Polycythemia vera megakaryocytes store and release lysozyme to a higher extent than megakaryocytes in secondary polycythemia (polyglobuly)

1999 ◽  
Vol 23 (3) ◽  
pp. 299-306 ◽  
Author(s):  
Claudia Wickenhauser ◽  
Juergen Thiele ◽  
Beate Schmitz ◽  
Semra Frimpong ◽  
Ilka Neumann ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Secondary Polycythemia

scholarly journals The Plasma Disappearance of Radioactive Vitamin B12 in Myeloproliferative Diseases and Other Blood Disorders

Blood ◽  
1961 ◽  
Vol 18 (6) ◽  
pp. 717-726 ◽  
Author(s):  
CHARLES A. HALL ◽  
Alexander E. Finkler ◽  
Edward S. Allen ◽  
Booker T. Moore
Keyword(s):  
Vitamin B12 ◽  
Polycythemia Vera ◽  
Chronic Myelogenous Leukemia ◽  
Intravenous Dose ◽  
Myelogenous Leukemia ◽  
Blood Disorders ◽  
Myeloid Metaplasia ◽  
Myeloproliferative Diseases ◽  
Close Relationship ◽  
Secondary Polycythemia

Abstract 1. The plasma disappearance of a small intravenous dose of radioactive vitamin B12 was determined in control subjects and in patients with various blood disorders. 2. A delayed, sometimes irregular, disappearance was observed in the majority of patients with acute and chronic myelogenous leukemia, myeloid metaplasia, and polycythemia vera. 3. Disappearance was normal in the lymphogenous leukemias, secondary polycythemia and relative polycythemia. 4. The abnormalities observed are believed to indicate an abnormality of vitamin B12 metabolism common to the diseases of the myeloproliferative group and are further evidence of the close relationship between these diseases.

Hematocrit-Driven Referrals for Measurement of RBC Mass. Is the Natural History of Polycythemia Vera Changing?.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4960-4960
Author(s):  
Thein H. Oo ◽  
Sumit Gaur ◽  
Janet Tierney ◽  
Alan Ashare ◽  
Robert Weinstein
Keyword(s):  
Natural History ◽  
Polycythemia Vera ◽  
Hereditary Hemochromatosis ◽  
Cell Mass ◽  
Renal Cysts ◽  
Initial Therapy ◽  
P Value ◽  
Asymptomatic Patients ◽  
History Of ◽  
Secondary Polycythemia

Abstract Red blood cell mass (RCM) elevation is the sine qua non for diagnosis of polycythemia vera (pvera). The Polycythemia Vera Study Group (PVSG) employed therapeutic phlebotomy to lower the HCT to <55% as initial therapy. In the current era of frequent blood testing, RCM is usually requested based only on a high HCT often <55%. Thus the natural history of pvera may be changing if it is being diagnosed at an earlier stage. We reviewed 101 consecutive patients referred in 2002–2003 for RCM testing to characterize RCM requests from a large community referral base. There were 61 men and 40 women with mean HCT<55% (Table 1). 20 had lung disease or were smokers, 3 presented with arterial or venous thrombosis, 7 had cancer, 2 had renal cysts. HCT of men was higher than HCT of women if RCM was normal, but HCT were equivalent between genders if RCM was elevated. HCT was higher in men (p=0.0010) and women (p<0.0001) if RCM was elevated versus normal. HCT elevation was similar in secondary polycythemia vs pvera in men (51.0±4.02 vs 54.3±6.23; p=0.3397) or women (50.5±4.80 vs 49.3±2.12; p=0.4762). Table 1. HCT of patients referred for RCM measurement Gender Whole Group Normal RCM High RCM n HCT n HCT n HCT HCT shown as Mean (Median) ±SD Male 61 48.3 (47.8) ±4.89 45 46.9 (47.2) ±4.0 16 52.1 (51.3) ±5.16 Female 40 45.0 (43.8)± 4.72 32 43.3 (42.5) ±3.69 10 50.0 (50.0) ±3.84 p value 0.0001 <0.0001 0.4932 We examined whether, despite a lower presenting HCT, those with normal RCM demonstrated PVSG criteria that justified referral to our Nuclear Medicine department. Only 7 of the 75 patients with normal RCM met at least 2 of the PVSG “B” criteria or had splenomegaly, thus might have been diagnosed with pvera had RCM been high (Table 2). Table 2. 2 PVSG “B” criteria or splenomegaly and normal RCM Gender WBC ≥12K PLT ≥400K B12 ≥900 ≥ LAP 100 Splenomegaly M 14.4 413 640 158 “normal” M 12.1 585 1336 53 “normal” M 9.4 428 1139 68 “normal” M 8.6 132 1343 15 cm M 8.2 312 492 186 17 cm F 18.2 582 719 23 “normal” F 6.3 226 318 “enlarged” F 7.1 146 813 15.3 cm Overall, 10 of 40 women and 16 of 61 men had an elevated RCM: 4 women and 5 men with polycythemia vera; 6 women and 11 men with secondary polycythemia. One other man was diagnosed with polycythemia vera on the basis of a borderline elevated RCM (33.8 ml/kg), normal O2 Sat on room air, popliteal artery thrombosis, and 3 “B” criteria. Serum erythropoietin (EPO) was low at 2.6 mU/ml (ref range 4–16). He was receiving therapeutic phlebotomy for hereditary hemochromatosis when his RCM was measured. He was the only patient with pvera whose EPO was low (three others had normal EPO levels). The 11 patients with secondary polycythemia who had EPO measured had normal levels. In summary, the HCT was the primary criterion for RCM testing for 2/3 of these patients. Only 5 presented with HCT>55%; mean HCT was ~50% in patients with elevated RCM. The bottom quartile HCT of women with elevated RCM in our patient population was 48.7%. This is a sensitive “cut-off” for finding an elevated RCM (p<0.0001, Chi square with Yates correction). We conclude that patients are referred for RCM testing when a high HCT is found, but at HCT far below the original PVSG parameters. Therefore polycythemia vera is now diagnosed earlier and in mostly asymptomatic patients. A normal EPO level does not rule out a diagnosis of pvera. A HCT < 48.7% may not warrant the measurement of RBC mass.

scholarly journals Erythropoietic Activity in the Plasma of Patients with Polycythemia Vera and Secondary Polycythemia

Blood ◽  
1957 ◽  
Vol 12 (7) ◽  
pp. 614-619 ◽  
Author(s):  
A. N. CONTOPOULOS ◽  
ROLLIN MCCOMBS ◽  
JOHN H. LAWRENCE ◽  
MIRIAM E. SIMPSON
Keyword(s):  
Polycythemia Vera ◽  
Erythropoietic Activity ◽  
Secondary Polycythemia

Thrombotic risk in secondary polycythemia resembles low-risk polycythemia vera and increases in specific subsets of patients

2021 ◽  
Author(s):  
Ivan Krečak ◽  
Hrvoje Holik ◽  
Ivan Zekanović ◽  
Martina Morić Perić ◽  
Tina Marketin ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Low Risk ◽  
Thrombotic Risk ◽  
Secondary Polycythemia

Diagnostic Usefulness of JAK2 Mutation in Manistest or Latent Polycythemia Vera and Secondary Polycythemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4901-4901
Author(s):  
Soo-Mee Bang ◽  
Eun Mi Nam ◽  
Jeong Yeal Ahn ◽  
Jae-Hoon Lee ◽  
Bong-Seog Kim ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Janus Kinase ◽  
Vascular Events ◽  
Jak2 Mutation ◽  
Specific Pcr ◽  
Mutational Status ◽  
Number Of Patients ◽  
Positive Rate ◽  
Clinical Records ◽  
Secondary Polycythemia

Abstract Backgrounds: The discovery of Janus kinase 2 (JAK2) mutation make it easy to diagnose polycythemia vera (PV) in which the prevalence of this mutation is reported up to 96%. The latent PV is defined in patients who show the lower hemoglobin than WHO criteria at the time of diagnosis without any cause of secondary erythrocytosis; 17~18.5g/dL in male and 15 ~16.5g/dL in female. We investigated the mutational status of JAK2 in polycythemia vera either of latent or manifest type and secondary polycythemia. Methods : We reviewed the clinical records of 182 patients from 8 centers, who underwent bone marrow (BM) examination with a suspicion of non-BCR/ABL myeloproliferative diseases (nMPD). JAK2 mutation was examined by allele-specific PCR in 110 patients. Results : The positive rate of JAK2 mutation was 69% of 48 patients with PV, 54% in 41 patients with essential thrombocythemia (ET), 25% of 8 patients with chronic idiopathic myelofibrosis and 100% of 1 patient with unclassifiable MPD. JAK2 mutation was not detected in 7 patients with secondary polycythemia and 5 with reactive thrombocytosis. The number of patients with latent PV was eleven (23%) among the 48 PV patients. Nine of these 11 patients were positive for JAK2 mutation. Finally, sensitivity of JAK2 mutation was 69% (33/48) in all PV, 82% (9/11) in latent PV, and 65% (24/37) in manifest PV. The specificity of JAK2 mutation was 100% for secondary polycythemia. Among the 41 patients with ET, four patient had high hemoglobin met the latent PV criteria. Only one patient among them showed JAK2 mutation. The presence of a JAK2 mutation was closely correlated with the diagnosis of PV (p=0.006), leukocytosis (p=0.000), increased megakaryocytes (p=0.013), and decreased serum EPO (p=0.034) in 98 nMPD patients. In these patients, vascular events before or after the diagnosis and survival were not affected according to JAK2 status during the median follow-up of 25 months (range; 0~211). Conclusions : The JAK2 mutation help diagnose PV with higher sensitivity in latent PV than manifest PV. This finding can be adopted in the diagnosis of PV in newly visited polycythemic patient whose hemoglobin were under the criteria and condition could be excluded for secondary erythrocytosis. Therefore, JAK2 mutation should be incorporated into the work-up of nMPD including PV for accurate diagnosis. In the meeting, we can present the additional JAK2 data of untested 72 patients.

scholarly journals Serum erythropoietin (ESF) titers in polycythemia

Blood ◽  
1981 ◽  
Vol 58 (6) ◽  
pp. 1171-1174 ◽  
Author(s):  
G de Klerk ◽  
PC Rosengarten ◽  
RJ Vet ◽  
R Goudsmit
Keyword(s):  
Polycythemia Vera ◽  
Clinical Importance ◽  
Hemoglobin Concentration ◽  
In Vitro Bioassay ◽  
Mouse Liver Cell ◽  
Fetal Mouse Liver ◽  
The Mean ◽  
Serum Erythropoietin ◽  
Secondary Polycythemia

Abstract Serum ESF titers were measured in 42 polycythemic patients using the fetal mouse liver cell bioassay. ESF titers in patients with secondary polycythemia differed significantly from those in patients with polycythemia vera (p less than 0.0001). Among the 21 patients with secondary polycythemia, 1 patient had an ESF titer less than 10 mU/ml (the lower limit of sensitivity) and 20 had ESF titers that ranged between 11 and 112 mU/ml, with a mean titer of 56 mU/ml. Among the 21 patients with polycythemia vera, 13 patients had ESF titers less than 10 mU/ml and 8 had ESF titers ranging between 12 and 55 mU/ml, with a mean titer of 26 mU/ml. The mean hemoglobin concentration in the 8 patients with ESF titers greater than 10 mU/ml was significantly below that in the 13 polycythemia vera patients with ESF titers less than 10 mU/ml (p less than 0.03). If ESF titers less than 10 mU/ml had been indicative of polycythemia vera and ESF titers greater than 10 mU/ml had been indicative of secondary polycythemia in patients with hemoglobin concentrations greater than 17.7 g/dl, but not indicative of either condition in patients with hemoglobin concentrations less than 17.7 g/dl, 71.5% of the polycythemic patients in this study would have been diagnosed correctly, 9.5% incorrectly, and in the 19% the diagnosis would have remained uncertain. It was concluded that measurement of serum ESF titers using this in vitro bioassay can be of clinical importance in differentiating between polycythemia vera and secondary polycythemia.

scholarly journals Polycythemia

2020 ◽  
pp. 38-43
Author(s):  
Stanley Kim ◽  
Ricardo Saca ◽  
Pamela Harford
Keyword(s):  
Stem Cell ◽  
Polycythemia Vera ◽  
Myeloproliferative Neoplasm ◽  
Hematocrit Level ◽  
Cell Disease ◽  
Erythroid Progenitors ◽  
Cell Numbers ◽  
Erythropoietin Level ◽  
Secondary Polycythemia ◽  
Rbc Count

Polycythemia is a disease state in which the red blood cell numbers are increased in the blood (erythrocytosis), which in turn makes blood thicker and can cause circulatory problems. Polycythemia Vera is a stem cell disease belonged to a group of myeloproliferative neoplasm in which the erythroid progenitors are overly proliferated by acquired mutation of the JAK2 gene, resulting in excessive erythrocytosis. Secondary Polycythemia refers erythrocytosis due to underlying conditions. It is usually associated with increased blood erythropoietin levels as a compensatory reaction to tissue hypoxia, which can be seen in patients with chronic lung disease or sleep apnea or living at high altitudes. Certain tumors produce the erythropoiet ©Win and testosterestern University of Heone incralth Scienceseases the blood erythropoietin level, resulting in secondary polycythemia. Relative polycythemia is the consequence of plasma volume contraction, falsely raising the RBC count and hemoglobin/hematocrit level in CBC. Two cases of polycythemia are presented: 1) a patient with polycythemia vera and 2) a patient with secondary polycythemia. Various types of polycythemia are discussed with an updated review covering the etiology, clinical manifestation, diagnostic approach and treatment.

scholarly journals Differential cytokine network profile in polycythemia vera and secondary polycythemia

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Maira da Costa Cacemiro ◽  
Juçara Gastaldi Cominal ◽  
Maria Gabriela Berzoti-Coelho ◽  
Raquel Tognon ◽  
Natalia de Souza Nunes ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Cytokine Network ◽  
Network Profile ◽  
Secondary Polycythemia

Polycythemia Vera Vs. Secondary Erythrocytosis Mortality Outcome in a Multiracial Inner City Cohort

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5183-5183
Author(s):  
Jose Nahun Galeas ◽  
Yiting Yu ◽  
Rahul Polineni ◽  
Vineeth Sukrithan ◽  
Cosmin Tegla ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Conflicts Of Interest ◽  
Population History ◽  
Mortality Data ◽  
P Value ◽  
Inpatient Setting ◽  
Kaplan Meier ◽  
Significant Difference ◽  
History Of ◽  
Secondary Polycythemia

Abstract Background Life expectancy of patients with Polycythemia Vera is reduced compared with the general population. History of thrombosis has been found to be the main predictor of death in this disease. Factors like older age, leukocytosis and thrombosis have been shown to increase the risk of mortality. Secondary polycythemia on the other hand is largely an unknown entity. This study aims to compare the various prognostic and predictive factors for mortality between primary polycythemia vera and secondary polycythemia group. Patients and Methods Patients >=18 years of age seen at a Montefiore outpatient clinic and/or inpatient setting from 1998 to 2014 with a diagnosis of polycythemia, either primary or secondary, who have died, were included. This was done with the help of Clinical Looking Glass software after obtaining Institutional Review Board approval. Patients were stratified by race and ethnic groups. Primary polycythemia was confirmed by evaluating each medical record in detail, lab values including erythropoietin level, JAK2 mutation status and bone marrow biopsy reports. Other laboratory results evaluated were WBC, Hg, platelets, hemoglobin, MCV, RDW and albumin. Morbidity including history of arterial/venous thrombosis and mortality data were obtained to compare survival and identify potential predictors of morbidity and mortality. All analyses were conducted using STATA statistical software. Baseline characteristics were compared using two sample tests of proportions and Student t-tests for means. Kaplan-Meier curves were constructed to compare survival by various subgroups. Hazard ratios were calculated using multivariate Cox regression models to adjust for co-variates to compare mortality risk and identify potential independent predictors of mortality. Results Total of 112 patients with polycythemia, who have died within the period of 1998-2014, were identified. 64 (57%) patients had primary polycythemia and 48 (43%) patients were diagnosed with secondary erythrocytosis. The median age for diagnosis was 63 years for secondary and 72 years for primary. The commonest causes of secondary polycythemia in these patients were severe COPD, congenital heart disease and severe pulmonary hypertension. In patients with polycythemia who died within the specified period of 16 years, median time to mortality from diagnosis was significantly shorter (p value: 0.004) in secondary polycythemia (21.1 months or 634 days) compared to primary polycythemia group (42.6 months or 1277 days). Figure 1 illustrates the duration in survival from time to diagnosis between the two groups. There was no significant difference amongst the white, black and Hispanic populations in the 2 groups. 23 (38%) patients in the primary polycythemia group had venous/arterial thrombosis compared to 6 (5%) in the secondary group. This difference was also significant with a p value of 0.005. As expected, erythropoietin was lower in primary compared to secondary group. Also, WBC and platelet counts were significantly higher in primary polycythemia versus secondary polycythemia group. An interesting finding was that the mean albumin was significantly lower in the secondary polycythemia (3.3) patients who died compared to the primary polycythemia patients (3.8) with a p value of 0.004. Conclusions Our findings demonstrate that patients who die with secondary erythrocytosis have shorter time to death from diagnosis compared to primary polycythemia vera. This is most likely due to the worse prognosis associated with the underlying disease causing erythrocytosis. Even though, secondary polycythemia is not generally treated in the same way as primary polycythemia, development of increased hemoglobin should be taken as a cautionary sign for these patients as this is likely a strong predictor of mortality. Moreover, patients who died with secondary polycythemia have lower albumin levels compared to primary polycythemia vera patients. This signifies the importance of albumin levels as a prognostic factor in patients with secondary erythrocytosis. This is a novel study which compares primary and secondary polycythemia and helps to delineate various prognostic factors in these disease groups. Figure 1. Kaplan Meier curve: Primary Polycythemia Vera vs Secondary Erythrocytosis Figure 1. Kaplan Meier curve: Primary Polycythemia Vera vs Secondary Erythrocytosis Disclosures No relevant conflicts of interest to declare.

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