Journal of Clinical Oncology, Vol 24, No 15 (May 20), 2006: pp. 2388-2389
© 2006 American Society of Clinical Oncology.
DOI: 10.1200/JCO.2005.03.3571
Paraneoplastic Erythropoietin-Induced Polycythemia Associated With Small Lymphocytic Lymphoma
Abdulwahab J. Al-Tourah,
Peter W.K. Tsang,
Brian F. Skinnider,
Paul J. Hoskins
Division of Medical Oncology, British Columbia Cancer Agency, Division of Hematology, Vancouver General Hospital, Department of Pathology, British Columbia Cancer Agency, and the University of British Columbia, Vancouver, BC, Canada
A 64-year-old man presented with a 3-month history of intermittent abdominal pain with cramps, documented fevers of 39.5C, drenching night sweats, and small lumps on the right side of his neck for 1 year. Past medical history was unremarkable; the patient is a life-long nonsmoker and has no prior history of respiratory or cardiac disease. Physical examination revealed palmar erythema, peripheral lymphadenopathy, and a palpable large central abdominal mass. He had no hepatosplenomegaly. Computed tomography (CT) scan of the abdomen and pelvis revealed extensive retroperitoneal lymphadenopathy, with a normal spleen. Lymph node biopsy and bone marrow examination showed morphologic and immunohistochemical features consistent with small lymphocytic lymphoma. Pretreatment hemoglobin of 19.7 g/dL (normal range, 13.0 to 18.0 g/dL), hematocrit of 59% (normal range, 40% to 50%), with normal platelet and white cell/differential counts. He had a routine CBC 2 years previously that showed a normal hemoglobin and hematocrit at 13.5 g/dL and 40%, respectively. Pretreatment evaluation to identify the cause of the patient's polycythemia was undertaken. Serum erythropoietin (EPO) level was 93.0 mU/mL (normal range, 3.3 to 16.6 mU/mL), and stem cell culture assay revealed no evidence of EPO-independent erythroid colony growth, both findings rule out polycythemia rubra vera (PRV). No other causes of secondary polycythemia were identified; normal CT scan of the head (ruling out cerebellar hemangioblastoma), no evidence of renal, adrenal, or liver tumors. He had no history of hypoxic lung disease and oxygen saturation was 94% on room air. A total of 1,200 mL of whole blood phlebotomy was undertaken in two separate sessions before starting chemotherapy. The postphlebotomy hemoglobin was 16.3 g/dL. The patient was treated with a chemotherapy regimen that included cyclophosphamide, vincristine, prednisone, and rituximab (CVP-R). Despite phlebotomy he developed deep venous thrombosis in his left leg, following a 4-hour car trip. His hemoglobin was 16.4 g/dL and his hematocrit was 49%. Anticoagulation with low-molecular weight heparin was initiated. After two cycles of CVP-R chemotherapy, his hemoglobin returned to normal (13.4 g/dL), with the serum EPO level also rapidly normalizing (12.1 mU/mL), and both remained within normal limits on subsequent measurements (Fig 1; red arrows, phlebotomy; black arrows, cyclophosphamide, vincristine, prednisone, and rituximab therapy). Restaging CT scan of the abdomen following four cycles of CVP-R showed marked response to therapy (Fig 2A and 2B; A: pretreatment; B: after four cycles of therapy).
Polycythemia as a paraneoplastic syndrome has been described in association with certain malignancies (Table 1). 1,2 The diagnosis is usually based on the findings of elevated EPO level, erythrocytosis, normal bone marrow stem cell culture studies, and absence of EPO-independent erythroid colony growth, as well as lack of secondary causes. The most common cause of polycythemia in the setting of non-Hodgkin's lymphoma is PRV.3,4 This association has been reported to occur either concomitantly4 or as a second malignancy in patients with previously diagnosed PRV.3 To the best of our knowledge, paraneoplastic polycythemia secondary to EPO production by small lymphocytic lymphoma has not been previously reported in the literature. We believe it was secondary to the ectopic production of EPO by the lymphoma cells as evidenced by the normalization of EPO serum levels after successful treatment with chemotherapy.
Authors' Disclosures of Potential Conflicts of Interest
The authors indicated no potential conflicts of interest.
REFERENCES
1. Hammond D, Winnick S: Paraneoplastic erythrocytosis and ectopic erythropoietins. Ann NY Acad Sci 230:219-227, 1974[CrossRef][Medline] 2. Valentine WN, Hennessy TG, Lang E, et al: Polycythemia: Erythrocytosis and erythema. Ann Intern Med 69:587, 1968[Abstract/Free Full Text] 3. Heinle EW, Sarasti HO, Garcia D, et al: Polycythemia vera associated with lymphomatous disease and myeloma. Arch Intern Med 118:255-351, 1966[Abstract/Free Full Text] 4. Rizzi R, Liso A, Pannuzio A, et al: Concomitant primary polycythemia vera and follicle centre cell non-Hodgkin lymphoma: A case report and review of the literature. Leuk Lymphoma 43:2217-2220, 2002[CrossRef][Medline]
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