Dyspnea, pancytopenia, and splenomegaly
An extensive initial laboratory assessment, including testing for viral disorders, left the diagnosis unsolved.
Adjust treatment to symptoms and severity of pancytopenia
Indications for treatment include the development of one or more the following: ANC <1000/mcL with repeat infections; symptomatic anemia with hemoglobin <11 g/dL; platelet count <100,000/mcL associated with bleeding, symptomatic splenomegaly, or adenopathy; or constitutional symptoms.9
First-line therapy is with one of the cytotoxic agents, either cladribine or pentostatin. Splenectomy is another effective option, with results that last up to 10 years in 50% of cases.9 Another option is interferon-alpha.10
Before the advent of cytotoxic agents, the 4-year survival rate for hairy cell leukemia was reported as 68%. Today, durable remission is attained for many patients. Even after relapse, retreatment yields good responses. The 5-year survival rate now is higher than 85%.11
Close follow-up of these patients is key
Potential complications of hairy cell leukemia include infections, bleeding, anemia, splenic rupture, and a second primary malignancy. There is a 2- to 3-fold increased risk of developing a second malignancy at a median interval of 40 months after initial diagnosis.10
This increased risk may be related to immunosuppression due to hairy cell leukemia or its treatment. The incidence of a second malignancy occurring before the diagnosis of hairy cell leukemia is 10.2%, and concurrently with the diagnosis is 2.6%.10 This finding suggests some pretreatment predisposition to cancer; further studies are being carried out to evaluate this matter. The most common solid tumors include prostate cancer, skin cancer, lung cancer, and gastrointestinal adenocarcinomas.10
Our patient’s outcome
During our patient’s hospital admission, he received 6 units of packed red blood cells, 3 daily injections of 480 mcg subcutaneous filgrastim for 5 days, and ongoing vitamin B12 supplementation to assist myeloid recovery. Upon discharge, his ANC level had increased to 1634/mcL. He began outpatient treatment with cladribine under the care of a hematologist. After almost a year of treatment, our patient is in complete remission.
CORRESPONDENCE
Sheena Boury, MD, Christ Hospital Family Medicine, 830 Thomas More Parkway, Edgewood, KY 41017; sheenaboury@hotmail.com
