Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors examined 143 members of a family, where they found 37 (= 25.87%) of Osler patients. It was only in 30% of these patients that the symptoms occurred before the tenth year of age. As in one patient the symptoms did not appear until the age of 58 years, the possibility cannot be excluded that symptoms of the disease will become manifest even in other, sill younger members of the family in the course of time. Epistaxis was observed in 93% of the cases, nephrorrhagia in no case, hepatopathy and gastrorhagy were found only once in each case. The X-ray examination revealed arteriovenous pulmonary aneurysm in 5 cases. As a rule, oestrogen treatment led to good results. A case of death occurred during an influenza epidemic in a severe anaemic patient. Clinical main symptoms of Osler's disease were epistaxis and arteriovenous fistulae which could be roentgenologically identified in the lung. Teleangiectasia could be detected during the autopsy besides vessel anomalies on the surface even in the bronchi, oesophagus, trachea, stomach, kidneys, small intestine and particularly in the large intestines. Conditions of iron deficiency may very often occur in osler Patients; they require a substituting treatment.
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PMID:[Studies on families with Osler's disease]. 7 32

We report the disease characteristics and therapeutic results for 25 patients suffering from essential thrombocythaemia (ET), treated with recombinant interferon-alpha-2b (IFN-alpha 2b). ET was diagnosed according to the criteria of the Polycythaemia Vera Study Group. All patients were programmed to receive a subcutaneous induction treatment consisting of 3 MU of IFN-alpha 2b daily for 6 months. In responding patients, treatment was continued for a further 6 months with 3 MU of IFN-alpha 2b three times a week. Complete response was achieved in 13 of 25 patients, partial response in 10 of 25. In 2 cases, therapy was unsuccessful. Side effects were usually mild, consisting of flu-like symptoms in most cases, and were easily controlled by paracetamol. After a median follow-up of 14 months after discontinuation of the treatment, most patients retained the therapeutic response in the absence of toxicity.
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PMID:Recombinant interferon alpha-2b as treatment of essential thrombocythaemia. 809 33

Erythromelalgia is a rare disease characterised by palmar and plantar erythema, burning pain and local increases in temperature. Erythromelalgia in adults most commonly appears secondary to myeloproliferative disorders, essential thrombocytosis and polycythemia vera; however, in children primary forms predominate. Erythromelalgia in children is characterised by a chronic relapsing course, usually refractory to treatment. We describe a case of erythromelalgia which developed in a 4.5 year old girl following influenza vaccination. Low dose aspirin, carbamazepine and propranolol induced a rapid resolution of the syndrome.
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PMID:Erythromelalgia following influenza vaccine in a child. 909 85

The effect of interferon alpha (IFN) on myeloproliferation and vascular complications was studied in 32 patients (17 female, 15 male; median age 60.5 yr) with polycythemia vera (PV). IFN therapy was initiated at a median time of 19 months after diagnosis. Ten patients were pretreated with chemotherapy in addition to phlebotomy. IFN dose was 12 megaU/wk during the first year, 9 megaU/wk during the second year and 12 megaU/wk thereafter. During IFN alpha treatment hematocrit level was 45.7% and remained at this level after the second year of treatment, compared to 46.5% before IFN. The frequency of phlebotomy before IFN was 0.49/month and dropped to 0.19/month (p <0.0005) during the first year of IFN treatment. IFN normalized high platelet and leukocyte counts in a majority of patients. The incidence of deep venous thromboses was 3.6%/yr before IFN alpha and 1.8%/yr during the first year of treatment. IFN-induced side-effects were mainly flu-like symptoms, fever, fatigue and arthralgia. In conclusion, IFN allowed the reduction of the dose of chemotherapy and decreased the need of phlebotomy. Despite improvement of hematological parameters, it is still uncertain whether IFN alpha can improve clinical symptoms in PV.
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PMID:The effect of interferon alpha on myeloproliferation and vascular complications in polycythemia vera. 991 8

Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic disorders for which there are no medical cures. Clinical sequelae of PV and ET fall into three categories: primary, such as thrombosis and hemorrhage; secondary, resulting from disease progression or treatment. The decision whether to treat the patient is based on the sequelae of no treatment versus short- and long-term toxicities of the three classes of drugs available for treatment: hydroxyurea, interferon-alpha, and anagrelide. Thrombosis is the most common short-term sequelae of untreated disease; the risk increases with age and after the first thrombotic complication. Hydroxyurea, a nonalkylating myelosuppressive agent, is mutagenic and probably leukemogenic over 5 to 15 years, which makes it unsuitable for treating most younger patients. Interferon-alpha, a cytokine that is myelosuppressive and immunomodulatory, has been shown to have a therapeutic effect in both PV and ET. Tolerance to the initial flu-like symptoms of interferon-alpha is usually developed, but dose-limiting symptoms of anorexia, asthenia, and neuropsychiatric disease can occur, along with exacerbation or development of autoimmune diseases. Anagrelide, a quinazoline that inhibits cyclic nucleotide phosphodiesterase, inhibits platelet aggregation and has an idiosyncratic effect of inhibiting megakaryocyte maturation and platelet budding at doses below those that affect platelet function. This agent is a vasodilator with positive inotropic activity and a side-effect profile that may include palpitations, forceful heartbeat, tachycardia, and headache. One in four patients develop fluid retention and/or edema that are controllable with diuretic therapy. Dizziness is frequent, but mild. Because these side effects usually abate in 2 to 4 weeks, successful management of patients taking anagrelide depends on encouraging them to maintain therapy. The availability of these three classes of drugs with differing modes of action suggests that combination therapy may offer the opportunity to achieve better control of proliferation while reducing short-term side effects as well as the risks of dose-related cumulative sequelae.
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PMID:Other secondary sequelae of treatments for myeloproliferative disorders. 1209 54

Pteropine orthoreovirus (PRV; Reoviridae: Spinareovirinae) is an emerging bat-borne zoonotic virus that causes influenza-like illness (ILI). PRV has thus far been found only in Australia and Asia, where diverse old-world fruit bats (Pteropodidae) serve as hosts. In this study, we report the discovery of PRV in Africa, in an Angolan soft-furred fruit bat (Lissonycteris angolensis ruwenzorii) from Bundibugyo District, Uganda. Metagenomic characterization of a rectal swab yielded 10 dsRNA genome segments, revealing this virus to cluster within the known diversity of PRV variants detected in bats and humans in Southeast Asia. Phylogeographic analyses revealed a correlation between geographic distance and genetic divergence of PRVs globally, which suggests a geographic continuum of PRV diversity spanning Southeast Asia to sub-Saharan Africa. The discovery of PRV in an African bat dramatically expands the geographic range of this zoonotic virus and warrants further surveillance for PRVs outside of Southeast Asia.
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PMID:Pteropine Orthoreovirus in an Angolan Soft-Furred Fruit Bat (Lissonycteris angolensis) in Uganda Dramatically Expands the Global Distribution of an Emerging Bat-Borne Respiratory Virus. 3265 60

Ruxolitinib (RUX), a JAK1/JAK2 inhibitor, is approved for second-line therapy in patients with polycythemia vera (PV) who are resistant or intolerant to hydroxyurea. Due to the immunomodulatory and immunosuppressive effect of RUX, there is an increased susceptibility to infections. However, an increased risk of infection is inherent to even untreated myeloproliferative neoplasms (MPN). To obtain more information on the clinical significance of RUX-associated infections in PV, we reviewed the available literature. There is no evidence-based approach to managing infection risks. Most data on RUX-associated infections are available for MF. In all studies, the infection rates in the RUX and control groups were fairly similar, with the exception of infections with the varicella zoster virus (VZV). However, individual cases of bilateral toxoplasmosis retinitis, disseminated molluscum contagiosum, or a mycobacterium tuberculosis infection or a hepatitis B reactivation are reported. A careful assessment of the risk of infection for PV patients is required at the initial presentation and before the start of RUX. Screening for hepatitis B is recommended in all patients. The risk of RUX-associated infections is lower with PV than with MF, but compared to a normal population there is an increased risk of VZV infection. However, primary VZV prophylaxis for PV patients is not recommended, while secondary prophylaxis can be considered individually. As early treatment is most effective for VZV, patients should be properly informed and trained to seek medical advice immediately if cutaneous signs of VZV develop. Vaccination against influenza, herpes zoster, and pneumococci should be considered in all PV patients at risk of infection, especially if RUX treatment is planned. Current recommendations do not support adjusting or discontinuing JAK inhibition in MPN patients to reduce the risk of COVID-19.
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PMID:Ruxolitinib-Associated Infections in Polycythemia Vera: Review of the Literature, Clinical Significance, and Recommendations. 3311 33