Abstract
BackgroundPolycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia.Case presentationA middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis. Further morphological analyses of bone marrow and blood smears revealed probable transformation into acute myeloid leukemia. However, there were also cells indicating hairy cell leukemia. Morphological and immunohistochemical analyses later confirmed the presence of hairy cell leukemia in biopsies that had been present for 3 years. Treatment with cladribine temporarily reversed the patient’s neutropenia.ConclusionsHairy cell leukemia may mimic development to myelofibrosis in patients with polycythemia vera.
Highlights
Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia
The BCR-ABL-negative myeloproliferative neoplasms (MPN) polycythemia vera, essential thrombocythemia, and primary myelofibrosis are characterized by excessive production of terminally differentiated and functional blood cells
They clinically overlap, because primary myelofibrosis sometimes presents with thrombocythemia and may be difficult to distinguish from essential thrombocythemia, whereas both essential thrombocythemia and polycythemia vera can progress to myelofibrosis [1]
Summary
The BCR-ABL-negative myeloproliferative neoplasms (MPN) polycythemia vera, essential thrombocythemia, and primary myelofibrosis are characterized by excessive production of terminally differentiated and functional blood cells. We report diagnostic and therapeutic difficulties with a patient presenting with myelofibrosis secondary to polycythemia vera, further development to AML, and hairy cell leukemia. A bone marrow biopsy showed mildly increased total cellularity (55%), increased numbers of megakaryocytes, no significant increase in erythropoiesis or granulopoiesis, normal maturing granulopoiesis, no dysplasia, no increase in CD34-positive cells, and fibrosis grade 1 interpreted as spent phase polycythemia vera. Considering progressive myelofibrosis as the main reason for the bone marrow failure, treatment with thalidomide 50 mg/day and prednisolone 30 mg/day was initiated in August 2014 [10] This was stopped after 1 month, owing to a dental abscess/severe infections and no effect on blood counts.
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