Given the patient’s abdominal pain, she was evaluated sonographically as well. It was normal except for a 2-cm ovoid soft-tissue nodule anterior to the head of the pancreas. Thereafter, she was sent for CT scan of the abdomen with contrast showing splenomegaly (Figure 1), which descended nearly to the iliac crest. Adenopathy was found in porta-hepatis and near the pancreas head, and there were some ascites surrounding the rectum.
Now suspect for leukemia or lymphoma, Mrs. C was set up for immediate consult with hematology/oncology. Through the specialist, she underwent further blood testing and bone-marrow biopsy. The bone-marrow aspiration was performed exactly two weeks after first presentation to our office, at the right posterior iliac crest. The hypercellular bone marrow was positive for chronic lymphocytic leukemia.
Chronic lymphocytic leukemia (CLL) is characterized by the accumulation of functionally incompetent lymphocytes. It is the most common leukemia in Western countries.1 There is a direct relationship between its incidence and patient age, and the median age of diagnosis is 70 years.2 The disorder is more common in men than women (ratio 1.7:1), and has a preponderance toward Caucasians over their African and Asian counterparts.3
About a quarter of patients with CLL are asymptomatic and the diagnosis is found incidentally on routine laboratory studies.4 Others may note painless swelling of lymph nodes (most commonly in the cervical, supraclavicular and axillary regions), unintentional weight loss, fever >100.5°F, drenching night sweats and extreme fatigue. Spleno- and hepatomegaly are seen on exam in a minority of patients as well.5 The skin is the most commonly involved non-lymphoid organ (leukemia cutis), presenting as an array of macules, papules, blisters or ulcers.6
Naturally, lymphocytosis is the most prominent lab finding, with numbers reaching more than 100,000 mL possible. Neutropenia, anemia and thrombocytopenia, although commonly seen concurrent with CLL, are usually not severe.
The clinical course of the disease is variable, with survival rates ranging from two years to 20 years, with a median survival of approximately 10 years.7 The Rai system breaks down CLL into five stages starting with stage 0, characterized by lymphocytosis only. In stage I there are also enlarged lymph nodes; stage II features enlarged lymph nodes and hepatomegaly or splenomegaly; stage III notes anemia (hemoglobin <11 g/dL); and stage IV, thrombocytopenia (platelets <100,000/µL).8 The median survival from time of diagnosis is 150 months, 101 months, 71 months and 19 months, for stages 0, I, II, and III and IV combined, respectively.5
“Watchful waiting” is acceptable in lieu of treatment in an asymptomatic patient with CLL. Observation usually consists of quarterly laboratory evaluation and clinical examination. However, if the patient presents in advanced disease, has a high tumor burden, repeated infections or several of the aforementioned symptoms, then chemotherapeutic agents are initiated.
Early stage I CLL may be subject to localized radiation therapy.9 Chemotherapeutic treatment options include such purine analogs as fludarabine (Fludara) and pentostatin (Nipent), such alkylating agents as chlorambucil (Leukeran) and bendamustine (Treanda), and such monoclonal antibodies as rituximab (Rituxan) and alemtuzumab (Campath).
Mrs. C was classified as CLL stage III/IV. She was given rituximab for three days followed by two days of bendamustine. After two cycles, she is in remission. At the time of publication, she is symptomatically improved. She still has mild leukopenia, but with no increase in lymphocytes. CT scan showed resolution of splenomegaly and lymphadenopathy. Quarterly observation will continue.
Michele D. Tinge, MPAS, PA-C, practices at Alton Internal Medicine in Alton, Ill.
- Siegel R, Naishadham D, Jemal A. Cancer statistics, 2012. CA Cancer J Clin. 2012;62:10-29.
- Smith A, Howell D, Patmore R, et al. Incidence of haematological malignancy by subtype: a report from the Haematological Malignancy Research Network. Br J Cancer. 2011;105:1684-1692.
- Hernández JA, Land KJ, McKenna RW. Leukemias, myeloma, and other lymphoreticular neoplasms. Cancer. 1995;75:381-394.
- Hallek M, Cheson BD, Catovsky D, et al. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines. Blood. 2008;111:5446-5456.
- Rai KR, Sawitsky A, Cronkite EP, et al. Clinical staging of chronic lymphocytic leukemia. Blood. 1975; 46:215-234.
- Agnew KL, Ruchlemer R, Catovsky D, et al. Cutaneous findings in chronic lymphocytic anemia. Br J Dermatol. 2004; 150:1129-1135.
- Boggs DR, Sofferman SA, Wintrobe MM, Cartwright GE. Factors influencing the duration of survival of patients with chronic lymphocytic leukemia. Am J Med. 1966;40:243-254.
- Epstein AL, Marder RJ, Winter JN, et al. Two new monoclonal antibodies, Lym-1 and Lym-2, reactive with human B-lymphocytes and derived tumors, with immunodiagnostic and immunotherapeutic potential. Cancer Res. 1987;47:830-840.
- Morrison WH, Hoppe RT, Weiss, LM, et al. Small lymphocytic lymphoma. J Clin Oncol. 1989;7:598-606.