• Asian Pacific Journal of Cancer Prevention
• /
• 제16권18호
• /
• pp.8629-8631
• /
• 2016

Background: Primary idiopathic myelofibrosis (PMF) is a clonal Philadelphia chromosome-negative myeloproliferative neoplasm characterized by extramedullary hematopoiesis and marrow fibrosis. It is an uncommon hematopoietic malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-epidemiological profile along with risk stratification in Pakistani patients. Materials and Methods: In this retrospective cross sectional study, 20 patients with idiopathic myelofibrosis were enrolled from January 2011 to December 2014. Data were analyzed with SPSS version 22. Results: The mean age was $57.9{\pm}16.5years$ with 70% of patients aged above 50. The male to female ratio was 3:1. Overall only 10% of patients were asymptomatic and the remainder presented with constitutional symptoms. In symptomatic patients, major complaints were weakness (80%), weight loss (75%), abdominal discomfort (60%), night sweats (13%), pruritus (5%) and cardiovascular accidents (5%). Physical examination revealed splenomegaly as a predominant finding detected in 17 patients (85%) with the mean splenic span of $22.2{\pm}2.04cm$. The mean hemoglobin was $9.16{\pm}2.52g/dl$ with the mean MCV of $88.2{\pm}19.7fl$. The total leukocyte count of $17.6{\pm}19.2{\times}10^9/l$ and platelets count were $346.5{\pm}321.9{\times}10^9/l$. Serum lactate dehydrogenase, serum creatinine and uric acid were $731.0{\pm}154.1$, $0.82{\pm}0.22$ and $4.76{\pm}1.33$ respectively. According to risk stratification, 35% were in high risk, 40% in intermediate risk and 25% in low risk groups. Conclusions: The majority of PMF patients were male and presented with constitutional symptoms in our setting. Risk stratification revealed predominance of advanced disease in our series.

• Tuberculosis and Respiratory Diseases
• /
• 제67권3호
• /
• pp.244-248
• /
• 2009

Primary myelofibrosis is characterized by replacement of bone marrow with fibrotic tissue and the development of extramedullary hematopoiesis. Extramedullary hematopoiesis primarily involves the spleen and liver, but can also occur in the lungs. We report the case of an 80-year-old male who was admitted for evaluation of a lung mass and persistent thrombocytopenia. A percutaneous needle aspiration from the mass in the right lower lung showed myelopoietic cells with fatty tissue. A bone marrow biopsy revealed a hypercellular marrow with an increased number of atypical megakaryocytes. The final diagnosis was a prefibrotic stage of primary myelofibrosis leading to extramedullary hematopoiesis in the lung.

• Journal of Medicine and Life Science
• /
• 제17권3호
• /
• pp.103-106
• /
• 2020

Bone marrow failure, such as aplastic or myelophthisic anemia, can occur due to an underlying lymphoid malignancy and cause life-threatening events. A 58-year-old man diagnosed with angioimmunoblastic T-cell lymphoma had recently visited the emergency department because of an altered level of consciousness caused by acute severe anemia. The laboratory findings were strongly suggestive of bone marrow failure syndrome. Bone marrow examination was immediately performed and, subsequently, dexamethasone was initiated to control the underlying lymphoma. Intravenous immunoglobulin was also administered in combination due to combined immune hemolytic anemia and thrombocytopenia. Bone marrow examination revealed a packed marrow with marked fibrosis and lymphoma involvement. A diagnosis of secondary myelofibrosis related to the underlying lymphoma was made, and sequential combination chemotherapy was introduced despite the presence of severe anemia and thrombocytopenia. After combination chemotherapy, his hematologic profile and underlying lymphoma improved. Better understanding of various hematologic manifestations and knowledge of the rare condition of lymphoma are essential for appropriate diagnostic approaches and treatment.

• Asian Pacific Journal of Cancer Prevention
• /
• 제16권17호
• /
• pp.7889-7892
• /
• 2015

Background: Primary idiopathic myelofibrosis (PMF) is a clonal Ph-chromosome negative myeloproliferative neoplasm characterized by dysregulated kinase signaling and release of abnormal cytokines. In the recent past, following JAK2 V617F mutation invention, important revolution has been made in the molecular diagnostic biology of this disease. The rational of this study was to determine the mutational status of JAK2 V617F in Pakistan patients with PMF. Materials and Methods: In this cross sectional study, 20 patients with PMF were enrolled from January 2011 to December 2014. Diagnosis was based on WHO criteria for PMF. All patients were screened for G-T point mutation (V617F) in the JAK2 gene on chromosome 9 by allele specific PCR. Results: The mean age was $57.9{\pm}16.5years$. The male to female ratio was 3:1. The frequency of JAK2 V617F positivity in our PMF patients was found to be 55%. Positive correlations of JAK2 V617F mutation were established with high TLC count, raised LDH and marked splenomegaly (P<0.05). No correlation of JAK2 V617F could be established with age and gender (P>0.05). Conclusions: The JAK2 V617F mutation frequency in our PMF patients was similar to those reported previously. In our hands JAK2 V617F mutated patients expressed an aggressive disease phenotype. Screening for the mutation in all suspected PMF cases could be beneficial in differentiating patients with reactive and clonal marrow fibrosis.

• Asian Pacific Journal of Cancer Prevention
• /
• 제16권12호
• /
• pp.5013-5018
• /
• 2015

Background: Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell disorders characterized by proliferation of one or more myeloid lineages. Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are classical Philadelphia chromosome (Ph)-negative MPN that have a Janus Kinase 2 (JAK2) mutation, especially JAK2V617F in the majority of patients. The major complications of Ph-negative MPNs are thrombosis, hemorrhage, and leukemic transformation. Objective: To study clinical manifestations including symptoms, signs, laboratory findings, and JAK2V617F mutations of Ph-negative MPN (PV, ET and PMF) as well as their complications. Materials and Methods: All Ph-negative MPN (PV, ET and PMF) patients who attended the Hematology Clinic at Maharaj Nakorn Chiang Mai Hospital from January, 1 2003 through December, 31 2013 were retrospectively reviewed for demographic data, clinical characteristics, complete blood count, JAK2V617F mutation analysis, treatment, and complications. Results: One hundred and fifty seven patients were included in the study. They were classified as PV, ET and PMF for 68, 83 and 6 with median ages of 60, 61, and 68 years, respectively. JAK2V617F mutations were detected in 88%, 69%, and 100% of PV, ET and PMF patients. PV had the highest incidence of thrombosis (PV 29%, ET 14%, and PMF 0%) that occurred in both arterial and venous sites whereas PMF had the highest incidence of bleeding (PMF 17%, ET 11%, and PV 7%). During follow up, there was one ET patient that transformed to acute leukemia and five cases that developed thrombosis (three ET and two PV patients). No secondary myelofibrosis and death cases were encountered. Conclusions: Ph-negative MPNs have various clinical manifestations. JAK2V617F mutations are present in the majority of PV, ET, and PMF patients. This study confirmed that thrombosis and bleeding are the most significant complications in patients with Ph-negative MPN.

• 대한핵의학회지
• /
• 제11권1호
• /
• pp.59-70
• /
• 1977

Eight patients with polycythemia vera were diagnosed from 1965 through 1977 among the patients of Seoul National University Hospital. In our series there were 4 men and 4 women with average age of 54 years at the time of diagnosis. Symptoms of facial plethora, headache, pruritus and dizziness were the most frequent manifestations of disease in order of frequency. At inital examination 8 of 8 patients had facial plethora, 6 of 8 patients splenomegaly and 4 if 8 patients hepatomegaly. The laboratory data revealed mean Red cell volume of 55+10ml/kg which was elevated in all cases. Leukocytosis was observed in 5 of 8 patients and thrombocytosis 2 of 8 patients. Leukocyte alkaline phosphatase was increased in all cases. Bone marrow aspiration disclosed typical panmyelosis in 5 of 8 patients. In one patient there was diffuse myelofibrosis on bone marrow biopsy. The combined diseases included 2 patient with hypertension and 1 patient with thrombosis of aorta and left renal artery. The patients were treated with phlebotomy alone in 2 patients, chemotherapy with busulfan in 1 patient and $^{32}P$ in 5 patients with favorable results. During the mean 29.9 months follow up period, there were development of iron deficiency in 3 patients, hyperuricemia in 2 patients and thrombosis in 1 patient. The occurrence of acute leukemia of myelofibrosis was not observed in the course of disease.

• IMMUNE NETWORK
• /
• 제13권5호
• /
• pp.222-226
• /
• 2013

Translocations involving chromosome 21q22 are frequently observed in hematologic malignancies including acute myeloid leukemia (AML), most of which have been known to be involved in malignant transformation through transcriptional dysregulation of Runt-related transcription factor 1 (RUNX1) target genes. Nineteen RUNX1 translocational partner genes, at least, have been identified, but not Homeobox A (HOXA) genes so far. We report a novel translocation of RUNX1 into the HOXA gene cluster in a 57-year-old female AML patient who had been diagnosed with myelofibrosis 39 months ahead. G-banding showed 46,XX,t(7;21)(p15;q22). The involvement of RUNX1 and HOXA genes was confirmed by fluorescence in situ hybridization.

• 대한핵의학회지
• /
• 제1권1호
• /
• pp.79-82
• /
• 1967

Patients with aplastic anemia were treated with a combination of depo-testosterone cyclopentylpropionate(Upjohn) and dexamethasone. In 7 of 15 patients treated, there was response in which either a significant increase in hemoglobin concentration, a prolonged interval or a cessation of blood transfusion requirement developed during androgen therapy. Younger patients with cellular marrow appeared to be better responding to androgen. EEI(Effective Erythropoietic Index) formulated by Gardner & Nathan(1966) which was a helpful measurement as to whether patients with myelofibrosis whould respond to androgen, was evaluated in patients with aplastic anemia. It was concluded that EEI as well as ferrokinetics indices (Plasma-$^{59}Fe$-disappearance rate, RBC $^{59}Fe$ net incorporation) did not significantly correlate with the degree of response to androgen in aplastic anemia.

• Journal of Yeungnam Medical Science
• /
• 제8권2호
• /
• pp.209-216
• /
• 1991

Acute Megakaryoblastic leukemia is a rare and rapidly fatal disease characterized by proliferation of megakaryocyte series and atypical megakaryocytes in the bone marrow. Acute Megakaryoblastic leukemia is suspicious when 1) megakaryocyte in peripheral blood, mixture of large and small mononuclear megakaryoblast in the bone marrow 2) cytoplasmic budding in blast 3) myelofibrosis (dense medullary overgrowth of reticulin fibers) 4) PAS(+), ANAE(+), SBB(-), peroxidase(-) and which is confirmed by platelet peroxidase oxidation on electromicroscope or monoclonal antibody. A case of aute megakaryoblastic leukemia was studied morphologically and monoclonal antibody.

• 대한수의학회지
• /
• 제63권2호
• /
• pp.8.1-8.3
• /
• 2023

A 13-year-old neutered male Maltese dog presented to our hospital with lethargy and anorexia. Laboratory abnormalities included severe non-regenerative anemia (hematocrit, 12.9%; reticulocyte count 12.8 K/µL). The cytology of bone marrow revealed erythroid hypercellularity with mild myelofibrosis. Therefore, late-stage precursor-targeted immune-mediated anemia (PIMA) was diagnosed. Multimodal treatment including 2 immunosuppressant drugs (prednisolone and mycophenolate mofetil), antithrombic drug (clopidogrel), and blood transfusion was performed. The dog showed complete remission from PIMA, and the total duration of follow-up was 622 days. This is the first case report of canine PIMA managed successfully with prednisolone and mycophenolate mofetil in Korea.