A 60-year-old female with a history of hypertension and hyperl ipidemia presen ts in Decem ber for further evaluation of several months history of general ized fatigue. Patien t denies bleeding. weigh t loss, or changes in appetite. Physical exami nation reveals no jaundice. lymphadenopathy, or organomegaly. Laboratory evaluation shows hemoglobi n of 8.7 g/dL, mean corpuscular volume (MCV) of 1 1 0 FL. leukocyte count of 5,600/J.LL, and platelet count of 1 7LJ ,OOO/J.LL. Peripheral blood smear is shown below.
Patien t is counseled to avoid cold exposure. and on a fol low-up visit 2 months later. her hemoglobi n is 1 0.8 g/dl. In August. patien t presents to the emergency depart- men t with recurrent fa tigue and is found to have a hemoglobin of 7.2 g/dl. Patient denies recent infections . On exami nation. no lymphadenopathy or organomegaly are detec ted . CT scan is performed with no evidence of underlying malignancy.
What is the most appropriate management to control her disease at this time?
- Corticosteroids
- Intravenous immunoglobulins (IVIG)
- Rituximab
- Cyclophosphamide, vincristine, prednisone, rituximab (CVP-R)
- Splenectomy
Medicine
C. Rituximab should be considered the treatment of choice. Although corticosteroids are effective in the management of warm autoimmune hemolytic anemia, they do not have a role in the management of cold agglutinin disease. IVIG infusions are also without significant benefits. Splenec-tomy is unlikely to provide clinical benefit as the spleen is not the site ofhemolysis. Rituximab has been shown to induce remissions either alone or when used in combination with other agents; however, the additional toxicity provided by a combination cytotoxic regimen is not justified at this time. Plasma exchange has been used as a means to rapidly remove the abnormal antibody from the circulation; however, the benefits of this procedure are short lived (Br J Haematol. 2007;138(4):422, Blood. 20 10; 1 16(17):3 1 80, Br J Haematol. 201 1;153(3):309, Blood. 2012;1 19(16):3691).
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