A Case Report of Hairy Cell Leukemia and Breast Cancer

Evgeny Mikler, PA-C, Bruce Petersen, MD, John Mascarenhas, MD

Clinical Advances in Hematology & Oncology

July 2013, Volume 11, Issue 7


Evgeny Mikler, PA-C1
Bruce Petersen, MD2
John Mascarenhas, MD1

1Division of Hematology and Medical Oncology, Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY; 2Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY


Hairy cell leukemia (HCL) is a rare, chronic, B-cell lymphoproliferative disorder characterized by pancytopenia, splenomegaly, and constitutional symptoms.1 Although HCL is an indolent disorder, most patients will require treatment at some point. Indications to initiate therapy include disease-related symptoms, signs of bone marrow failure, or frequent infections.2,3 Asymptomatic patients without cytopenias can be observed.2 Therapeutic options usually include purine analogs, either alone or in combination with monoclonal antibodies, biological agents, and surgery.2,3 We report a case of a female who developed HCL years after receiving adjuvant chemotherapy and hormonal therapy for breast cancer.

Case Report

In August 2012, a 65-year-old white woman presented for evaluation of a 14-month history of leukopenia, neutropenia, and monocytopenia, which were initially noticed during a routine complete blood count (CBC) that revealed the following: white blood cell (WBC), 3,000/µL; hemoglobin (Hgb), 12.3 g/dL; hematocrit (Hct), 36.2%; mean corpuscular volume (MCV), 95.8 fL; platelets, 188,000/µL; absolute neutrophil count (ANC), 1,300/µL; and absolute monocyte count (AMC), 200/µL. In 1991, she was diagnosed with estrogen receptor (ER)-positive, progesterone receptor (PR)-positive, human epidermal growth factor receptor 2 (HER2)-negative tubulolobular carcinoma of the left breast. The patient underwent left modified radical mastectomy with reconstruction using a saline implant followed by adjuvant therapy with 6 cycles of cyclophosphamide, methotrexate, and 5-fluorouracil (CMF) plus tamoxifen for 4 years. However, in 2010, she noted pain in the left reconstructed breast that was associated with a firm palpable mass, and biopsy proved positive for recurrent breast cancer. She underwent surgical excision followed by chemotherapy with paclitaxel and carboplatin for 4 cycles, and has since remained on the aromatase inhibitor exemestane (Aromasin, Pfizer).

On physical examination, the patient looked well, with no signs of cachexia, pallor, lymphadenopathy, or organomegaly. Blood studies revealed a WBC of 2,800/µL, Hgb and Hct of 12.0 g/dL and 34.5%, respectively, MCV of 94.0 fL, platelet count of 156,000/µL, ANC of 1,200/µ, and AMC of 100/µL. Review of the peripheral blood smear was notable for the presence of atypical lymphocytes, which had circumferential hairy projections with indented atypical nuclei (Figure1).

Bone marrow biopsy performed in November 2012 demonstrated an infiltrate of atypical small B-lymphocytes (CD20+, CD79a+, and CD5–, by immunohistochemical stains) comprising approximately 60% of overall cellularity, which were associated with grade 2 reticulin fibrosis (modified Bauermeister scale). The aspirate was hemodilute and aspicular. Flow cytometry revealed a small, aberrant monoclonal B-cell population with expression of CD19 (bright), CD20, CD22 (bright), CD25, FMC7 (bright), CD103 (bright), and lambda (bright). Additionally, there was no expression of CD5, CD10, and kappa, which is consistent with HCL. Cytogenetic analysis revealed a normal female karyotype.


HCL accounts for 2% of all adult leukemia cases in the United States.4,5 There are approximately 600 patients diagnosed annually.6 The incidence is 4 times higher in men than in women, and whites are affected more frequently than African Americans.7,8 The median age at the time of diagnosis is 56 years.8 The exact cause is still unknown.

The clinical presentation of HCL can vary. Approximately 25% of patients are asymptomatic. However, the majority of patients complain of generalized fatigue and weakness. They may also have left upper quadrant (LUQ) abdominal pain, frequent infections, bruising, and weight loss. Physical examination may reveal splenomegaly, with or without hepatomegaly and lymphadenopathy. In addition, patients may present with cutaneous manifestations, such as herpes zoster, cellulitis, abscess, pyoderma, dermatophytosis, leukemia cutis, ecchymosis, and purpura.Laboratory findings may reveal leukopenia, neutropenia, monocytopenia, anemia, thrombocytopenia, and hypocholesterolemia. Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) can also be observed.7

Indicators to initiate therapy include fatigue and weakness that interfere with quality of life, symptomatic organomegaly, frequent infections, and signs of bone marrow failure (eg, hemoglobin <10 g/dL, platelets <100,000/µL, and ANC <1,000/µL).2 Symptomatic disease is initially treated with purine analogs, such as cladribine or pentostatin.2,3 Cladribine is considered to be the treatment of choice, as it produces durable overall response rates ranging from 97–100%, with a 4-year disease-free survival rate of over 80%.6,9 Other therapeutic options may include combination therapy with rituximab (Rituxan, Genentech/Biogen Idec), interferon α, and splenectomy.2,3 Complications associated with HCL include increased risk of infections and splenic rupture, with infections being the most common cause of death.7 We plan to observe our patient without therapeutic intervention until she develops symptoms or worsening cytopenias.


The incidence of HCL in breast cancer patients treated with chemotherapy and hormonal therapy has not been well described in the literature. Alkylating agents, antimetabolites, taxanes, and topoisomerase II inhibitors are known to cause secondary myelodysplastic syndromes (MDS) and acute leukemia, but an association with HCL has not been reported.10-16 As such, the possibilities for the etiology of this patient’s HCL are either de novo or related to prior treatment.17,18 In conclusion, we report the first case of HCL in a patient treated with chemotherapy and hormonal therapy for breast cancer. We raise the possibility of prior therapy-related HCL in this individual.


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