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Review Question - QID 104663

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QID 104663 (Type "104663" in App Search)
A 66-year old man presents to your clinic with a complaint of abdominal fullness and six months of worsening fatigue, weakness, and easy bruising. On physical exam, vital signs are stable, but you note marked splenomegaly and scattered ecchymoses. Labs are noteworthy for a hematocrit of 25%, total white blood cell count of 3000/uL, and platelet count of 35,000/uL. A peripheral blood smear is shown in Figure A; a tartrate-resistant acid phosphatase (TRAP) assay comes back positive. On further diagnostic workup, a bone marrow aspirate does not yield a sample due to extensive fibrosis. Which of the following is the first-line agent for the treatment of this condition?
  • A

Vincristine

8%

29/359

Rituximab

19%

69/359

Cyclophosphamide

11%

40/359

Cladribine

48%

173/359

Methotrexate

11%

39/359

  • A

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This clinical presentation is consistent with hairy cell leukemia; first-line treatment for hairy cell leukemia consists of cladribine, an adenosine deaminase inhibitor.

Hairy cell leukemia is an uncommon chronic B cell lymphoproliferative disorder, which typically affects older adults, and is more common in men. Patients commonly present with symptoms related to massive splenomegaly and cytopenias. Diagnosis of hairy cell leukemia is made on the basis of several factors: recognition of the characteristic hairy leukocyte on blood smear with a “fried egg” appearance; bone marrow that cannot be aspirated due to extensive fibrosis; and a positive tartrate-resistant acid phosphatase (TRAP) assay. Treatment in hairy cell leukemia is indicated for alleviation of symptoms related to splenomegaly and cytopenias; cladribine is considered first-line therapy.

Summers and Jaffe discuss diagnostic criteria for hairy cell leukemia. They point out that certain features of the disease, such as the characteristic villous cytoplasmic projections may be seen in other B-cell lymphoproliferative disorders and are therefore not specific to hairy cell leukemia.

Dearden et al. report data on a 25-year retrospective review of 242 patients with hairy cell leukemia treated with either pentostatin or cladribine for a median follow-up of 16 years and find that there was no significant difference in outcome between the two agents as first or subsequent lines of therapy.

Figure A depicts a characteristic circulating hairy leukocyte from a peripheral blood smear. It is a mononuclear cell, usually one to two times the size of a mature lymphocyte, with a cytoplasmic outline that is often indistinct with varying projections. Illustration A shows a patient with massive splenomegaly, which is a common presenting sign in hairy cell leukemia.

Incorrect Answers:
Answers 1-3, 5: While these are all agents used in the treatment of various malignancies, they are not considered first-line therapy for hairy cell leukemia.

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