Myeloproliferative Disorders

and, George Vassiliou; Green, Anthony

doi:10.1002/9780470987056.ch46

Cited by 4 publications

(2 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The main pathological feature is expansion of total red cell mass with independent of erythropoietin, elevation in platelets and neutrophils 17,18 .…”

Section: Discussion Of Pathologymentioning

confidence: 99%

Portal Vein Thrombosis in Polycythaemia Vera: A Case Report

et al. 2012

View full text Add to dashboard Cite

At presentation lab investigation revealed Hb(Haemoglobn) 16.5g/dl, WBC 11.0 x109/L, ESR 10 mm1st hour, Hct( Haematocrit) 0.51, Platelet 350 x109/L, serum bilirubin 1.8 g/dl, serum total protein 8.1 g/dl, and albumin 4.5g/dl.The first USG (Ultrasongogram) done on September 9, 2007 (Fig-1) showed liver with homogenous echo texture with increased periportal echogenecity and echogenic structures noted within the portal vein along with cavernous formation. Duplex scan confirmed the echogenic structure to be thrombus in the lumen of main portal trunk causing partial obliteration (Fig-2). Mean velocity within the cavernous was 30.6 cm/sec. Thrombus was still present within the portal vein after anticoagulation (Fig-3), however, cavernous velocity was increased (35.6 cm/sec). Endoscopy upper GIT showed grade I esophageal varices with duodenal erosion. Later on he was treated with warfarin 5 mg and Propranolol 20 mg Daily. Two months later a follow up Color Doppler showed re-canalization of portal vein, a normal hepatic vein flow and development of collateral vessels in hilar and peripancreatic region along with moderate splenomegaly (18.5 cm). Differential diagnoses:The diagnosis of acute EPVT was confirmed by USG. Causes of abdominal pain like appendicitis, pancreatitis, cholecystitis, cholangitis and inflammatory bowel diseases were excluded by history, examination and USG. Liver function tests including the viral markers and renal function were also normal. Amongst other causes of systemic prothrombotic states were inherited thrombophilia, MPD, so our differential diagnoses were inherited thrombophilia and MPD.Coagulation screening test were done PT( Prothrombin time) 12.4s, APTT( activated partial thromboplastin time) 35.5s, TT (Thrombin time) 12s, Fibrinogen 200mg/dl were found to be normal. ATIII (Anti-thrombin III, 118.6%) and APCR ( Activated protein C resistance, 2.45) were found negative, protein C (121.7%) and protein S (76.4%) were also normal. Ham's test, sucrose lysis test and sickle test were negative (Table-I A 26 year old youngman presented with the complaints of sudden severe abdominal pain for 4 days in August, 2007. It was dull aching in nature, associated with nausea and vomiting not accompanied by haematemesis and melaena. He was afebrile and gave no history of dark urine,headache, seizure, loss of consciousness, generalized itching or erythromelalgia.Physical examination revealed congested lower palpebral conjunctiva without facial plethora. He was non icteric. Spleen was palpable 10 cm from left costal margin in nontender soft abdomen. Ascites was absent. Initial investigations and treatment with anticoagulation was done in the dept. of Gastroenterology in collaboration with the dept of Haematology and Vascular Surgery.

show abstract

“…The main pathological feature is expansion of total red cell mass with independent of erythropoietin, elevation in platelets and neutrophils 17,18 .…”

Section: Discussion Of Pathologymentioning

confidence: 99%

Portal Vein Thrombosis in Polycythaemia Vera: A Case Report

et al. 2012

View full text Add to dashboard Cite

show abstract

“…[3] These are uncommon hematological malignancies with worldwide incidence of 2-3/100,000, 1.5-2 per 100,000, and 0.5-1.5/100,000, respectively. [4] At present, the mutation which is found most commonly in classical BCR-ABL-negative MPNs is Janus-associated kinase 2 (JAK2) V617F5. In 2005, the JAK2V617F mutation [5][6][7] was shown in 95% of patients with PV and in just more than half of those with ET and PMF.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological spectrum of BCR-ABL-Negative myeloproliferative neoplasms with correlation with janus-associated kinase 2 mutation

Dixith

Sara

Vangala

et al. 2019

Indian Journal of Medical and Paediatric Oncology

View full text Add to dashboard Cite

Background: Non chronic myelogenous leukemia (non-CML)/BCR-ABL-negative myeloproliferative neoplasms (MPNs) include essential thrombocythemia (ET), polycythemia vera ( P V), and primary myelofibrosis ( P MF) (apart from chronic neutrophilic leukemia and chronic eosinophilic leukemia, which are rare). They are uncommon clonal disorders of adults, with an incidence ranging from 0.5 to 3/100,000 persons, BCR-ABL negative, and characterized by the activation of Janus-associated kinase 2 (JAK2). Very few studies have been reported from India. Aims and Objectives: The aims and objectives of this study were to analyze the clinicopathological spectrum and to determine the frequency of JAK2 mutation in patients of non-CML/BCR-ABL negative MPNs. Materials and Methods: Clinical and morphological features and frequency of JAK2 mutation in patients with PV, ET, and PMF were studied at a tertiary care hospital. The material was retrieved from the hematopathology records and reviewed. Results: JAK2V617F mutation was found in 10 of 14 cases (71%) of MPNs, 100% in PV, 50% in ET, and 71% of idiopathic myelofibrosis. The presence of JAK2V617F mutation was associated with a higher hemoglobin level ( P < 0.05), a higher TLC ( P < 0.05), and higher age ( P < 0.05). Results showed that there are morphologic differences, and megakaryocytic morphology represents a useful clue for the differential diagnosis of these three BCR-ABL-negative MPN subtypes. Conclusion: The JAK2 V617F mutation was detected in 71% of patients with MPN disorders. Peripheral blood mutation screening for JAK2 V617F should be incorporated into the initial evaluation of patients suspected to have MPNs. Differences in megakaryocytic morphology provide the histomorphological hallmark of BCR-ABL-negative MPN subtypes.

show abstract

Pulmonary hypertension in patients with essential thrombocythemia and reactive thrombocytosis

Altıntaş

Karahan

Paşa

et al. 2007

Leukemia & Lymphoma

View full text Add to dashboard Cite

Increased incidence of pulmonary hypertension (PH) has been reported in patients with chronic myeloproliferative disorders. The exact incidence of PH in essential thrombocythemia (ET) is unknown. Most of the reported literature consists of case reports or small studies. We designed this study to asses the incidence of PH in patients with ET and reactive thrombocytosis. Previously or newly diagnosed 46 patients with ET, and 40 patients with reactive thrombocytosis secondary to iron deficiency anemia were found to be eligible for this study. Diagnosis of PH was established via transthoracic echocardiography. PH was found in 22 (47.8%) out of 46 patients with ET. Seven patients with PH were newly diagnosed ET, 5 patients with PH were in low, and the other patients with PH were in intermediate or high risk category. We found statistically significant difference in terms of platelet counts between ET patients with PH and without PH (p = 0.027). None of the patients with reactive thrombocytosis had PH. In conclusion, PH appears to be common in patients with ET. Therefore, all patients with ET should be evaluated for PH. Larger and prospective studies are required to clarify the long-term impact of PH on the survival of these patients. Future studies are also needed to determine whether cytoreductive treatment and aspirin prevent the development of PH, and to determine the effects of cytoreductive treatments and aspirin on the prognosis of PH. The effect of PH on ET prognosis should also be determined in low risk ET patients.

show abstract

Myeloproliferative Disorders

Cited by 4 publications

References 11 publications

Portal Vein Thrombosis in Polycythaemia Vera: A Case Report

Portal Vein Thrombosis in Polycythaemia Vera: A Case Report

Clinicopathological spectrum of BCR-ABL-Negative myeloproliferative neoplasms with correlation with janus-associated kinase 2 mutation

Pulmonary hypertension in patients with essential thrombocythemia and reactive thrombocytosis

Contact Info

Product

Resources

About