3.The assessment of symptomatic burden among Ph/BCR- ABL negative myeloproliferative neoplasm patients.
Junqing XU ; Zefeng XU ; Jingya WANG ; Bing LI ; Xiujuan SUN ; Tiejun QIN ; Yue ZHANG ; Hongli ZHANG ; Liwei FANG ; Lijuan PAN ; Naibo HU ; Shiqiang QU ; Zhijian XIAO
Chinese Journal of Hematology 2016;37(1):26-29
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
4.Acute myocardial infarction and sub-acute stent thrombosis associated with occult essential thrombocythemia.
Zafer ISILAK ; Mehmet TEZCAN ; Murat ATALAY ; Ejder KARDESOGLU
Chinese Medical Journal 2014;127(19):3512-3513
Aged
;
Female
;
Humans
;
Myocardial Infarction
;
diagnosis
;
etiology
;
Stents
;
adverse effects
;
Thrombocythemia, Essential
;
complications
;
diagnosis
;
Thrombosis
;
diagnosis
;
etiology
5.A Case of Post-Essential Thrombocythemia Myelofibrosis with Severe Osteosclerosis.
Kyo Kwan LEE ; Han Ik CHO ; Hyun Sook CHI ; Do Yeun KIM ; Seok Lae CHAE ; Hee Jin HUH
The Korean Journal of Laboratory Medicine 2010;30(2):122-125
Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. After many years, a few patients with ET may develop bone marrow (BM) fibrosis and rarely develop osteosclerosis. A 60-yr-old female was admitted due to severe left upper quadrant abdominal discomfort. She had been diagnosed as ET 19 yrs ago. On liver computed tomography severe splenomegaly was shown. Laboratory tests revealed WBC 24.3x10(9)/L, hemoglobin 13.4 g/dL, platelets 432x10(9)/L, lactate dehydrogenase 4,065 IU/L (reference range; 240-480). Blood smear demonstrated leukoerythroblastosis, teardrop cells, and giant and hypogranular platelets. BM study revealed inadequate aspirate due to dry tap. BM biopsy showed clusters of dysplastic megakaryocytes, grade 3 fibrosis, and severe osteosclerosis. Major/minor BCR-ABL1 rearrangement and JAK2 V617F mutation were not detected. Cytogenetic studies revealed normal karyotype. According to the 2008 WHO diagnostic criteria, the patient was diagnosed as having post-essential thrombocythemia myelofibrosis with severe osteosclerosis.
Bone Marrow/pathology
;
Female
;
Humans
;
Megakaryocytes/pathology
;
Middle Aged
;
Osteosclerosis/complications/*diagnosis
;
Primary Myelofibrosis/complications/*diagnosis
;
Splenomegaly/etiology
;
Thrombocythemia, Essential/complications/*diagnosis
;
Tomography, X-Ray Computed
6.Effects of sodium ozagrel in primary thrombocytosis combined with thrombosis.
Hong-Xia YAO ; Li HUANG ; Cong-Ming WU ; Li-E LIN ; Zhao-Qian HUANG ; Ju-Feng WU ; Shu-Wen WANG ; Wen-Ting CHEN ; Rui-Mei TANG
Journal of Experimental Hematology 2009;17(5):1360-1362
This study was aimed to investigate the incidence of thrombosis in patients with primary thrombocytosis (PT) and its correlation with function changes of platelets, and to explore the effect of thromboxane A2 (TXA2) inhibitor-ozagrel sodium on platelet activity and its efficacy for prevention and treatment of thrombosis. The CD62P and PAC-1 levels on platelet surface were detected by flow cytometry; the levels of TXB2 (metabolic product of TXA2) and 6-keto-PGFIalpha (metabolic product of prostacyclin) were detected by FLISA. The function change of platelets and its correlation with thrombosis were observed and compared in PT patients with and without thrombosis. The results indicated that the TXB2, PAC-1 and CD62P level, and TXB2/6-keto-PGF1alpha ratio in PT patients with thrombosis were higher than those in PT patients without thrombosis before treatment with ozagrel sodium (p<0.01). After treatment with ozagrel sodium, the function indexes of platelets such as CD62P, PAC-1, TXB2 and TXB2/6-keto-PGF1alpha except 6-keto-PGF1alpha in PT patients with and without thrombosis decreased obviously (p<0.01), but there was no significant difference in TXB2, 6-keto-PGF1alpha and TXB2/6-keto-PGF1alpha levels between PT patients with and without thrombosis except CD62P and PAC-1. It is concluded that the multi-index of platelets in PT patients with thrombosis are higher than that in PT patients without thrombosis, the activation of platelet function is a high risk factor for thrombosis of PT patients. The ozagrel sodium can obviously reduce the platelet activation, decrease the production of TXA2 and ameliorate the TXB2/6-keto-PGF1alpha ratio. The ozagrel sodium not only possesses therapeutic effect, but also preventive efficacy for thrombosis.
Adolescent
;
Adult
;
Aged
;
Female
;
Fibrinolytic Agents
;
therapeutic use
;
Humans
;
Male
;
Methacrylates
;
therapeutic use
;
Middle Aged
;
Thrombocythemia, Essential
;
complications
;
drug therapy
;
Thrombosis
;
complications
;
drug therapy
;
Young Adult
7.Correlation of thrombosis with increased platelet turnover in essential thrombocythemia.
Xin-Pu GAO ; Zheng-Min LIU ; Jian LI ; Xue-Ying HAN
Journal of Experimental Hematology 2006;14(1):165-167
This study was aimed to investigate the correlation of thrombosis with increased platelet turnover in essential thrombocythemia. According to presence or absence of thrombosis, 26 patients with ET were divided into two groups. Reticulated platelets (RP) were measured by flow cytometry and 26 healthy volunteers were selected as healthy controls. The ET patients with thrombosis were treated with hydroxyurea and interferon-alpha. The results demonstrated that the ET patients with thrombotic events had a significantly higher RP percentage (14.8% +/- 7.2%) than that in both asymptomatic ET patients (4.5% +/- 2.3%) and normal control (3.3% +/- 1.5%), (P < 0.05); the RP percentage in asymptomatic ET patients did not differ significantly from controls. ET patients with thrombosis also had a significantly higher absolute RP (ARP) count than those in ET patients without thrombosis [(176 +/- 37) x 10(9)/L vs (46 +/- 12) x 10(9)/L]. The ET patients with thrombosis were successfully treated with hydroxyurea plus INF-alpha, the RP percentage and ARP counts obviously reduced. In conclusion, when the ET patients had thrombotic events, those patients had a significantly higher RP percentage and ARP compared with patients without thrombosis and healthy controls. The ET patients with thrombosis were successfully treated with hydroxyurea plus INF-alpha.
Adult
;
Aged
;
Aged, 80 and over
;
Blood Platelets
;
cytology
;
physiology
;
Female
;
Humans
;
Male
;
Middle Aged
;
Platelet Count
;
Thrombocythemia, Essential
;
blood
;
complications
;
Thrombosis
;
blood
;
complications
9.Chronic myeloid leukemia onset with marked thrombocythemia.
Qun SHEN ; Jian-Wei ZHOU ; Guang-Rong ZHU ; Yue-Yan YANG ; Hai-Rong QIU ; Guang-Rong ZHU ; Wen XIA ; Peng-Jun JIANG
Journal of Experimental Hematology 2006;14(2):247-251
This study was aimed to investigate the clinical, pathological and biological features of a special case of chronic myeloid leukemia (CML) with marked thrombocythemic onset. The morphological changes of cells were analyzed by using bone marrow smear and biopsy; Ph chromosome, a specific marker of CML, was assayed by conventional chromosomal analysis and fluorescence in situ hybridization, bcr/abl fusion gene was detected by reverse transcription-polymerase chain reaction. The results indicated that CML mimicked essential thrombocythemia (ET) at presentation was relatively rare and might be misdiagnosed as ET, bone marrow smear and biopsy revealed, marked thrombocytosis and moderate leukocytosis; RT-PCR, FISH and conventional chromosomal analysis demonstrated the existence of Ph chromosome and bcr/abl fusion gene. This special CML could progress into accelerated phase or blast crisis. The megakaryocytes in Ph+ ET were smaller than normal ones and had typically hypolobulated round nuclei. Patients diagnosed as Ph+ ET might progress into CML and showed a high tendency to myelofibrosis and blastic transformation. It is concluded that the value of routine cytogenetical and molecular biological analysis in diagnosis for potential CML cases, which mimicked ET as in this presentation, is very distinctive, and the importance is magnified by the recent availability of imatinib, a specific inhibitor of the bcr/abl tyrosine kinase produced by the Philadelphia chromosome. Every case of "ET" should be tested for the Philadelphia chromosome and bcr/abl transcript.
Adult
;
Diagnosis, Differential
;
Female
;
Fusion Proteins, bcr-abl
;
genetics
;
Gene Rearrangement
;
Humans
;
In Situ Hybridization, Fluorescence
;
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
;
complications
;
diagnosis
;
genetics
;
Megakaryocytes
;
pathology
;
ultrastructure
;
Philadelphia Chromosome
;
Reverse Transcriptase Polymerase Chain Reaction
;
Thrombocythemia, Essential
;
diagnosis
10.Detection and clinical significance of JAK2 mutation in 412 patients with chronic myeloproliferative neoplasms.
Hong-Ying CHAO ; Zheng FAN ; Ri ZHANG ; Yi-Min SHEN ; Wan CHEN ; Hai-Rong FEI ; Zi-Ling ZHU ; Yu-Feng FENG ; Zi-Xing CHEN ; Yong-Quan XUE
Chinese Journal of Oncology 2009;31(7):510-514
OBJECTIVETo investigate the frequency of JAK2V617F mutation in Chinese patients with chronic myeloproliferative neoplasms (MPN) and to study the relationship between JAK2V617F mutation and clinical characteristics.
METHODSJAK2V617F mutation was screened by allele-specific polymerase chain reaction (AS-PCR).
RESULTSJAK2V617F mutation was detected in 277 of the 412 patients with MPN. The frequency of JAK2V617F mutation was similar among essential thrombocythemia (ET), idiopathic myelofibrosis (IMF) and chronic myeloproliferative disorders-unclassified (MPD-U) (P > 0.05), but it was significantly lower than that in polycythemia vera (PV) (P < 0.05). The presence of JAK2V617F was found to be significantly correlative with advanced age at diagnosis (P < 0.01) and with higher hemoglobin levels and higher leukocyte counts (P < 0.05). Significant difference was found in complication of vascular events between JAK2V617 positive and negative patients (P < 0.05). JAK2V617F positive MPD-U patients were more prone to progress into typical MPN compared with JAK2V617F negative MPD-U patients. The association between abnormal karyotype and JAK2V617F was not found in cytogenetical analysis of 301 patients.
CONCLUSIONThe presence of JAK2V617F in MPD-U is associated with the disease development. There is a correlation between JAK2V617F mutation in MPN and advanced age, higher leukocyte counts, hemoglobin level and vascular events.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Female ; Follow-Up Studies ; Hemoglobins ; metabolism ; Humans ; Janus Kinase 2 ; genetics ; Leukocyte Count ; Male ; Middle Aged ; Mutation ; Myeloproliferative Disorders ; blood ; complications ; genetics ; Polycythemia Vera ; blood ; complications ; genetics ; Primary Myelofibrosis ; blood ; complications ; genetics ; Thrombocythemia, Essential ; blood ; complications ; genetics ; Thrombosis ; etiology ; Young Adult