pentostatin and Lymphatic-Diseases

We have investigated the incidence and significance of abdominal lymphadenopathy in hairy cell leukemia (HCL) by routinely CT scanning 88 patients. These included 70 men and 18 women with a median age of 51 years (range 25-83). Abdominal CT scans were performed at diagnosis in 29 patients and in 59 during the course of the disease. Abdominal lymphadenopathy was documented in 25 patients (28%) overall; the incidence of abdominal lymphadenopathy was higher in relapse (56%) than at diagnosis (17%). All patients with lymphadenopathy had bone marrow disease. There is no association with age or sex but lymphadenopathy tends to be more common in patients with long-standing disease (median duration of disease 6 years v one year in those without nodes) and in patients with bulky disease, particularly in the relapse group (splenomegaly/splenectomy in 95% vs 40%). The presence of abdominal nodes is also associated with relative resistance to treatment, with more treatment failures and fewer complete responses seen in this group. Most patients with lymphadenopathy had large, immature-looking hairy cells present in both the bone marrow and lymph nodes, when these were examined. Abdominal lymphadenopathy in HCL is more common than previously recognised, particularly in relapsed patients, and is always associated with active disease. The presence of large hairy cells and the relative resistance to treatment suggest that this phenomenon represents a form of transformation of the disease. Longer follow up is required to confirm this.

Topics: Abdomen; Adult; Aged; Aged, 80 and over; Cladribine; Female; Humans; Incidence; Leukemia, Hairy Cell; Lymphatic Diseases; Male; Middle Aged; Pentostatin; Time Factors; Tomography, X-Ray Computed

Lymphadenopathy is an uncommon finding in hairy cell leukaemia (HCL). We report 12 HCL patients in whom relapse was associated with massive abdominal lymphadenopathy. All but one had long-standing HCL (range 3-25 years; median 10 years); in one it was discovered at presentation. Nine patients had been splenectomized and seven had previously been treated with 2'deoxycoformycin (DCF) and/or alpha-interferon (alpha IFN): three had achieved complete remission and four a partial response. The computerized tomography (CT) scan appearances were similar in all cases with a primary lymph node mass centred around the coeliac axis and involving upper para-aortic and retropancreatic regions. Histology and/or cytology confirmed nodal involvement by HCL in six patients. Large immature hairy cells were seen in both lymph nodes and bone marrow, suggesting a degree of transformation. Nine patients were treated with DCF: one had complete resolution, six responded with 50-90% reduction of the lymphadenopathy, one did not respond and one is still on treatment; alpha-IFN was used concomitantly or sequentially in two of the responders. One responding patient died of sepsis after four injections of DCF. Three patients received either alpha- or beta-IFN alone with no response. One elderly patient was not treated. Abdominal lymphadenopathy could be part of the natural history of HCL and/or may represent a transformation analogous to that seen in other low-grade lymphoproliferative disorders. Routine abdominal CT scanning should be part of the work up of all patients with HCL.

Topics: Adult; Aged; Female; Humans; Interferons; Leukemia, Hairy Cell; Lymphatic Diseases; Male; Middle Aged; Pentostatin; Radiography, Abdominal; Tomography, X-Ray Computed