspirated. A key item in the diagnosis of HCL is the cyto-chemical stain known as TRAP, or tartrate-resistant acid phosphatase stain. Most lymphocytes contain many isoenzymes, and isoenzyme 5 is especially abundant in hairy cells.8 When blood smears from patients with HCL are stained with the acid phosphatase, most cells will take up the stain. Once tartrate is added, lymphocytes from HCL patients will remain stained while the staining in other cells will fade. This resistance to tartrate is directly related to the level of isoenzyme 5 activity in hairy cells. CD markers present in hairy cells are CD22, CD11c, CD25, and CD103 (Table 13.2).

Treatment for patients with HCL is individualized according to the progress and course of disease. Therapeutic splenectomy will provide an improvement in cytopenias and hypersplenism, as well as providing an improvement in physical symptoms such as abdominal fullness and satiety. Other treatments with interferonalfa and 2-chlorodeoxyadenosine (2-CdA) have offered positive remissions.9

Sézary Syndrome

T-cell lymphomas may present with a cutaneous manifestation in some patients. The name for this is mycosis fungoides. Individuals with mycosis fungoides will show reddened itchy areas (generalized erythroderma) that become thickened, scaly, and pronounced. Skin biopsies of these areas will show an infiltration of lymphocytes. As this disease progresses, the spleen, bone marrow, and lymph nodes become involved, presenting the characteristic Sézary syndrome, which is the leukemic phase of T-cell lymphoma. Sézary cells can be identified in the peripheral blood as large cells, approximately 8 to 20 pm, with a convoluted, cerebriform, ovoid nucleus. Although they may be mistaken for monocytes, the concentration of chromatin is much thicker and more compact in Sézary cells. Sézary cells are pathognomonic for cutaneous T-cell lymphoma

Figure 13.4 Sezary cells. Note the folded or convoluted nuclear membrane that may appear cerebriform.

Table 13.2

CD Markers in Hairy Cell Leukemia

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