Second malignancies in hairy cell leukemia

Abstract

Among 172 patients with hairy cell leukemia (HCL) seen at the University of Chicago over a 10-year period, 15 were found to have a second malignancy. Neoplasia of the skin was noted most frequently; there were three cases of basal cell carcinoma, one case of anaplastic squamous cell carcinoma, and one case of malignant melanoma. This was followed in frequency by three cases of carcinoma of the lung. The clinical characteristics of these 15 patients did not differ from those of the general population of patients with HCL. A variety of second hematologic malignant disorders and solid tumors were identified. In one case, the second neoplasm occurred before the diagnosis of HCL; six were diagnosed concurrently; and eight followed the diagnosis of HCL. Since HCL is a well-defined clinicopathologic entity, patients with HCL who exhibit unusual features of the disease should be investigated further for the presence of second malignancies.Cancer 56:1462-1467, 1985. AIRY CELL LEUKEMIA (HCL) is a well-defined clin-H icopathologic entity characterized by the presence of abnormal circulating mononuclear cells with large cytoplasmic projections.' Patients often have initial symptoms related to cytopenias, as well as massive splenomegaly and absent or minimal lymphadenopathy.',' The disease has a chronic course; however, progressive infiltration of bone marrow by hairy cells can occur. Granulocytopenia and monocytopenia are known to predispose patients to a variety of infectious complications.'~~ When cytopenias exist, splenectomy is generally regarded as appropriate initial therapy. ' The origin of hairy cells remains a subject of continued debate. Generally, HCL is considered to be a B-cell neoplasm'; however, hairy cells also have monocytic features7** The occurrence of second malignancies in HCL noted in published reports has led to further questions regarding the etiology of HCL. In addition, the presence of a second malignancy can complicate the management of the patient. In this report, we describe second malignancies in 15 patients with HCL. Materials and MethodsWe reviewed the records of all patients with hairy cell leukemia who were referred to the University of Chicago from 1975 through February 1984. Of 172 patients who were identified, 15 had a malignant disease in addition to HCL. We recorded patient sex, age at diagnosis of HCL, the nature of the second malignancy, the interval to the diagnosis of the second malignancy, the treatment for HCL and the other disease, and the hematologic status of the patients with HCL at the time the second malignancy was diagnosed. When splenectomy was undertaken within I month of the diagnosis of HCL, it was considered as being done at the time of diagnosis. The second malignancy and HCL were considered to be concurrent if they were diagnosed within 12 months of each other. ResultsPatient characteristics, as well as data on the diagnosis and treatment of HCL and the nature of the second malignancy, are summarized in Table 1. In our series, there were 14 men and 1 woman. The ag...

Long-lasting complete remission in patients with hairy cell leukemia treated with 2-CdA: a 5-year survey

“…22 However, the occurrence of a second malignancy has been previously observed even after treatment with Discussion ␣-IFN. 23 In conclusion, this study confirms the efficacy and the relaThe use of ␣-IFN first and more recently of DCF and 2-CdA purine analogs in the treatment of HCL, has dramatically tive safety of 2-CdA in inducing a very high proportion of complete and long-lasting remissions (Figure 1) in HCL improved the clinical course and the prognosis of this disorder. 14 In particular, the latter two agents, although responpatients.…”

Second Cancer Risk in Hairy Cell Leukemia

“…Most of these second neoplasms have been solid tumors and few reports of lymphoid malignancies exist. An estimated incidence of 8.7% of second cancers has been reported in HCL patients, 2 and a possible role of IFN-α in their pathogenesis has also been reported. 3 Our cases were treated with IFN-α and 2-CDA, and achieved morphological as well as clinical remission.…”

Hodgkin's disease in hairy cell leukemia: Phenotypic characterization of neoplastic cells

“…Several cases of HCL and a second malignancy have been reported,33 and are divided into three groups based on the development of associated malignancies before, concurrently with, or after a diagnosis of HCL. 34 The usual associated malignancies are carcinomas, but hematologic neoplasms including a related syndrome such as polycythemia v e~a ,~~ dysmyelopoietic syndrome,36 multiple myeloma^,^^,^^ diffuse "histiocytic" lymp h o m a~,~~,~~ SCzary ~yndrome,~' and HD2' also have been reported. Many of these authors,35'37,38,40,41 however, suggest a common clonal origin of the two neoplasms rather than unrelated primary neoplasms which occurred by chance or due to treatment of the antedating neoplasms and/or immune deficiency ~t a t e .~~,~~ In our case, the hairy cells could not be fully characterized, since the number of hairy cells remaining at the time HD was diagnosed was too small to allow further examinations.…”