Anaplastic neoplasm in a patient with hairy cell leukemia

Abstract

A 63-year-old white man had a history of recurrent pneumonia, pancytopenia, and splenomegaly when the diagnosis of hairy cell leukemia was made on bone marrow biopsy examination. Splenectomy confirmed that diagnosis and his pancytopenia moderately improved. Three years following the diagnosis, the patient developed an upper abdominal mass involving the stomach wall that was found to be an anaplastic "large cell" neoplasm. Palliative radiotherapy was started, but the patient died 2 months later. Cytochemical studies of the anaplastic gastric neoplasm revealed cytoplasmic tartrate resistant acid phosphatase activity. Electron microscopy showed no epithelial differentiation. These observations suggest that the gastric neoplasm represented an evolution of hairy cell leukemia into a more aggressive tumor analogous to the transformation that occurs in other B-cell neoplasms.

Transformation of hairy cell leukemia to high-grade lymphoma: A case report and review of the literature

“…A case of HCL with coexistent anaplastic neoplasm in the stomach demonstrated tartrateresistant acid phosphatase in the anaplastic tumor cells. 7 However, electron microscopic study did not provide convincing evidence of a lymphoid tumor.…”

“…[4][5][6] Three studies provided circumstantial evidence. [7][8][9] Only 2 cases were well documented with immunophenotyping and molecular genetic studies, but opposite conclusions were drawn in terms of the clonal relationship between these 2 tumors. 10, 11 The present report documents a case of HCL that transformed to a high-grade large cell lymphoma.…”

Partial remission of a newly diagnosed diffuse large B‐cell non‐Hodgkin's lymphoma in a hemodialysis patient after administration of immuno‐chemotherapy with rituximab‐CHOP

“…Although HCL carries an increased risk for secondary malignancies (Stewart & Keating, 1982; Sanchez et al. , 2003), we found only 14 previously published cases that describe occurrence of HCL and large B‐cell lymphoma in the same patient (Adler, Shetty & Golomb, 1979; Franssila, 1979; Vardiman, Variakojis & Golomb, 1979; Davis et al. , 1985; Downing et al.…”

“…Most of these reports are inconclusive regarding the question, whether the large cell lymphomas represent a blastic transformation of hairy cells, similar to the process referred to as Richter transformation in chronic lymphocytic leukemia (Armitage, Dick & Corder, 1978; Foucar & Rydell, 1980), or rather an unrelated second malignancy. Interestingly, the diagnosis of HCL preceded the occurrence of aggressive large B‐cell lymphoma by 1–10 years in 10/14 reports (Davis et al. , 1985; Arnalich et al.…”

Second Cancer Risk in Hairy Cell Leukemia

“…In summary, the possible mechanisms to explain the development of second or even tertiary cancers in patients treated for HCL include: presence of cell-mediated immune deficits 4 ; the expression of proto-oncogenes by such patients leading to the transformation and development of second tumors expressing the same proto-oncogenes 7 ; the immunological deficits produced by the treatment modalities including splenectomy 8 ; direct oncogenic defect of agents like IFN-α 3 ; old age further contributing to immune dysfunction 9 ; possibility of genetic components; shared etiological factors and environmental factors; and last but not the least, the observed increase in the incidence of second neoplasm in this group of patients who are immunocompromised because of HCL and thus prone to develop second tumors. If this is the case, then the frequency of second neoplasms in patients with HCL may be even greater in the future with continued improvements in therapy.…”