Periostitis associated with myelofibrosis

Abstract

Two patients with myelofibrosis developed fever, leg pain and periostitis. The first patient had myelofibrosis with myeloid metaplasia and was symptomatic for months before x-rays showed periosteal new bone formation in the lower extremities. He subsequently developed periostitis of both upper extremities. Radiation of the lower extremities resulted in significant pain relief. The second patient had a past history of polycythemia Vera and experienced painful periostitis of the tibiae and fibulae. 9mTechnetium pyrophosphate bone scans showed increased uptake in the involved bones in both patients. Asymptomatic or painful periostitis may be related to the increased bone blood flow associated with myelofibrosis. Radiation can afford successful palliation in the severely symptomatic patient. We recently observed two patients with myelofibrosis w h o also suffered f r o m fever, leg pain, a n d periostitis. O n e of these patients had diffuse periostitis of all the long bones t h a t resembled typical hypertrophic osteoarthropathy. CASE REPORTS Case 1A 56-year-old white man with myelofibrosis was admitted to the Washington Veterans Administration Hospital in August 1975 with fever and severe pain in the knees and shins. Eight months earlier, a bone marrow biopsy at another hospital revealed myelofibrosis. His course had been marked by unrelenting fever, anemia, thrombocytopenia, and gastrointestinal bleeding. He was hospitalized for The authors thank Dr. Jacquline Wheng-Peng for chromosome analysis of case 1 and Dr. F. P. Alepa for helpful review of the x-rays and the manuscript.Accepted for publication May 22, 1978. nearly 5 months receiving antibiotics, prednisone, anabolic steroids, and multiple blood and platelet transfusions. Physical examination revealed minimal splenomegaly and a loud murmur of aortic stenosis. T h e lower extremities were tender and warm with trace edema. T h e peripheral blood smear showed anisocytosis, poikilocytosis, tear-drop cells, nucleated red blood cells, myelocytes, occasional myeloblasts, and bizarre megathrombocytes. Bone marrow biopsy confirmed the diagnosis of myelofibrosis. Xrays of the hands, legs and feet were normal.Because of the persistent fever and modest spleen size, the patient underwent an exploratory laparotomy and splenectomy. T h e spleen weighed 450 g and histologic sections revealed myeloid metaplasia. A liver biopsy also showed extramedullary hematopoiesis. N o granulomas o r evidence of lymphoma were found. Cytogenetics of a direct spleen preparation showed 4% of cells had 44 chromosomes, 12% had 47 chromosomes, and 8% had ringed chromosomes.Although the patient recovered from his surgical procedure, daily fevers to 103 F continued, and leg pain requiring large amounts of parenteral narcotics became his dominant complaint. He was given one course of prednisone and vincristine; however, the lower extremity pain worsened. X-rays now showed striking tibia1 periostitis from ankle to knee with soft tissue swelling, although they had been normal just three months be...

Idiopathic Myelofibrosis With Generalized Periostitis in a 4-Year-Old Girl

“…Periostitis may be present, as in our patient, leading to bone pain, especially in the lower extremities. 12 The peripheral blood picture is characterized by teardrop-shaped (dacrocytes) and nucleated red blood cells, immature leukocytes, and abnormally large platelets. Anemia is invariably present and is usually of the normocytic normochromic type.…”

Multimodality imaging of osseous involvement In haematological malignancies

“…59 Also, periostitis occurs in few patients with myelofibrosis, seen on radiographs as periosteal new bone formation in extremities. 60 Other imaging manifestations in patients with myelofibrosis include hepatosplenomegaly, lymphadenopathy and extramedullary haematopoiesis in other organ systems. 61 In the spine, two different patterns of osteosclerosis can be seen: a diffuse homogeneous pattern or involvement of the superior and inferior margins of the vertebral body (sandwich vertebrae).…”

Single-Fraction Radiotherapy for Palliation of Myelofibrosis-Related Extremity Pain Syndrome

“…In the earliest published report of the merits of palliative external beam radiation therapy for MF-related bone pain, Beattie and Withey 28 described the case of a 48-year-old female diagnosed with polycythemia vera who received treatment to her femur and humerus. In 1979, Mason et al 10 described a 56-year-old man with MF who presented with severe lower extremity pain. He was treated with 200 cGy to each lower extremity and was noted to have a decrease in pain, swelling, and associated edema over the next six months.…”