OBJECTIVE: Myeloproliferative neoplasm (MPN) is considered as one of the risk factors of ischemic stroke. Some MPN patients manifest stroke as their first symptom. Our purpose was to assess diagnostic rate of MPN in newly diagnosed acute ischemic stroke patients. METHODS: This study was performed using National Health Insurance Service Ilsan Hospital dataset. Data retrieving was performed by defining by defining the patient with coding of acute ischemic stroke from January 2013 to June 2017. We selected only the patients who had checked brain magnetic resonance imaging and complete blood cell count (CBC) in emergency room or on admission. Among the results of CBC finding, hemoglobin and platelet count were analyzed. Erythrocytosis was defined >16.5 g/dL (male), >16 g/dL (female) according to revised World Health Organization (WHO) classification of polycythemia vera (PV) criteria. Thrombocytosis was >450,000/µL according to revised WHO classification of essential thrombocythemia (ET). RESULTS: Total number of newly diagnosed acute ischemic stroke was 1,613 patients. Seven patients (0.43%) were diagnosed MPN (ET=2, PV=5) after ischemic stroke. Patients who had thrombocytosis and erythrocytosis were 18 and 105, respectively. Three patients who had thrombocytosis were diagnosed MPN (ET=2, PV=1). Two patients with erythrocytosis were diagnosed MPN (PV=2). Two patients had both thrombocytosis and erythrocytosis, and two of them were diagnosed PV. Seventy-one patients who had erythrocytosis were normalized in follow-up period. Six patients who had thrombocytosis and 30 patients who had erythrocytosis did not further evaluate. CONCLUSION: CBC has to be carefully read and MPN can be suspected. Diagnosis must be confirmed by hematologist to initiate appropriate treatment. It is important to recognized suspected MPN patients to prevent stroke.
Blood Cell Count ; Brain ; Classification ; Clinical Coding ; Dataset ; Diagnosis ; Emergency Service, Hospital ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; National Health Programs ; Platelet Count ; Polycythemia ; Polycythemia Vera ; Risk Factors ; Stroke ; Thrombocythemia, Essential ; Thrombocytosis ; World Health Organization

The World Health Organization (WHO) Classification of Tumors of Haematopoietic and Lymphoid Tissues was recently published in a revised fourth edition. The categories of myeloproliferative neoplasms (MPNs) have not significantly changed since the 2008 fourth edition of the classification; however, newly discovered mutations including CALR and CSF3R and improved characterizations and standardizations of morphological features of some entities, particularly BCR-ABL1-negative MPNs, have impacted the diagnostic criteria of disease entities, increasing the reliability and reproducibility of diagnoses. The 2017 revised edition attempts to incorporate new clinical, prognostic, morphologic, and genetic data that have emerged since the last edition. This article reviews the major changes in the classification and their rationale for MPN classification within the revised 2017 WHO system.
Classification ; Diagnosis ; Global Health* ; Lymphoid Tissue ; Myeloproliferative Disorders ; Polycythemia Vera ; Primary Myelofibrosis ; Thrombocythemia, Essential ; World Health Organization*

No abstract available.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Amino Acid Sequence ; Calreticulin/*genetics ; Child ; DNA/chemistry/genetics/metabolism ; Exons ; Female ; Humans ; Janus Kinase 2/*genetics ; Male ; Middle Aged ; Molecular Sequence Data ; Polymorphism, Single Nucleotide ; Receptors, Thrombopoietin/genetics ; Sequence Analysis, DNA ; Sex Factors ; Thrombocythemia, Essential/*diagnosis/genetics ; Young Adult

OBJECTIVETo investigate the value of myeloproliferative neoplasms Symptom Assessment Form total symptom score (MPN-SAF-TSS)in assessing constitutional symptoms among Ph/BCR- ABL negative myeloproliferative neoplasm (MPN)patients.

METHODSA cohort of 628 MPN patients were evaluated by MPN- SAF- TSS.

RESULTSFatigue was the most common symptom (76.0%, 76.2%vs 89.9%)and the highest average severity of all the symptoms (3.46±2.97, 3.47±2.99vs 4.74±3.04 scores)among polycythemia vera (PV), essential thrombocythemia (ET)and primary myelofibrosis (PMF)patients. Using the MPN- SAF- TSS analysis, PMF patients showed highest burden of symptoms (28.9 ± 19.1), followed by PV patients (19.2 ± 16.8), and finally ET patients (17.1 ± 15.3). Instinct differences were observed between PMF and PV patients (χ(2)=6.371,P=0.021), PMF and ET patients (χ(2)= 14.020,P<0.001). No significant difference was found between PV and ET patients (χ(2)=2.281,P=0.191).

CONCLUSIONMPN- SAF- TSS was effective in evaluating the symptomatic burden among Ph/BCRABL negative MPN patients and could be used for serial assessment in this clinical setting.

Humans ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative ; diagnosis ; physiopathology ; Polycythemia Vera ; complications ; Primary Myelofibrosis ; complications ; Thrombocythemia, Essential ; complications

Although there have been substantial advances in confirming diagnoses and defining the molecular characteristics of polycythemia vera (PV) and essential thrombocythemia (ET), treatments for these conditions remain elementary. The main goal is still mainly focused on reducing the risk of thrombosis. The prevention of hemorrhage, leukemia transformation, and progression to myelofibrosis has yet to be established. To reduce the risk of thrombosis, risk-adapted treatment is recommended. Phlebotomy is the most important and effective treatment modality for patients with erythrocytosis, while cytoreduction using hydroxyurea, busulfan, or interferon-α is an ancillary treatment for patients at high risk for thrombosis. Anagrelide is used to decrease platelet counts in patients with thrombocytosis by inhibiting the maturation of platelets from megakaryocytes. Recent trials have shown that ruxolitinib, a Janus kinase (JAK) inhibitor, has clinical benefits in patients with polycythemia vera who show an inadequate response, or unacceptable side effects, to therapeutic doses of hydroxyurea. Theoretically, JAK inhibitors may also delay the progression of leukemia transformation and myelofibrosis but there is still no evidence of this. The cost of JAK inhibitors for the treatment of patients with PV/ET is a difficult hurdle for its use as a first-line treatment.
Busulfan ; Diagnosis ; Hemorrhage ; Humans ; Hydroxyurea ; Janus Kinases ; Leukemia ; Megakaryocytes ; Phlebotomy ; Phosphotransferases ; Platelet Count ; Polycythemia Vera* ; Polycythemia* ; Primary Myelofibrosis ; Thrombocythemia, Essential* ; Thrombocytosis ; Thrombosis

Philadelphia chromosome-negative classical myeloproliferative neoplasms include polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In recent years, there have been major advances in our understanding of the molecular pathophysiology of these rare subgroups of myeloproliferative neoplasms. The World Health Organization diagnostic criteria were revised in 2008, and incorporated new somatic mutations of JAK2 V617F, found in most patients with polycythemia vera, essential thrombocythemia, or primary myelofibrosis. Subsequently, other mutations (MPL W515 and CALR) were discovered and this led to substantial changes in the diagnosis and treatment guidelines. This article reviews the diagnostic criteria for Philadelphia chromosome-negative classical myeloproliferative neoplasms, and changes in the diagnostic algorithm for clinical practice in Korea.
Diagnosis* ; Humans ; Korea ; Polycythemia Vera ; Primary Myelofibrosis ; Thrombocythemia, Essential ; World Health Organization

No abstract available.
Adult ; Biomarkers, Tumor/genetics ; Bone Marrow/pathology ; Calreticulin/genetics ; Erythropoietin/blood ; Female ; Fusion Proteins, bcr-abl/*genetics ; Hematocrit ; Hemoglobins/analysis ; Humans ; Janus Kinase 2/genetics ; Male ; Middle Aged ; Mutation ; Myeloproliferative Disorders/*diagnosis/genetics ; Polycythemia Vera/*diagnosis/genetics ; Receptors, Thrombopoietin/genetics ; Thrombocythemia, Essential/diagnosis ; World Health Organization

We evaluated the incidence, clinical characteristics, and prognostic impact of calreticulin (CALR) mutations in essential thrombocythemia (ET) and primary myelofibrosis (PMF) patients. In all, 48 ET and 14 PMF patients were enrolled, and the presence of CALR mutations was analyzed by direct sequencing. Patients were classified into three subgroups according to Janus kinase 2 (JAK2) V617F and CALR mutation status, and their clinical features and prognosis were compared. CALR mutations were detected in 15 (24.2%) patients, and the incidence increased to 50.0% in 30 JAK2 V617F mutation-negative cases. These included 11 patients with three known mutations (c.1092_1143del [seven cases], c.1154_1155insTTGTC [three cases], and c.1102_1135del [one case]) and 4 patients with novel mutations. ET patients carrying CALR mutation were younger, had lower white blood cell counts, and experienced less thrombosis during follow-up than those carrying JAK2 V617F mutation, while both patient groups showed similar clinical features and prognosis. In ET patients without JAK2 V617F mutation, CALR mutation did not significantly affect clinical manifestation and prognosis. In conclusion, CALR mutation analysis could be a useful diagnostic tool for ET and PMF in 50% of the cases without JAK2 V617F mutations. The prognostic impact of CALR mutations needs further investigation.
Adult ; Aged ; Calreticulin/*genetics ; DNA Mutational Analysis ; Exons ; Female ; Genotype ; Humans ; INDEL Mutation ; Janus Kinase 2/genetics ; Male ; Middle Aged ; Primary Myelofibrosis/diagnosis/epidemiology/*genetics ; Prognosis ; Republic of Korea ; Tertiary Care Centers ; Thrombocythemia, Essential/diagnosis/epidemiology/*genetics ; Young Adult

Bone marrow examination is useful in the diagnosis and staging of hematologic disease. This procedure is generally considered safe; however, there are several adverse events associated with bone marrow biopsy. The most frequent and serious adverse event is hemorrhage. Risk factors include coagulopathy, myeloproliferative disorders, and anticoagulant or antiplatelet medications. Most hemorrhage is local hematoma; however, infrequently retroperitoneal hemorrhage occurs. In the case of massive hemorrhage, operation or angiographic embolization may be required. We report on a case of retroperitoneal hemorrhage after bone marrow aspiration and biopsy in an essential thrombocythemia patient.
Biopsy* ; Bone Marrow Examination ; Bone Marrow* ; Diagnosis ; Hematologic Diseases ; Hematoma ; Hemorrhage* ; Humans ; Methods ; Myeloproliferative Disorders ; Risk Factors ; Thrombocythemia, Essential*

Aged ; Female ; Humans ; Myocardial Infarction ; diagnosis ; etiology ; Stents ; adverse effects ; Thrombocythemia, Essential ; complications ; diagnosis ; Thrombosis ; diagnosis ; etiology