Massive Splenomegaly and Pancytopenia: It’s a Hairy Situation

A 33-year-old previously healthy Middle Eastern male presented to the emergency department with four weeks of progressively worsening fatigue, dyspnea on exertion, night sweats, and a 10-pound weight loss after suffering a self-limiting viral upper respiratory illness. He was found to be profoundly anemic and thrombocytopenic with normal white blood cell count with a lymphocytic predominance. His anemia was refractory to red blood cell transfusions, to which he developed hyperbilirubinemia. A CT scan revealed hepatomegaly and massive splenomegaly associated with multi-station abdominopelvic lymphadenopathy. A peripheral blood smear revealed several lymphocytes with hairy cell features and bone marrow biopsy revealed hypercellularity with interstitial infiltration by mature lymphoid cells. Flow cytometry confirmed the diagnosis of hairy cell leukemia (HCL) and this patient was initiated on cladribine chemotherapy. This case illustrates the uniqueness of this patient presenting within a short time course, at an atypical age, and with uncommon features for HCL including lymphadenopathy, hepatomegaly, and petechial skin rash. This case also highlights an important point regarding the management of severe anemia in the acute setting while undergoing splenic sequestration. His lack of response to red blood cell transfusions highlights the need for more research on the use of transfusions in patients who are not current surgical candidates for splenectomy.