A 67-year-old man with polycythemia vera comes to the physician for a follow-up visit. He complains of mild fatigue and early satiety. He was diagnosed with polycythemia vera 7 years ago and has had no complications or chemotherapy. The patient underwent therapeutic phlebotomy for 6 years but not during the last year. His other medical problems include hypertension and acid reflux disease. His medications include amlodipine, esomeprazole, and low-dose aspirin.
His blood pressure is 150/75 mm Hg, and pulse is 78/min. Physical examination reveals pale conjunctivae. The abdomen is soft and nontender, and the liver span is 12 cm. The spleen is palpable 7 cm below the costal margin. The remainder of the examination is within normal limits.
Laboratory results are as follows:

Which of the following conditions is the most likely diagnosis?

Question

A 67-year-old man with polycythemia vera comes to the physician for a follow-up visit.  He complains of mild fatigue and early satiety.  He was diagnosed with polycythemia vera 7 years ago and has had no complications or chemotherapy.  The patient underwent therapeutic phlebotomy for 6 years but not during the last year.  His other medical problems include hypertension and acid reflux disease.  His medications include amlodipine, esomeprazole, and low-dose aspirin.
His blood pressure is 150/75 mm Hg, and pulse is 78/min.  Physical examination reveals pale conjunctivae.  The abdomen is soft and nontender, and the liver span is 12 cm.  The spleen is palpable 7 cm below the costal margin.  The remainder of the examination is within normal limits.
Laboratory results are as follows:

Which of the following conditions is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_9838_59b1_853d_937656907fa0_MD0002_00 This patient with a long history of polycythemia vera (PV) presents with anemia, decreased need for phlebotomy, and massive splenomegaly, findings highly suspicious for post-PV myelofibrosis. In general, patients with PV are at risk of developing the following: Thrombotic events (DVT, stroke, MI)Post-PV myelofibrosisAcute myeloid leukemia/myelodysplastic syndrome Thrombotic events (DVT, stroke, MI) Post-PV myelofibrosis Acute myeloid leukemia/myelodysplastic syndrome Post-PV myelofibrosis usually occurs 5-10 years after the diagnosis of PV. Patients may be asymptomatic or have signs and symptoms of fatigue, progressive splenomegaly, and anemia in the setting of long-standing PV. Peripheral blood smear in myelofibrosis shows tear drop cells and immature forms. About 95% of patients also have JAK2 mutations. Bone marrow biopsy showing fibrosis confirms the diagnosis. tear drop cells Treatment for post-PV myelofibrosis is similar to that for primary myelofibrosis. The only definitive treatment is allogenic hematopoietic stem-cell transplantation. Patients with severe splenomegaly and debilitating constitutional symptoms can be treated with JAK2 inhibitors (eg, ruxolitinib). (Choice A) Patients with hairy cell leukemia also present with massive splenomegaly and pancytopenia. Given this patient's PV, however, myelofibrosis is more likely. (Choice B) Mild-to-moderate splenomegaly and hepatomegaly can be present in PV. However, this patient's massive splenomegaly, bicytopenia, and normal MCV make myelofibrosis more likely than co-existing iron deficiency as the cause of his anemia. (Choice C) Liver cirrhosis can cause splenomegaly, resulting in anemia and thrombocytopenia due to splenic sequestration. However, this patient's degree of anemia, hepatomegaly, and lack of risk factors for cirrhosis make it unlikely. (Choice E) Patients with splenic marginal-zone lymphoma usually present with massive splenomegaly, lymphocytosis, and cytopenias (eg, hemolytic anemia) related to hypersplenism. There is no association between PV and splenic marginal-zone lymphoma. Educational objective: Myelofibrosis and acute myeloid leukemia/myelodysplastic syndrome are the major long-term complications of polycythemia vera (PV). Patients with post-PV myelofibrosis present with anemia, decreased need for phlebotomy, worsening splenomegaly, and constitutional symptoms. Diagnosis is made by bone marrow biopsy. __________ References: Postpolycythaemic myelofibrosis: frequency and risk factors for this complication in 116 patients. (http://www.ncbi.nlm.nih.gov/pubmed/19604233) A dynamic prognostic model to predict survival in post-polycythemia vera myelofibrosis. (http://www.ncbi.nlm.nih.gov/pubmed/18187660) Emerging drugs for the therapy of primary and post essential thrombocythemia, post polycythemia vera myelofibrosis. (http://www.ncbi.nlm.nih.gov/pubmed/19552608)

A 67-Year-Old Man with Polycythemia Vera Comes to the Physician