A 26-year-old male patient with pachydermoperiostosis is reported. He had severe anemia with myelofibrosis. Treatment with iron, prednisolone, oxymethorone and 1 alpha (OH)D3 were not satisfactory. But steroid pulse therapy with parenteral iron improved his anemia and pancytopenia, but was not sufficient to relieve the bone marrow fibrosis or splenomegaly. The mechanism of anemia which was cons...

A case of primary myelofibrosis involving lymph node and with a novel cytogenetic abnormality [del (18) (p11.2-3)] is reported. The abnormalities are identical among specimens from the lymph node, peripheral blood, and bone marrow that were analyzed years apart. Additionally, we show that the infiltrate by dysplastic megakaryocytes in the lymph node morphologically mimics a metastatic mesenchym...

Single-nucleotide polymorphism arrays allow for genome-wide profiling of copy-number alterations and copy-neutral runs of homozygosity at high resolution. To identify novel genetic lesions in myeloproliferative neoplasms, a large series of 151 clinically well characterized patients was analyzed in our study. Copy-number alterations were rare in essential thrombocythemia and polycythemia vera. I...

In the present study, we investigated disease characteristics and clinical outcome in young patients (< 40 years) with World Health Organization (WHO)-defined essential thrombocythemia (ET) compared with early/prefibrotic primary myelofibrosis (PMF) with presenting thrombocythemia. We recruited 213 young patients (median age, 33.6 years), including 178 patients (84%) with WHO-defined ET and 35 ...

Introduction. Coexistence of myeloproliferative neoplasms with lymphoproliferative syndromes has been described in the past, whereas plasma cell dyscrasias seem to be the most common cases. Case Presentation. We present a case of a 59-year-old Caucasian female of Greek origin who presented with thrombocytosis. Clinical and laboratory investigation disclosed the presence of a smoldering myeloma ...

A 50-year-old man with a 20-year history of myelofibrosis developed mild impairment of dorsal column sensation and ataxia of gait. A myelogram and subsequent peroperative biopsy demonstrated spinal cord compression due to extramedullary haematopoiesis. There was an excellent clinical response to surgery and radiotherapy. The characteristic clinical features and the pathogenesis of this unusual ...

The histogenesis of blasts in acute myelofibrosis is generally regarded to be of megakaryocytic origin. Three case reports are presented and 19 other reported cases were reviewed from the literature where the cells of origin appear to be myeloblasts, myelomonoblasts, lymphoblasts, or undifferentiated blasts. It is therefore postulated that acute myelofibrosis is a hemopoietic stem cell disorder...

OBJECTIVES The only known curative therapy for primary myelofibrosis is allogeneic hematopoietic stem cell transplant. MATERIALS AND METHODS We retrospectively evaluated 11 transplant procedures involving 10 patients (5 men and 5 women) diagnosed with primary myelofibrosis between 2005 and 2014. RESULTS The median age at the time of transplant was 60.5 years (range, 22-62 years). Stem cell ...

Primary myelofibrosis is a myeloproliferative neoplasm characterized by bone marrow fibrosis, megakaryocyte atypia, extramedullary hematopoiesis, and transformation to acute myeloid leukemia. To date the stem cell that undergoes the spatial and temporal chain of events during the development of this disease has not been identified. Here we describe a CD133(+) stem cell population that drives th...

3. Harrison C, Kiladjian JJ, Al-Ali HK, Gisslinger H, Waltzman R, Stalbovskaya V, et al. JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med. 2012;366:787–98. 4. Ciurea SO, Sadegi B, Wilbur A, Alagiozian-Angelova V, Gaitonde S, Dobogai LC, et al. Effects of extensive splenomegaly in patients with myelofibrosis undergoing a reduced intensity allogeneic s...